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Sample records for single vascules located

  1. Single-Organ Gallbladder Vasculitis

    Science.gov (United States)

    Hernández-Rodríguez, José; Tan, Carmela D.; Rodríguez, E. René; Hoffman, Gary S.

    2014-01-01

    Abstract Systemic vasculitis (SV) involving abdominal structures usually has a poor prognosis. Gallbladder vasculitis (GV) has been reported as part of SV (GB-SV) and focal single-organ vasculitis (GB-SOV). We analyzed clinical and histologic characteristics of patients with GV to identify features that differentiate GB-SOV from the systemic forms of GV. To identify affected patients with GV we used pathology databases from our institution and an English-language PubMed search. Clinical manifestations, laboratory and histologic features, treatment administered, and outcomes were recorded. Patients were divided in 2 groups, GB-SOV and GB-SV. As in previous studies of single-organ vasculitis, GB-SOV was only considered to be a sustainable diagnosis if disease beyond the gallbladder was not apparent after a follow-up period of at least 6 months. Sixty-one well-characterized patients with GV were included (6 from our institution). There was no significant sex bias (32 female patients, 29 male). Median age was 52 years (range, 18–94 yr). GB-SOV was found in 20 (33%) and GB-SV in 41 (67%) patients. No differences were observed in age, sex frequency, or duration of gallbladder symptoms between groups. Past episodes of recurrent right-upper quadrant or abdominal pain and lithiasic cholecystitis were more frequent in GB-SOV patients, whereas acalculous cholecystitis occurred more often in GB-SV. In GB-SV, gallbladder-related symptoms occurred more often concomitantly with or after the systemic features, but they sometimes appeared before SV was fully developed (13.5%). Constitutional and musculoskeletal symptoms were reported only in GB-SV patients. Compared to GB-SOV, GB-SV patients presented more often with fever (62.5% vs 20%; p = 0.003) and exhibited higher erythrocyte sedimentation rate levels (80 ± 28 vs 37 ± 25 mm/h, respectively; p = 0.006). All GB-SV patients required glucocorticoids and 50% of them also received cytotoxic agents. Mortality in

  2. Vasculitis

    Science.gov (United States)

    Vasculitis is an inflammation of the blood vessels. It happens when the body's immune system attacks the ... or another disease. The cause is often unknown. Vasculitis can affect arteries, veins and capillaries. Arteries are ...

  3. Single-organ gallbladder vasculitis: characterization and distinction from systemic vasculitis involving the gallbladder. An analysis of 61 patients.

    Science.gov (United States)

    Hernández-Rodríguez, José; Tan, Carmela D; Rodríguez, E René; Hoffman, Gary S

    2014-11-01

    Systemic vasculitis (SV) involving abdominal structures usually has a poor prognosis. Gallbladder vasculitis (GV) has been reported as part of SV (GB-SV) and focal single-organ vasculitis (GB-SOV). We analyzed clinical and histologic characteristics of patients with GV to identify features that differentiate GB-SOV from the systemic forms of GV. To identify affected patients with GV we used pathology databases from our institution and an English-language PubMed search. Clinical manifestations, laboratory and histologic features, treatment administered, and outcomes were recorded. Patients were divided in 2 groups, GB-SOV and GB-SV. As in previous studies of single-organ vasculitis, GB-SOV was only considered to be a sustainable diagnosis if disease beyond the gallbladder was not apparent after a follow-up period of at least 6 months. Sixty-one well-characterized patients with GV were included (6 from our institution). There was no significant sex bias (32 female patients, 29 male). Median age was 52 years (range, 18-94 yr). GB-SOV was found in 20 (33%) and GB-SV in 41 (67%) patients. No differences were observed in age, sex frequency, or duration of gallbladder symptoms between groups. Past episodes of recurrent right-upper quadrant or abdominal pain and lithiasic cholecystitis were more frequent in GB-SOV patients, whereas acalculous cholecystitis occurred more often in GB-SV. In GB-SV, gallbladder-related symptoms occurred more often concomitantly with or after the systemic features, but they sometimes appeared before SV was fully developed (13.5%). Constitutional and musculoskeletal symptoms were reported only in GB-SV patients. Compared to GB-SOV, GB-SV patients presented more often with fever (62.5% vs 20%; p = 0.003) and exhibited higher erythrocyte sedimentation rate levels (80 ± 28 vs 37 ± 25 mm/h, respectively; p = 0.006). All GB-SV patients required glucocorticoids and 50% of them also received cytotoxic agents. Mortality in GB-SV was

  4. Hypersensitivity vasculitis

    Science.gov (United States)

    Cutaneous small vessel vasculitis; Allergic vasculitis; Leukocytoclastic vasculitis ... Hypersensitivity vasculitis, or cutaneous small vessel vasculitis, is caused by: An allergic reaction to a drug or other foreign ...

  5. Urticarial Vasculitis

    Science.gov (United States)

    ... Home / Resources / Forms of Vasculitis / Urticarial Vasculitis Urticarial Vasculitis Swap out your current Facebook Profile Cover with ... complements; it’s called hypocomplementemic vasculitis. What causes Urticarial Vasculitis? The cause of most cases of urticarial vasculitis ...

  6. Rheumatoid Vasculitis

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    ... here: Home / Types of Vasculitis / Rheumatoid Vasculitis Rheumatoid Vasculitis First Description Who gets Rheumatoid Vasculitis (the “typical” ... or scarring of the lungs). Who gets Rheumatoid Vasculitis? A typical patient RV can affect a person ...

  7. Percutaneous Management of Occlusive Arterial Disease Associated with Vasculitis: A Single Center Experience

    International Nuclear Information System (INIS)

    Both, M.; Jahnke, T.; Reinhold-Keller, E.; Reuter, M.; Grimm, J.; Biederer, J.; Brossmann, J.; Gross, W.L.; Heller, M.; Mueller-Huelsbeck, S.

    2003-01-01

    The purpose of this study was to evaluate the safety and effectiveness of percutaneous transluminal angioplasty for occlusive arterial disease associated with vasculitis. Eleven patients(10 women, 1 man; ages 35-82 years) with the diagnosis of vasculitis of the large vessels underwent interventional treatment during intraarterial angiography. The causes included giant cell arteritis(n = 8) and Takayasu arteritis (n = 3).Thirty-three occlusive lesions (including brachiocephalic and renalarteries, and arteries of upper and lower extremities) were treated with balloon angioplasty and/or stent placement. Follow-up included clinical examination, angiography, and color duplex ultrasound.Technical success was 100% (25/25) for stenoses and 50% (4/8) for occlusive lesions, representing all lesions combined from different anatomic locations. Dissection (n = 3) and arterial rupture with retroperitoneal hematoma (n = 1) was found in three patients. During follow-up (mean 12 months), restenoses(n = 8) and re-restenoses (n = 1)occurred in 8 vascular areas. Three of these lesions were treated with repeated PTA (n = 4). The cumulative primary clinical success rate was 67.6%, cumulative secondary success rate 74.4%, and cumulative tertiary success rate 75.9%. Interventional therapy in systemic vasculitis provides promising results in technical success rates and followup. Angioplasty may result in arterial injury, but the rate of complications is low

  8. Vasculitis Foundation

    Science.gov (United States)

    ... Read More Click here to view full calendar Vasculitis Patient-Powered Research Network The V-PPRN seeks ... with various forms of vasculitis. Join The Network! Vasculitis Foundation News Introducing our 2017-2018 VCRC-VF ...

  9. Necrotizing vasculitis

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    ... page: //medlineplus.gov/ency/article/000432.htm Necrotizing vasculitis To use the sharing features on this page, please enable JavaScript. Necrotizing vasculitis is a group of disorders that involve inflammation ...

  10. Hypersensitivity Vasculitis

    Science.gov (United States)

    ... to describe different types of vasculitis related to drug reactions, skin disorders or allergic vasculitis; however this is not always the correct use of the term. The American College of Rheumatology established a list of criteria for ...

  11. Cryoglobulinemia Vasculitis.

    Science.gov (United States)

    Cacoub, Patrice; Comarmond, Cloe; Domont, Fanny; Savey, Léa; Saadoun, David

    2015-09-01

    Cryoglobulinemic vasculitis (CryoVas) is a small-vessel vasculitis involving mainly the skin, the joints, the peripheral nervous system, and the kidneys. Type I CryoVas is single monoclonal immunoglobulins related to an underlying B-cell lymphoproliferative disorder. Type II and III cryoglobulins, often referred to as mixed cryoglobulinemia, consist of polyclonal immunoglobulin (Ig)G with or without monoclonal IgM with rheumatoid factor activity. Hepatitis C virus (HCV) infection represents the main cause of mixed CryoVas. The 10-year survival rates are 63%, 65%, and 87% in HCV-positive mixed CryoVas, HCV-negative mixed CryoVas, and type I CryoVas patients, respectively. In HCV-positive patients, baseline poor prognostic factors include the presence of severe liver fibrosis, and central nervous system, kidney, and heart involvement. Treatment with antivirals is associated with a good prognosis, whereas use of immunosuppressants (including corticosteroids) is associated with a poor outcome. In HCV-negative patients, pulmonary and gastrointestinal involvement, renal insufficiency, and age > 65 years are independently associated with death. Increased risk of lymphoma also should be underlined. Treatment of type I CryoVas is that of the hemopathy; specific treatment also includes plasma exchange, corticosteroids, rituximab, and ilomedine. In HCV-CryoVas with mild-to-moderate disease, an optimal antiviral treatment should be given. For HCV-CryoVas with severe vasculitis (ie, worsening of renal function, mononeuritis multiplex, extensive skin disease, intestinal ischemia…) control of disease with rituximab, with or without plasmapheresis, is required before initiation of antiviral therapy. Other immunosuppressants should be given only in case of refractory forms of CryoVas, frequently associated with underlying B-cell lymphoma. Copyright © 2015 Elsevier Inc. All rights reserved.

  12. Refractory vasculitis

    NARCIS (Netherlands)

    Rutgers, Bram; Kallenberg, Cees G. M.

    Refractory vasculitis occurs in 4-5% of patients with anti-neutrophil cytoplasmic antibody associated vasculitis (AAV). Differences between therapies used for refractory disease are mostly reflected in the percentages of complete and partial remissions, but also in the number of serious side

  13. Retinal Vasculitis

    Science.gov (United States)

    Rosenbaum, James T.; Sibley, Cailin H.; Lin, Phoebe

    2016-01-01

    Purpose of review Ophthalmologists and rheumatologists frequently miscommunicate in consulting on patients with retinal vasculitis. This report seeks to establish a common understanding of the term, retinal vasculitis, and to review recent papers on this diagnosis. Recent findings 1) The genetic basis of some rare forms of retinal vascular disease have recently been described. Identified genes include CAPN5, TREX1, and TNFAIP3; 2) Behçet’s disease is a systemic illness that is very commonly associated with occlusive retinal vasculitis; 3) retinal imaging including fluorescein angiography and other newer imaging modalities has proven crucial to the identification and characterization of retinal vasculitis and its complications; 4) although monoclonal antibodies to IL-17A or IL-1 beta failed in trials for Behçet’s disease, antibodies to TNF alpha, either infliximab or adalimumab, have demonstrated consistent benefit in managing this disease. Interferon treatment and B cell depletion therapy via rituximab may be beneficial in certain types of retinal vasculitis. Summary Retinal vasculitis is an important entity for rheumatologists to understand. Retinal vasculitis associated with Behçet’s disease responds to monoclonal antibodies that neutralize TNF, but the many other forms of non-infectious retinal vasculitis may require alternate therapeutic management. PMID:26945335

  14. Pediatric vasculitis.

    Science.gov (United States)

    Barut, Kenan; Sahin, Sezgin; Kasapcopur, Ozgur

    2016-01-01

    The aim of this review is to define childhood vasculitis and to highlight new causative factors and treatment modalities under the guidance of recently published studies. Childhood vasculitis is difficult to diagnose because of the wide variation in the symptoms and signs. New nomenclature and classification criteria were proposed for the diagnosis of pediatric vasculitis. Recently, progress has been made toward understanding the genetic susceptibility to pediatric vasculitis as it was in other diseases. Various radiological techniques provide great opportunities in establishing the diagnosis of pediatric vasculitis. Mild central nervous system disease can accompany Henoch-Schonlein purpura and can go unnoticed. Antineutrophilic cytoplasmic antibody-associated vasculitis is rare in children. Increased severity of the disease, subglottic stenosis, and renal disease are described more frequently among children. Biological therapies are used with success in children as in adults. Future studies, whose aims are to evaluate treatment responses, prognosis and to design guidelines for activity, and damage index of vasculitis for children are required. Henoch-Schonlein purpura and Kawasaki disease are the most frequent vasculitides of children. Experience from adult studies for treatment and prognosis are usually used because of low incidence of other vasculitides in children. Multicenter studies of pediatric vasculitis should be conducted to detail treatment responses and prognosis in children.

  15. Cerebral vasculitis

    International Nuclear Information System (INIS)

    Greenan, T.J.; Grossman, R.I.

    1990-01-01

    This paper reviews retrospectively MR, CT, and angiographic findings in patients with cerebral vasculitis in order to understand the strengths and weaknesses of the various imaging modalities, as well as the spectrum of imaging abnormalities in this disease entity. Studies were retrospectively reviewed in 12 patients with cerebral vasculitis proved by means of angiography and/or brain biopsy

  16. Drug-associated cutaneous vasculitis: study of 239 patients from a single referral center.

    Science.gov (United States)

    Ortiz-Sanjuán, Francisco; Blanco, Ricardo; Hernández, José L; Pina, Trinitario; González-Vela, María C; Fernández-Llaca, Héctor; Calvo-Río, Vanesa; Loricera, Javier; Armesto, Susana; González-López, Marcos A; Rueda-Gotor, Javier; González-Gay, Miguel A

    2014-11-01

    The 2012 International Chapel Hill Consensus Conference on the Nomenclature of Vasculitides defined drug-associated immune complex vasculitis as a distinct entity included within the category of vasculitis associated with probable etiology. In the present study we assessed the clinical spectrum of patients with drug-associated cutaneous vasculitis (DACV). Case records were reviewed of patients with DACV treated at a tertiary referral hospital over a 36-year period. A diagnosis of DACV was considered if the drug was taken within a week before the onset of the disease. From a series of 773 unselected cutaneous vasculitis cases, 239 patients (30.9%; 133 men and 106 women; mean age 36 yrs) were diagnosed with DACV. Antibiotics (n=149; 62.3%), mainly β-lactams and nonsteroidal antiinflammatory drugs (NSAID; n=24; 10%) were the most common drugs. Besides skin lesions (100%), the most common clinical features were joint (51%) and gastrointestinal (38.1%) manifestations, nephropathy (34.7%), and fever (23.8%). The most remarkable laboratory data were increased erythrocyte sedimentation rate (40.2%), presence of serum cryoglobulins (26%), leukocytosis (24.7%), positive antinuclear antibodies (21.1%), anemia (18.8%), and positive rheumatoid factor (17.5%). Despite drug discontinuation and bed rest, 108 patients (45.2%) required medical treatment, mainly corticosteroids (n=71) or immunosuppressive drugs (n=7). After a median followup of 5 months, relapses occurred in 18.4% of patients, and persistent microhematuria or renal insufficiency in 3.3% and 5%, respectively. DACV is generally associated with antibiotics and NSAID. In most cases it has a favorable prognosis, although a small percentage of patients may develop residual renal damage.

  17. Retinal vasculitis.

    Science.gov (United States)

    Abu El-Asrar, Ahmed M; Herbort, Carl P; Tabbara, Khalid F

    2005-12-01

    Retinal vasculitis is a sight-threatening intraocular inflammation affecting the retinal vessels. It may occur as an isolated ocular condition, as a manifestation of infectious or neoplastic disorders, or in association with a systemic inflammatory disease. The search for an underlying etiology should be approached in a multidisciplinary fashion based on a thorough history, review of systems, physical examination, and laboratory evaluation. Discrimination between infectious and noninfectious etiologies of retinal vasculitis is important because their treatment is different. This review is based on recently published articles on retinal vasculitis and deals with its clinical diagnosis, its link with systemic diseases, and its laboratory investigation.

  18. Childhood primary large vessel CNS vasculitis: single-centre experience and review of the literature.

    Science.gov (United States)

    Walsh, Sonja; Knöfler, Ralf; Hahn, Gabriele; Lohse, Judith; Berner, Reinhard; Brenner, Sebastian; Smitka, Martin; von der Hagen, Maja; Hedrich, Christian M

    2017-01-01

    Ischaemic brain injuries are rare conditions in the paediatric age group. Main causes include non-arteriosclerotic arteriopathies, which in childhood usually result from primary vasculitis of large or small vessels and lead to impaired perfusion and subsequent ischaemic brain lesions. In accordance with the nomenclature of systemic forms, CNS vasculitis is subdivided into groups, based on the size of affected vessels: angiography-positive primary angiitis of medium-sized and large vessels (pPACNS), and angiography-negative angiitis of small vessels (svPACNS). We report the clinical presentation, diagnostic approach, and therapy of four children with progressive pPACNS. Patients were treated with high-dose corticosteroids and anticoagulation with unfractionated heparin in the acute phase, followed by immune modulatory treatment with mycophenolate mofetil (MMF) and dual antiplatelet therapy with acetylsalicylic acid and clopidogrel. In this manuscript, we illustrate the experience gained in our hospital, resulting in significantly faster diagnosis and treatment initiation, and discuss the applied immune modulating treatment regimen in the context of the literature. Based on our observations, we conclude that immune modulating therapy with initial high-dose corticosteroids, followed by steroid-sparing maintenance treatment with MMF, may be safe and effective in childhood progressive pPACNS.

  19. Central Nervous System Vasculitis

    Science.gov (United States)

    ... Forms of Vasculitis / Central Nervous System (CNS) Vasculitis Central Nervous System (CNS) Vasculitis Swap out your current Facebook Profile ... your Facebook personal page. Replace with this image. Central nervous system (CNS) vasculitis is inflammation of blood vessel walls ...

  20. Vasculitis Pregnancy Registry

    Science.gov (United States)

    2018-04-30

    Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatosis With Polyangiitis (EGPA); Churg-Strauss Syndrome (CSS); Granulomatosis With Polyangiitis (GPA); Wegener's Granulomatosis; IgA Vasculitis; Henoch-Schoenlein Purpura (HSP); Microscopic Polyangiitis (MPA); Polyarteritis Nodosa (PAN); Takayasu Arteritis (TAK); Urticarial Vasculitis; Systemic Vasculitis

  1. Cerebral Vasculitis

    Directory of Open Access Journals (Sweden)

    Fariborz Khorvash

    2017-02-01

    Full Text Available Introduction: Vasculitis is an inflammation systems may be involved of blood vessels due to various origins. Vessels of the peripheral and/or central nervous. Vasculitis of the CNS is rare and occurs in the context of systemic diseases or as primary angiitis of the CNS. Epidemiology: The overall incidence of primary vasculitis is about 40/1,000,000 persons [excluding giant cell (temporal arteritis, GCA]. Its incidence increases with age. The incidence of GCA is much higher (around 200/1,000,000 persons in the age group[50 years. Clinical Presentation: Clinical and pathological presentation in CNS vasculitis represents a wide spectrum. Among others, headache, cranial nerve affections, encephalopathy, seizures, psychosis, myelitis, stroke, intracranial haemorrhage and aseptic meningoencephalitis are described. Primary and secondary vasculitides leading more frequently to CNS manifestations are discussed. Primary and secondary Vasculitides: Including Giant Cell (Temporal Arteritis , Takayasu arteritis, Polyarteritis nodosa, Primary angiitis of the CNS, Wegener’s granulomatosis, and Connective tissue diseases, such as systemic lupus erythematosus (SLE, scleroderma, rheumatoid arthritis, mixed connective disease and Sjögren syndrome, are systemic immune-mediated diseases that lead to multiple organ affections. Cerebral Vasculitis: Imaging and Differential Diagnosis: Vasculitides represent a heterogeneous group of inflammatory diseases that affect blood vessel walls of varying calibers (inflammatory vasculopathy. Since the devastating symptoms of CNS vasculitis are at least partially reversible, early diagnosis and appropriate treatment are important. In order to establish a differential diagnosis clinical features, disease progression, age of onset, blood results, as well as CSF examinations have to be taken into consideration. Neuroimaging techniques, such as MRI and DSA, play a central role in the diagnosis and disease monitoring .The diagnostic

  2. Pediatric Vasculitis Initiative

    Science.gov (United States)

    2017-05-01

    Wegeners Granulomatosis (Granulomatosis With Polyangiitis); Microscopic Polyangiitis; Churg Strauss Syndrome (Eosinophilic Granulomatosis With Polyangiitis); Polyarteritis Nodosa; Takayasu Arteritis; Primary CNS Vasculitis; Unclassified Vasculitis

  3. Vasculitis and inflammatory arthritis.

    Science.gov (United States)

    Watts, Richard A; Scott, David G I

    2016-10-01

    Vasculitis has been described in most types of inflammatory arthritis. The best described and most widely recognised form is rheumatoid vasculitis. The incidence of systemic rheumatoid vasculitis has declined significantly following the general early use of methotrexate in the 1990s, and it is now a rare form of vasculitis. Treatment of rheumatoid vasculitis is conventionally with glucocorticoids and cyclophosphamide, but there is an increasing role for rituximab similar to that in other types of vasculitis. Despite these developments the mortality of rheumatoid vasculitis remains high. Vasculitis in other types of inflammatory arthritis is less well described and the treatment remains empirical. Copyright © 2016 Elsevier Ltd. All rights reserved.

  4. [Clinical features of 32 patients with cutaneous small vessel vasculitis].

    Science.gov (United States)

    López de Maturana, Donaldo; Amaro, Patricio; Segovia, Laura; Balestrini, Claudia

    2004-02-01

    The skin is a common target of small vessel vasculitis, with a wide assortment of pathological changes. This condition is usually associated to systemic diseases. To report the clinical and pathological features of patients with cutaneous small vessel vasculitis. A retrospective review of 32 patients with a pathological diagnosis of cutaneous vasculitis. Seventy two percent of patients were women. Cutaneous lesions were mainly located in the lower limbs (94%). The most common lesion was palpable purpura (62%). Connective tissue diseases and systemic vasculitis were the most commonly associated systemic diseases. Palpable purpura is the most common manifestation of cutaneous small vessel vasculitis, that is usually associated to connective tissue diseases or systemic vasculitis.

  5. Impact of Vasculitis on Employment and Income

    Science.gov (United States)

    2016-01-26

    Vasculitis; Systemic Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatosis; Temporal Arteritis; Wegener Granulomatosis; Henoch-Schoenlein Purpura; Microscopic Polyangiitis; Polyarteritis Nodosa (PAN); Takayasu's Arteritis; Urticarial Vasculitis

  6. Acute vasculitis after endovascular brachytherapy

    International Nuclear Information System (INIS)

    Fajardo L-G, Luis F.; Prionas, Stavros D.; Kaluza, Grzegorz L.; Raizner, Albert E.

    2002-01-01

    Purpose: Angioplasty effectively relieves coronary artery stenosis but is often followed by restenosis. Endovascular radiation (β or γ) at the time of angioplasty prevents restenosis in a large proportion of vessels in swine (short term) and humans (short and long term). Little information is available about the effects of this radiation exposure beyond the wall of the coronary arteries. Methods and Materials: Samples were obtained from 76 minipigs in the course of several experiments designed to evaluate endovascular brachytherapy: 76 of 114 coronary arteries and 6 of 12 iliac arteries were exposed to endovascular radiation from 32 P sources (35 Gy at 0.5 mm from the intima). Two-thirds of the vessels had angioplasty or stenting. The vessels were systematically examined either at 28 days or at 6 months after radiation. Results: We found an unexpected lesion: acute necrotizing vasculitis in arterioles located ≤2.05 mm from the target artery. It was characterized by fibrinoid necrosis of the wall, often associated with lymphocytic exudates or thrombosis. Based on the review of perpendicular sections of tissue samples, the arterioles had received between 6 and 40 Gy. This arteriolar vasculitis occurred at 28 days in samples from 51% of irradiated coronary arteries and 100% of irradiated iliac arteries. By 6 months, the incidence of acute vasculitis decreased to 24% around the coronary arteries. However, at that time, healing vasculitis was evident, often with luminal narrowing, in 46% of samples. Vasculitis was not seen in any of 44 samples from unirradiated vessels (0%) and had no relation to angioplasty, stenting, or their sequelae. This radiation-associated vasculitis in the swine resembles the localized lymphocytic vasculitis that we have reported in tissues of humans exposed to external radiation. On the other hand, it is quite different from the various types of systemic vasculitis that occur in nonirradiated humans. Conclusion: Endoarterial brachytherapy

  7. Pathogenesis of pulmonary vasculitis

    NARCIS (Netherlands)

    Heeringa, P; Schreiber, A; Falk, RJ; Jennette, JC

    2004-01-01

    Vasculitis is inflammation of blood vessels and can affect any type of vessel in any organ. Pulmonary vasculitis usually is a component of a systemic small vessel vasculitis. Three major forms of small vessel vasculitis that often affect the lungs are Wegener's granulomatosis, microscopic

  8. Protein Laboratories in Single Location | Poster

    Science.gov (United States)

    By Andrew Stephen, Timothy Veenstra, and Gordon Whiteley, Guest Writers, and Ken Michaels, Staff Writer The Laboratory of Proteomics and Analytical Technologies (LPAT), Antibody Characterization Laboratory (ACL), and Protein Chemistry Laboratory (PCL), previously located on different floors or in different buildings, are now together on the first floor of C wing in the ATRF.

  9. Clinical Transcriptomics in Systemic Vasculitis (CUTIS)

    Science.gov (United States)

    2018-04-10

    Cryoglobulinemic Vasculitis (CV); Drug-induced Vasculitis; Eosinophilic Granulomatosis With Polyangiitis (EGPA); IgA Vasculitis; Isolated Cutaneous Vasculitis; Granulomatosis With Polyangiitis (GPA); Microscopic Polyangiitis (MPA); Polyarteritis Nodosa (PAN); Urticarial Vasculitis; Vasculitis

  10. A cross-sectional study of the Birmingham Vasculitis Activity Score version 3 in systemic vasculitis

    NARCIS (Netherlands)

    Suppiah, Ravi; Mukhtyar, Chetan; Flossmann, Oliver; Alberici, Federico; Baslund, Bo; Batra, Rajbir; Brown, Denise; Holle, Julia; Hruskova, Zdenka; Jayne, David R. W.; Judge, Andrew; Little, Mark A.; Palmisano, Alessandra; Stegeman, Coen; Tesar, Vladimir; Vaglio, Augusto; Westman, Kerstin; Luqmani, Raashid

    Methods. A total of 238 patients with vasculitis from seven countries in Europe were evaluated at a single time point. Spearman's correlation coefficients were calculated between BVAS v. 3 scores, vasculitis activity index (VAI), physician's global assessment (PGA), the physician's treatment

  11. Vasculitis and infectious diseases.

    Science.gov (United States)

    Satta, R; Biondi, G

    2015-04-01

    Vasculitis usually presents without a well-known underline cause (idiopathic vasculitis), nevertheless, it is sometimes possible to find out one or more causative agents (secondary vasculitis). Nowadays, thanks to the increasing amount of precise diagnostic tools, a piece of idiopathic vasculitis is reclassified as associated with probable etiology, which can be set off by several factors, such as infections. Infections are considered to be the most common cause of secondary vasculitis. Virtually, every infectious agent can trigger a vasculitis by different mechanisms which can be divided in two main categories: direct and indirect. In the former, infectious agents destroy directly the vascular wall leading, eventually, to a subsequent inflammatory response. In the latter, indirect form, they stimulate an immune response against blood vessels. Different infectious agents are able to directly damage the vascular wall. Among these, it is possible to recognize Staphylococcus spp, Streptococcus spp, Salmonella spp, Treponema spp, Rickettsia spp, Cytomegalovirus, Herpes Simplex Virus 1 and 2, and many others which have a peculiar tropism for endothelial cells. Conversely, another group of microbial agents, such as Mycobacterium tuberculosis, Mycobacterium leprae, Hepatits B Virus, Human Immunodeficiency Virus and others, trigger vasculitis in the indirect way. This is due to the fact that they can share epitopes with the host or modify self-antigens, thus leading to a cross-self reaction of the immune system. These mechanism, in turn, leads to immunological responses classified as type I-IV by Gell-Coombs. Nevertheless, it is difficult to strictly separate the direct and indirect forms, because most infectious agents can cause vasculitis in both ways (mixed forms). This paper will analyze the link between infectious agents and vasculitis, focusing on direct and indirect secondary vasculitis, and on a group of probable infection-related idiopathic vasculitis, and finally

  12. A cross-sectional study of the Birmingham Vasculitis Activity Score version 3 in systemic vasculitis

    DEFF Research Database (Denmark)

    Suppiah, Ravi; Mukhtyar, Chetan; Flossmann, Oliver

    2011-01-01

    Objective. Assessment of disease activity in vasculitis can be achieved using the BVAS, a clinical checklist of relevant symptoms, signs and features of active disease. The aim of this study was to revalidate the BVAS version 3 (BVAS v. 3) in a cohort of patients with systemic vasculitis. Methods....... A total of 238 patients with vasculitis from seven countries in Europe were evaluated at a single time point. Spearman's correlation coefficients were calculated between BVAS v. 3 scores, vasculitis activity index (VAI), physician's global assessment (PGA), the physician's treatment decision, CRP...... and the vasculitis damage index (VDI) to demonstrate that the BVAS v. 3 measures disease activity. Results. WG (63%), Churg-Strauss syndrome (9%) and microscopic polyangiitis (9%) were the most common diagnoses. The BVAS v. 3 showed convergent validity with the VAI [¿¿=¿0.82 (95% CI 0.77, 0.85)], PGA [¿¿=¿0.85 (95...

  13. Neuroimaging of cerebral vasculitis

    International Nuclear Information System (INIS)

    Wengenroth, M.; Saam, T.; Haehnel, S.

    2016-01-01

    Cerebral vasculitis can have a variety of origins. Furthermore, there are no vasculitis-specific symptoms or imaging signs and vasculitis of the CNS can mimic many other neurological diseases, which require different treatment approaches. Thus, the clinical and radiological diagnosis of cerebral vasculitis is challenging. Magnetic resonance imaging (MRI) and MR angiography (MRA) should be the radiological imaging methods of choice to assess the degree of parenchymal damage and to detect vessel wall changes. If the results are unclear digital subtraction angiography (DSA) should be pursued in order to also detect changes in medium sized vessels. Vasculitis of small vessels cannot be detected by vascular imaging and requires brain or leptomeningeal biopsy. In this review we present the current diagnostic approach and a variety of imaging findings in cerebral vasculitis and discuss the main radiological differential diagnoses. (orig.) [de

  14. Pulmonary vasculitis: imaging features

    International Nuclear Information System (INIS)

    Seo, Joon Beom; Im, Jung Gi; Chung, Jin Wook; Goo, Jin Mo; Park, Jae Hyung; Yeon, Kyung Mo; Song, Jae Woo

    1999-01-01

    Vasculitis is defined as an inflammatory process involving blood vessels, and can lead to destruction of the vascular wall and ischemic damage to the organs supplied by these vessels. The lung is commonly affected. A number of attempts have been made to classify and organize pulmonary vasculitis, but because the clinical manifestations and pathologic features of the condition overlap considerably, these afforts have failed to achieve a consensus. We classified pulmonary vasculitis as belonging to either the angitiis-granulomatosis group, the diffuse pulmonary hemorrhage with capillaritis group, or 'other'. Characteristic radiographic and CT findings of the different types of pulmonary vasculitis are illustrated, with a brief discussion of the respective disease entities

  15. Vasculitis and Pregnancy.

    Science.gov (United States)

    Machen, Leah; Clowse, Megan E B

    2017-05-01

    Vasculitis is more often a disease of women beyond their reproductive years, leaving the challenges of pregnancy management difficult to study. Pregnancy complications, including pregnancy loss and preterm birth, are higher among women with all forms of vasculitis. It seems that controlling the disease before pregnancy may improve the chances of pregnancy success. Many medications used for vasculitis are considered low risk in pregnancy, including prednisone, colchicine, azathioprine, and tumor necrosis factor inhibitors. Cyclophosphamide, methotrexate, and mycophenolate mofetil should be avoided in pregnancy. Controlling disease with low-risk medications may allow women with vasculitis to have the pregnancies they desire. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. Managing mesenteric vasculitis.

    Science.gov (United States)

    Angle, John Fritz; Nida, Berhanemeskel A; Matsumoto, Alan H

    2015-03-01

    Mesenteric vasculitis is a rare diagnosis, but it comprises a group of disorders that may have devastating manifestations. It is often difficult to diagnose using clinical symptoms and biomarkers. Vascular imaging often provides the best opportunity for the noninvasive diagnosis of vasculitis and obviates the need for performing a biopsy. The medical management of vasculitis involves controlling the inflammatory process with the use of steroids or other immunosuppressants, but medical therapy does not consistently provide regression of the vascular changes (ie, aneurysms or vascular occlusions) seen at the time of the initial diagnosis. Operative management remains the mainstay of therapy for focal occlusive or aneurysms, but the treatment options for multifocal disease remain challenging. Endovascular treatment is increasingly being used as a first line of treatment for symptomatic vasculitis. Interventionalists should be familiar with the indications and outcomes associated with the various therapeutic options for mesenteric vasculitis-associated occlusive disease and aneurysms. Copyright © 2015 Elsevier Inc. All rights reserved.

  17. Journey of Patients With Vasculitis From First Symptom to Diagnosis

    Science.gov (United States)

    2018-01-18

    Vasculitis; Systemic Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatous Vasculitis; Temporal Arteritis; Giant Cell Arteritis; Granulomatosis With Polyangiitis; Wegener Granulomatosis; Henoch Schonlein Purpura; IgA Vasculitis; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu Arteritis; Urticarial Vasculitis

  18. Thromboembolic disease in vasculitis

    Science.gov (United States)

    Tomasson, Gunnar; Monach, Paul A.; Merkel, Peter A.

    2011-01-01

    Purpose of review To give an overview of recent clinical findings of thromboembolic disease in vasculitis and provide insight into possible explanations of the association between thrombosis and inflammation. Recent findings A high incidence of venous thrombotic events has recently been described in four distinct cohorts of patients with antineutrophil cytoplasmic antibodies-associated vasculitis (AAV), especially during periods of active disease. No factors other than the vasculitis itself have been identified that explain this high occurrence of thrombosis. Several studies have shown an increased rate of thrombosis in Behçet’s disease, with a different clinical presentation than that observed in AAV. Recent laboratory findings provide exciting insights into a bidirectional feedback loop between coagulation and inflammation that may be applicable to vasculitis. Summary Thrombosis is an important clinical manifestation of some types of vasculitis. Better understanding of the association of thrombosis with inflammation in vasculitis might lead to development of clinically useful biomarkers and new approaches to therapy. Additionally, study of the specific factors involved in thrombosis in systemic vasculitis could help explain the role of inflammation in more common settings of venous thrombotic events. PMID:19077717

  19. Lattice location of helium in uranium dioxide single crystals

    Energy Technology Data Exchange (ETDEWEB)

    Garrido, F.; Nowicki, L. E-mail: lech.nowicki@fuw.edu.pl; Sattonnay, G.; Sauvage, T.; Thome, L

    2004-06-01

    Lattice location of {sup 3}He atoms implanted into UO{sub 2} single crystals was performed by means of the channeling technique combined with nuclear reaction analysis (NRA) and Rutherford backscattering spectrometry (RBS). The {sup 3}He(d,p){sup 4}He reaction was used. The experimental angular scans show that helium atoms occupy octahedral interstitial positions.

  20. Omalizumab for Urticarial Vasculitis

    DEFF Research Database (Denmark)

    Ghazanfar, Misbah Nasheela; Thomsen, Simon Francis

    2015-01-01

    Urticarial vasculitis is characterised by inflamed itching or burning red patches or wheals that resemble urticaria but persist for greater than 24 hours. It is often idiopathic but is sometimes associated with collagen-vascular disease, particularly systemic lupus erythematosus. Treatment options...... include oral antihistamines, oral corticosteroids, dapsone, colchicine or hydroxychloroquine. We describe a male patient with urticarial vasculitis who was treated with omalizumab (anti-IgE) with convincing results and provide a review of previous reports of patients with urticarial vasculitis treated...

  1. [Pulmonary Manifestations of Vasculitis].

    Science.gov (United States)

    von Vietinghoff, S

    2016-11-01

    The variable symptoms and signs of pulmonary vasculitis are a diagnostic and therapeutic challenge. Vasculitis should be considered in rapidly progressing, severe and unusual manifestations of pulmonary disease. Clinical examination of other organ systems typically affected by vasculitis such as skin and kidney and autoantibody measurements are complementary approaches to manage this situation. Pulmonary involvement is common in small vessel vasculitis including anti-GBM disease (Goodpasture syndrome) and the ANCA-associated vasculitides. Life threatening pulmonary hemorrhage and irreversible damage of other organs, frequently the kidney, are important complications necessitating rapid diagnosis of these conditions.Vasculitides are rare diseases of multiple organs and therapies including biologics are evolving rapidly, requiring cooperation of specialities and with specialized centres to achieve best patient care. All involved physicians should be aware of typical complications of immunosuppressive therapy. © Georg Thieme Verlag KG Stuttgart · New York.

  2. Vasculitis in children.

    Science.gov (United States)

    Eleftheriou, Despina; Batu, Ezgi Deniz; Ozen, Seza; Brogan, Paul A

    2015-04-01

    Primary systemic vasculitides of the young are relatively rare diseases, but are associated with significant morbidity and mortality, particularly if there is diagnostic delay. We provide an overview of paediatric vasculitides with emphasis on key differences in vasculitis presentation and management between children and adults. Significant advances in the field of paediatric vasculitis research include the development of classification criteria and disease outcome tools for paediatric disease; inclusion of paediatric patients in international multicentre randomized controlled trials of therapies in vasculitis; and development of rare disease trial designs for therapeutic trials of paediatric vasculitis. The continuation of unmet needs as well as the exploration of potential therapeutic avenues and considerations in the design of future trials are also discussed. © The Author 2014. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

  3. Study of DNA uptake locations in single E. coli cells

    Science.gov (United States)

    Xu, C. Shan; Meadow Anderson, L.; Yang, Haw

    2006-03-01

    Artificial gene transfer of bacteria, such as E. coli, has become the main stream technique in genetic engineering and molecular cell biology studies. In spite of the great improvements in transformation efficiency, some fundamental questions remained to be answered. For instance, what are the DNA uptake channels and how do they form and function under external stimuli? Furthermore, where are these channels located on the cell membrane? Here we report a study aimed at DNA uptake locations in the two widely used gene transformation techniques: electroporation and heat shock. A direct visualization of the settling location of single DNA molecules inside individual E. coli cells was obtained by fluorescence imaging and spectroscopy. Electroporation and heat shock exhibit two distinct characteristics of DNA uptake locations. A preferential distribution toward cell poles during electroporation is consistent with earlier experiments and previously proposed models. However, the result from heat shock is unanticipated in which the majority of DNA enters the cell near the center. Such observation suggests that uptake channels form preferentially where newly-synthesized membrane is located under cation and low temperature treatment

  4. Isolated pulmonary vasculitis: case report and literature review.

    Science.gov (United States)

    Riancho-Zarrabeitia, Leyre; Zurbano, Felipe; Gómez-Román, Javier; Martínez-Meñaca, Amaya; López, Marta; Hernández, Miguel A; Pina, Trinitario; González-Gay, Miguel A

    2015-04-01

    Single-organ vasculitis has been reported to affect the skin, kidneys, central nervous system, peripheral nerves, genitourinary tract, calf muscles, aorta, coronary arteries, retina, or gastrointestinal tract. However, isolated pulmonary vasculitis is a very rare entity. Our aims were to describe a case of localized pulmonary vasculitis affecting medium-sized vessels and review the literature. A patient with localized pulmonary vasculitis affecting medium-sized vessels that presented as pulmonary arterial hypertension is described. A MEDLINE database search of cases with localized pulmonary vasculitis was also conducted. A 30-year-old man presented with pulmonary hypertension due to isolated pulmonary medium-sized vessel vasculitis that was confirmed histologically. Initially he responded to corticosteroids and vasodilator treatment, but therapy eventually lost efficacy. Treatment with rituximab was not effective, and as the clinical situation worsened, lung transplant was performed. Isolated large pulmonary vessel disease, often related to Takayasu disease or giant cell arteritis, may present as pulmonary artery hypertension, thus mimicking chronic thromboembolic disease. Medium- and small-vessel pulmonary vasculitis usually develops in the context of a systemic disease. Some cases of isolated small-vessel vasculitis have been reported presenting as diffuse alveolar hemorrhage. In contrast, our case developed pulmonary artery hypertension secondary to medium-sized vessels vasculitis. To our knowledge, this is the first case of lung transplantation in isolated pulmonary vasculitis. Pulmonary isolated vasculitis is a rare cause of pulmonary hypertension but it must be taken into consideration after more common disorders are excluded. Copyright © 2014 Elsevier Inc. All rights reserved.

  5. Single Station System and Method of Locating Lightning Strikes

    Science.gov (United States)

    Medelius, Pedro J. (Inventor); Starr, Stanley O. (Inventor)

    2003-01-01

    An embodiment of the present invention uses a single detection system to approximate a location of lightning strikes. This system is triggered by a broadband RF detector and measures a time until the arrival of a leading edge of the thunder acoustic pulse. This time difference is used to determine a slant range R from the detector to the closest approach of the lightning. The azimuth and elevation are determined by an array of acoustic sensors. The leading edge of the thunder waveform is cross-correlated between the various acoustic sensors in the array to determine the difference in time of arrival, AT. A set of AT S is used to determine the direction of arrival, AZ and EL. The three estimated variables (R, AZ, EL) are used to locate a probable point of the lightning strike.

  6. Circumscribed cicatricial alopecia due to localized sarcoidal granulomas and single-organ granulomatous arteritis: a case report and systematic review of sarcoidal vasculitis.

    Science.gov (United States)

    Yazdani Abyaneh, Mohammad-Ali; Raghu, Preethi; Kircher, Kenneth; Kutzner, Heinz; Kortz, Alison; Carlson, John Andrew

    2015-10-01

    Vasculitis associated with sarcoid granulomas is an uncommon phenomenon. A 72-year-old female presented with an expanding region of circumscribed alopecia and scalp atrophy of 2 months duration. Biopsy showed non-caseating granulomas, dermal thinning, loss of follicles, fibrosis and muscular vessels disrupted by mixed lymphocyte, macrophage and giant-cell infiltrates. Affected vessels had loss and fragmentation of the elastic lamina, fibrous replacement of their walls and luminal stenosis (endarteritis obliterans). Dermal and vascular advential intralymphatic granulomas and lymphangiectases were found by D2-40 expression, suggesting lymphatic obstruction and poor antigen clearance. No evidence of a post-zoster eruption, systemic sarcoidosis or systemic giant-cell arteritis was found. Two years later, prednisone had halted - but not reversed - progression of her alopecia. Review of the literature showed two types of vasculitis associated with sarcoid granulomas: (i) acute, self-limited leukocytoclastic vasculitis and (ii) chronic granulomatous vasculitis (GV). Persistence of non-degradable material or antigen contributes to the pathogenesis of granulomatous inflammation. In this case, lymphatic obstruction probably impeded clearance of nonimmunologic and/or immunologic stimuli permitting and sustaining the development of sarcoid granulomas and sarcoid GV, ultimately causing scarring alopecia and cutaneous atrophy. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  7. Genetically distinct subsets within ANCA-associated vasculitis

    DEFF Research Database (Denmark)

    Lyons, Paul A; Rayner, Tim F; Trivedi, Sapna

    2012-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single...

  8. Rheumatoid vasculitis - Case report.

    Science.gov (United States)

    Pecly, Inah Maria Drummond; Ocampo, Juan Felipe; Ramirez, Guillermo Pandales; de Oliveira, Hedi Marinho de Melo Guedes; Saud, Claudia Guerra Murad; Arantes, Milton Dos Reis

    2015-01-01

    Rheumatoid arthritis (RA) is a chronic systemic inflammatory autoimmune disease and its main manifestation is persistent synovitis affecting peripheral joints symmetrically, In spite of its destructive potential, the evolution of RA is highly variable. Some patients may have only a short-term process oligoarticular with minimum lesion, while others suffers a polyarthritis evolving with progressive and continuous involvement of other organ systems such as skin, heart, lungs, muscles and blood vessels rarely leading to rheumatoid vasculitis. The aim of this study was to describe a case of rheumatoid vasculitis a rare and severe condition. Copyright © 2013 Elsevier Editora Ltda. All rights reserved.

  9. Approach to cutaneous vasculitides with special emphasis on small vessel vasculitis: histopathology and direct immunofluorescence.

    Science.gov (United States)

    Demirkesen, Cuyan

    2017-01-01

    The present review will focus on recent publications in cutaneous vasculitides. Some histopathological and clinical features, such as papillary dermal edema, perivascular C3 deposition, clinically evident edema, and lesions above the waist, may point out renal or gastrointestinal involvement in Henoch-Schönlein purpura (HSP). HSP associated with familial Mediterranean fever differs from typical isolated HSP by showing no deposits of IgA, much younger age, and location of the lesions on the face or the trunk. Single-organ cutaneous small vessel vasculitis is a more restricted entity than hypersensitivity vasculitis and HSP. Because cutaneous polyarteritis nodosa and macular lymphocytic arteritis share some clinicopathologic features, the question is raised whether they are not two different entities. Several histopathological features defining IgG4-related disease are found in granuloma faciale and erythema elevatum diutinum, two localized chronic cutaneous vasculitis; however, in a recent series no diagnostic criteria for IgG4-related disease was detected in them. When a patient presents with skin lesions, in which necrotizing or leukocytoclastic vascuitis is confirmed histologically, irrespective of the size of the affected vessel, the possibility of systemic vasculitis, an infection, medication, or a systemic disease such as systemic lupus erythematosus must be searched before reaching definitive diagnosis.

  10. Locating Local Earthquakes Using Single 3-Component Broadband Seismological Data

    Science.gov (United States)

    Das, S. B.; Mitra, S.

    2015-12-01

    We devised a technique to locate local earthquakes using single 3-component broadband seismograph and analyze the factors governing the accuracy of our result. The need for devising such a technique arises in regions of sparse seismic network. In state-of-the-art location algorithms, a minimum of three station recordings are required for obtaining well resolved locations. However, the problem arises when an event is recorded by less than three stations. This may be because of the following reasons: (a) down time of stations in a sparse network; (b) geographically isolated regions with limited logistic support to setup large network; (c) regions of insufficient economy for financing multi-station network and (d) poor signal-to-noise ratio for smaller events at most stations, except the one in its closest vicinity. Our technique provides a workable solution to the above problematic scenarios. However, our methodology is strongly dependent on the velocity model of the region. Our method uses a three step processing: (a) ascertain the back-azimuth of the event from the P-wave particle motion recorded on the horizontal components; (b) estimate the hypocentral distance using the S-P time; and (c) ascertain the emergent angle from the vertical and radial components. Once this is obtained, one can ray-trace through the 1-D velocity model to estimate the hypocentral location. We test our method on synthetic data, which produces results with 99% precision. With observed data, the accuracy of our results are very encouraging. The precision of our results depend on the signal-to-noise ratio (SNR) and choice of the right band-pass filter to isolate the P-wave signal. We used our method on minor aftershocks (3 transverse strike-slip structure within the Indian plate, which was observed from source mechanism study of the mainshock and larger aftershocks.

  11. Epidemiology of ANCA associated vasculitis

    Directory of Open Access Journals (Sweden)

    Wenche Koldingsnes

    2009-10-01

    Full Text Available ANCA associated vasculitis (AAV comprises three syndromes with systemic vasculitis (Wegener’s granulomatosis (WG, Churg Strauss syndrome (CSS and icroscopic polyangiitis (MPA, which all involve small and medium sized vessels and are associated with antibodies against cytoplasmatic antibodies in neutrophils (ANCA. Polyarteritis nodosa (PAN is included in this review as it also affects medium sized vessels, and has many clinical findings in common with the AAV.Since the recognition of ANCA, increasing data have become available on the epidemiology of these vasculitidis. WG constitutes half of the AAV and its prevalence has increased from 30/million in the late 1980’s in the USA to 160/million in this century in northern Europe. The prevalence for the whole group of primary systemic vasculitides is now 300/million in Sweden. The annual incidence of WG increased from 6.0/million to 14/million during the 1990’s in Tromsø, but it is unknown if this is a true increase or the result of an increased awareness of the diagnosis. For the whole group of AAV, the annual incidence in most more recent studies is relatively constant over time and by geographical location, ranging from 13 to 21/million. Nonetheless there are interesting differences in the prevalence of specific vasculitis between different geographical areas, as well as for sub specificities of ANCA.There seems to be a South-North gradient for WG and PR3-ANCA with high figures reported from northern Europe and southern New Zealand. In European studies WG is 90% PR3-ANCA positive. MPA which is predominantly MPO-ANCA associated are more frequent in the Mediterranean countries and also has an increasing gradient towards east-Asia, as almost all AAV in China and Japan are diagnosed as MPA, predominantly MPO-ANCA positive.There are also some ethnic and gender differences. WG is most prevalent among Caucasians in the USA and in people with European ancestors in Paris and in New Zealand, less

  12. Clinical Spectrum of Medium-Sized Vessel Vasculitis.

    Science.gov (United States)

    Alibaz-Oner, Fatma; Koster, Matthew J; Crowson, Cynthia S; Makol, Ashima; Ytterberg, Steven R; Salvarani, Carlo; Matteson, Eric L; Warrington, Kenneth J

    2017-06-01

    Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of medium-sized visceral vessels. However, cutaneous arteritis (CA) and gastrointestinal (GI) vasculitis are forms of single-organ vasculitis having indistinguishable histopathologic findings from PAN. The aim of this study was to evaluate and compare the clinical characteristics, treatment, and outcomes of patients with systemic PAN, CA, and GI vasculitis. Retrospective cohorts were assembled, consisting of patients with PAN, CA, and GI vasculitis between 1980 and 2014. The demographics, clinical characteristics, treatment, and outcomes of patients were abstracted from medical records. We included 48 patients with PAN, 41 patients with CA, and 19 patients with GI vasculitis. The disease of 1 patient evolved from CA to systemic PAN during the disease course. At diagnosis, 94% of patients with PAN, 93% of patients with CA, and 67% of patients with GI vasculitis were treated with glucocorticoids. Additional immunosuppressive agents were used in 67% of PAN, 37% of GI vasculitis, and 32% of CA cases. The 5-year cumulative relapse rate was 45.2% in CA, and only 9.6% in PAN during a followup of approximately 6 years. No deaths were observed in the CA group. The survival rate at 10 years was 66% in the PAN group and 61% in the GI vasculitis group. Systemic PAN, CA, and GI vasculitis take different clinical courses and therefore may be different diseases, rather than existing on a spectrum of the same disease. Progression of CA to systemic PAN is very rare. Relapse risk is low during followup in PAN. Patients with CA have a higher relapse rate than those with systemic PAN, possibly due to less use of immunosuppressive therapy in CA. © 2016, American College of Rheumatology.

  13. Biomarkers in Vasculitis

    Science.gov (United States)

    Monach, Paul A.

    2014-01-01

    Purpose of review Better biomarkers are needed for guiding management of patients with vasculitis. Large cohorts and technological advances had led to an increase in pre-clinical studies of potential biomarkers. Recent findings The most interesting markers described recently include a gene expression signature in CD8+ T cells that predicts tendency to relapse or remain relapse-free in ANCA-associated vasculitis, and a pair of urinary proteins that are elevated in Kawasaki disease but not other febrile illnesses. Both of these studies used “omics” technologies to generate and then test hypotheses. More conventional hypothesis-based studies have indicated that the following circulating proteins have potential to improve upon clinically available tests: pentraxin-3 in giant cell arteritis and Takayasu’s arteritis; von Willebrand factor antigen in childhood central nervous system vasculitis; eotaxin-3 and other markers related to eosinophils or Th2 immune responses in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome); and MMP-3, TIMP-1, and CXCL13 in ANCA-associated vasculitis. Summary New markers testable in blood and urine have the potential to assist with diagnosis, staging, assessment of current disease activity, and prognosis. However, the standards for clinical usefulness, in particular the demonstration of either very high sensitivity or very high specificity, have yet to be met for clinically relevant outcomes. PMID:24257367

  14. A single geophone to locate seismic events on Mars

    Science.gov (United States)

    Roques, Aurélien; Berenguer, Jean-Luc; Bozdag, Ebru

    2016-04-01

    Knowing the structure of Mars is a key point in understanding the formation of Earth-like planets as plate tectonics and erosion have erased the original suface of the Earth formation. Installing a seismometer on Mars surface makes it possible to identify its structure. An important step in the identification of the structure of a planet is the epicenter's location of a seismic source, typically a meteoric impact or an earthquake. On Earth, the classical way of locating epicenters is triangulation, which requires at least 3 stations. The Mars InSight Project plans to set a single station with 3 components. We propose a software to locate seismic sources on Mars thanks to the 3-components simulated data of an earthquake given by Geoazur (Nice Sophia-Antipolis University, CNRS) researchers. Instrumental response of a sensor is crucial for data interpretation. We study the oscillations of geophone in several situations so as to awaken students to the meaning of damping in second order modeling. In physics, car shock absorbers are often used to illustrate the principle of damping but rarely in practical experiments. We propose the use of a simple seismometer (a string with a mass and a damper) that allows changing several parameters (inductive damping, temperature and pressure) so as to see the effects of these parameters on the impulse response and, in particular, on the damping coefficient. In a second step, we illustrate the effect of damping on a seismogram with the difficulty of identifying and interpreting the different phase arrival times with low damping.

  15. Systemic vasculitis and the gut.

    Science.gov (United States)

    Hatemi, Ibrahim; Hatemi, Gulen; Çelik, Aykut F

    2017-01-01

    Gastrointestinal system can be involved in primary and secondary vasculitides. The recent data regarding the pathophysiology, clinical findings, diagnosis, management, and outcome of gastrointestinal involvement in different types of vasculitis are reviewed. Diagnosis of gastrointestinal vasculitis may be difficult and relies mostly on imaging, because biopsy samples are hard to obtain and superficial mucosal biopsies have a low yield. There are conflicting reports on the association of antineutrophilic cytoplasmic antibodies (ANCA) type with the frequency of gastrointestinal involvement in ANCA-associated vasculitis. Pancreatitis is a rare but serious complication of ANCA-associated vasculitis. Terminal ileitis may be observed in immunoglobulin A vasculitis and can be hard to distinguish from Crohn's disease. High fecal calprotectin levels can indicate active gastrointestinal involvement in both immunoglobulin A vasculitis and Behçet's syndrome. Refractory gastrointestinal involvement in Behçet's syndrome can be treated with thalidomide and/or TNF-α antagonists. The outcome of mesenteric vasculitis in systemic lupus erythematosus can be improved with high-dose glucocorticoids and cyclophosphamide or rituximab. Gastrointestinal system can be commonly involved in immunoglobulin A vasculitis, ANCA-associated vasculitis, polyarteritis nodosa, and Behçet's syndrome and can be an important cause of morbidity and mortality. Treatment depends on the type of vasculitis and is usually with high-dose corticosteroids and immunosuppressives.

  16. Vasculitis renal en Colombia / Renal vasculitis in Colombia

    OpenAIRE

    Ramirez Rubio , Fabian Alberto

    2010-01-01

    Las vasculitis se caracterizan por la inflamación de las paredes vasculares y el riñón puede estar afectado de varias formas. La información epidemiológica en Colombia de la vasculitis renal es escasa. Objetivos: Identificar las causas de vasculitis renal en pacientes Colombianos. Métodos: Varios centros de Reumatología en Colombia, liderado por la Universidad Nacional de Colombia, recolectaron desde el año 2000, pacientes con diagnóstico de vasculitis primaria. Para el diagnóstico, cada c...

  17. Isolated vasculitis of the CNS

    International Nuclear Information System (INIS)

    Block, F.; Reith, W.

    2000-01-01

    Vasculitis is a rare cause for disease of the CNS. The isolated vasculitis of the CNS is restricted to the CNS whereas other forms of vasculitis affect various organs including the CNS. Headache, encephalopathy, focal deficits and epileptic seizures are the major symptoms suggestive for vasculitis. One major criterion of the isolated vasculitis of the CNS is the lack of evidence for other vasculitis forms or for pathology of other organs. Angiography displays multifocal segmental stenosis of intracranial vessels. MRI demonstrates multiple lesions which in part show enhancement after gadolinium. A definite diagnosis can only be made on the grounds of biopsy from leptomeninges and parenchyma. Therapy consists of corticosteroids and cyclophosphamid. (orig.) [de

  18. Cerebral MR imaging in vasculitis

    International Nuclear Information System (INIS)

    Ott, D.; Geissler, A.; Peter, H.H.

    1990-01-01

    This paper evaluates the diagnostic sensitivity and specificity of MR imaging for the detection of cerebral involvement in vasculitis. Seventy-six patient with proved systematic vasculitis and two with isolated cerebral vasculitis were examined. Half of them had central nervous system symptoms. T2-weighted sequences were performed at 2 T in most cases. All but two symptomatic and one-third of the asymptomatic patients showed pathologic changes in the white matter. The distribution and shape of lesions depend on the caliber of the involved vessel. In small-vessel vasculitis, the distribution was mostly subcortical, the average size was 0.5--10 mm, and lesions were predominantly round

  19. Vasculitis of the mesenteric circulation.

    Science.gov (United States)

    Koster, Matthew J; Warrington, Kenneth J

    2017-02-01

    Vasculitis of the mesenteric circulation is an uncommon but life-threatening manifestation of systemic vasculitis. Initial symptoms are frequently non-specific and therefore patients often present to primary care physicians and gastroenterologists with abdominal pain or gastrointestinal bleeding. Given the severity of the conditions associated with mesenteric vasculitis, it is imperative to appropriately diagnose and initiate treatment of suspected cases. This review will focus on diseases commonly associated with vasculitis of the mesenteric vessels. Imaging characteristics and clinical features assisting in diagnosis as well as initial approaches to treatment are emphasized. Copyright © 2016 Elsevier Ltd. All rights reserved.

  20. The Spectrum of Paraneoplastic Cutaneous Vasculitis in a Defined Population

    Science.gov (United States)

    Loricera, Javier; Calvo-Río, Vanesa; Ortiz-Sanjuán, Francisco; González-López, Marcos A.; Fernández-Llaca, Hector; Rueda-Gotor, Javier; Gonzalez-Vela, Maria C.; Alvarez, Lino; Mata, Cristina; González-Lamuño, Domingo; Martínez-Taboada, Victor M.; González-Gay, Miguel A.; Blanco, Ricardo

    2013-01-01

    Abstract Cutaneous vasculitis may be associated with malignancies, and may behave as a paraneoplastic syndrome. This association has been reported in a variable proportion of patients depending on population selection. We conducted the current study to assess the frequency, clinical features, treatment, and outcome of paraneoplastic vasculitis in a large unselected series of 766 patients with cutaneous vasculitis diagnosed at a single university hospital. Sixteen patients (10 men and 6 women; mean age ± standard deviation, 67.94 ± 14.20 yr; range, 40–85 yr) presenting with cutaneous vasculitis were ultimately diagnosed as having an underlying malignancy. They constituted 3.80% of the 421 adult patients. There were 9 hematologic and 7 solid underlying malignancies. Skin lesions were the initial clinical presentation in all of them, and the median interval from the onset of cutaneous vasculitis to the diagnosis of the malignancy was 17 days (range, 8–50 d). The most frequent skin lesions were palpable purpura (15 patients). Other clinical manifestations included constitutional syndrome (10 patients) and arthralgia and/or arthritis (4 cases). Hematologic cytopenias (11 cases) as well as immature peripheral blood cells (6 cases) were frequently observed in the full blood cell count, especially in those with vasculitis associated with hematologic malignancies. Specific treatment for vasculitis was prescribed in 10 patients; nonsteroidal antiinflammatory drugs (4 patients), corticosteroids (3 patients), chloroquine (1 patient), antihistamines (1 patient), and cyclophosphamide (1 patient). Ten patients died due to the malignancy and 6 patients recovered following malignancy therapy. Patients with paraneoplastic vasculitis were older, more frequently had constitutional syndrome, and less frequently had organ damage due to the vasculitis than the remaining patients with cutaneous vasculitis. In summary, cutaneous paraneoplastic vasculitis is an entity not uncommonly

  1. The Pharmacogenomic Association of Fc gamma Receptors and Cytochrome P450 Enzymes With Response to Rituximab or Cyclophosphamide Treatment in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

    NARCIS (Netherlands)

    Cartin-Ceba, Rodrigo; Indrakanti, Divya; Specks, Ulrich; Stone, John H.; Hoffman, Gary S.; Kallenberg, Cees G. M.; Langford, Carol A.; Merkel, Peter A.; Spiera, Robert F.; Monach, Paul A.; St. Clair, E. William; Seo, Philip; Tchao, Nadia K.; Ytterberg, Steven R.; Brunetta, Paul G.; Song, Huijuan; Birmingham, Dan; Rovin, Brad H.; Grp, RAVE-Immune Tolerance Network Res

    Objective The Rituximab in ANCA-Associated Vasculitis (RAVE) trial compared rituximab to cyclophosphamide as induction therapy for the treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. We undertook the current study to determine whether known single-nucleotide

  2. Vasculitis in the autoinflammatory diseases.

    Science.gov (United States)

    Peleg, Hagit; Ben-Chetrit, Eldad

    2017-01-01

    This article addresses the prevalence and relationship between autoinflammatory diseases and vasculitis. Autoimmune diseases (AIDs) are a group of syndromes characterized by episodes of unprovoked inflammation due to dysregulation of the innate immune system. Despite the common occurrence of rashes and other skin lesions in these diseases, vasculitis is reported in only a few. On the other hand, neutrophilic dermatoses are more prevalent. Large vessel vasculitis is reported in patients with Behcet's and Blau's syndromes. Small and medium size vasculitides are reported in familial Mediterranean fever mainly as Henoch-Schonlein purpura and polyarteritis nodosa, respectively. It is rarely described in hyper IgD with periodic fever syndrome, cryopyrin associated periodic syndromes, TNF receptor-associated periodic syndrome, deficiency of interleukin-1 receptor antagonist and pyoderma gangrenosum and acne syndrome. In most AID where bones and skin are mainly involved (CRMO, Majeed syndrome, Cherubism and DITRA) - vasculitis has not been described at all. In AID small vessel vasculitis affects mainly the skin with no involvement of internal organs. In AID, neutrophilic dermatoses are more common and prominent than vasculitis. This may reflect a minor role for interleukin-1 in the pathogenesis of vasculitis. The rarity of vasculitis in AID suggests that in most reported cases its occurrence has been probably coincidental rather than being an integral feature of the disease.

  3. Infections and vasculitis.

    Science.gov (United States)

    Thomas, Konstantinos; Vassilopoulos, Dimitrios

    2017-01-01

    To review recent evidence for infection rates in patients with systemic vasculitides, the role of specific infectious agents in the pathogenesis of vasculitis and recent breakthroughs in the treatment of virus-associated vasculitides. In well designed recent studies, infections were found to be common during the first 6-12 months in patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) and giant cell arteritis (GCA) and to contribute significantly to increased mortality during this period. New therapeutic schemes with lower cyclophosphamide doses and shorter corticosteroid courses were associated with decreased infectious rates in elderly patients with AAV whereas a prednisone dose greater than 10 mg/day at the end of the first year were associated with increased infectious-related mortality in patients with GCA. Recently, a potential role for varicella zoster virus in GCA pathogenesis has been proposed but more data are needed in order to establish a causal relationship. Finally, preliminary data show excellent short-term efficacy and safety of the new, interferon-free, oral antiviral agents in the treatment of hepatitis C virus-associated cryoglobulinemic vasculitis. Infections continue to be one of the main causes of mortality in patients with systemic vasculitides, emphasizing the need for safer immunosuppressive therapies and appropriate prophylaxis.

  4. Systemic vasculitis and the lung.

    Science.gov (United States)

    Talarico, Rosaria; Barsotti, Simone; Elefante, Elena; Baldini, Chiara; Tani, Chiara; Mosca, Marta

    2017-01-01

    The purpose of this review is to provide a critical analysis of the recent literature on this topic, with particular focus on the most relevant studies published over the last year. Many studies are published every year on the diagnosis, pathogenesis and treatment of pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). The main subjects covered by this article are the pathogenesis, diagnosis and clinical aspects of lung involvement in ANCA-associated vasculitis and non-ANCA-associated vasculitis. Lung involvement is a common feature in systemic vasculitis. The lungs are one of the most frequently involved organs in systemic vasculitis. In order to provide an update on the recent advances in the pathogenesis, clinical features and novel treatments of lung involvement in systemic vasculitis, a systematic MedLine search has been performed.Most of the data analyzed have confirmed that lung involvement seems to develop more frequently in patients with myeloperoxidase-ANCA-positive AAV, mainly in those with a diagnosis of microscopic polyangiitis (MPA), compared with patients with proteinase 3 ANCA-positive AAV. Moreover, among non-ANCA-associated vasculitis lung involvement may represent a worrying complication of the disease, mainly when associated with vascular involvement.

  5. Cerebral Vasculitis Complicating Pneumococcal Meningitis

    Directory of Open Access Journals (Sweden)

    Ahmed Khedher

    2018-03-01

    Full Text Available Introduction: Cerebral vasculitis is an uncommon life-threatening complication of community-acquired bacterial meningitis. Patient and methods: We report the case of a 64-year-old woman with pneumococcal meningitis who developed parainfectious vasculitis causing ischaemic brain damage. Cerebral magnetic resonance imaging (MRI confirmed the diagnosis. Clinical and radiological recovery after delayed addition of corticosteroid was achieved. Discussion: This report shows that the onset of neurological deficits following pneumococcal meningitis can be caused by cerebral vasculitis. Underdosing with antibiotics and delayed adjunctive dexamethasone seem to favour this complication. There are no guidelines for treatment but high doses of steroids led to resolution in this case.

  6. The last classification of vasculitis

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.

    2008-01-01

    Systemic vasculitides are a group of diverse conditions characterized by inflammation of the blood vessels. To obtain homogeneity in clinical characteristics, prognosis, and response to treatment, patients with vasculitis should be classified into defined disease categories. Many classification

  7. Differential diagnosis of retinal vasculitis.

    Science.gov (United States)

    Abu El-Asrar, Ahmed M; Herbort, Carl P; Tabbara, Khalid F

    2009-10-01

    Retinal vaculitis is a sight-threatening inflammatory eye condition that involves the retinal vessels. Detection of retinal vasculitis is made clinically, and confirmed with the help of fundus fluorescein angiography. Active vascular disease is characterized by exudates around retinal vessels resulting in white sheathing or cuffing of the affected vessels. In this review, a practical approach to the diagnosis of retinal vasculitis is discussed based on ophthalmoscopic and fundus fluorescein angiographic findings.

  8. Genetically Distinct Subsets within ANCA-Associated Vasculitis

    Science.gov (United States)

    Lyons, Paul A.; Rayner, Tim F.; Trivedi, Sapna; Holle, Julia U.; Watts, Richard A.; Jayne, David R.W.; Baslund, Bo; Brenchley, Paul; Bruchfeld, Annette; Chaudhry, Afzal N.; Tervaert, Jan Willem Cohen; Deloukas, Panos; Feighery, Conleth; Gross, Wolfgang L.; Guillevin, Loic; Gunnarsson, Iva; P, Lorraine Harper M.R.C; Hrušková, Zdenka; Little, Mark A.; Martorana, Davide; Neumann, Thomas; Ohlsson, Sophie; Padmanabhan, Sandosh; Pusey, Charles D.; Salama, Alan D.; Sanders, Jan-Stephan F.; Savage, Caroline O.; Segelmark, Mårten; Stegeman, Coen A.; Tesař, Vladimir; Vaglio, Augusto; Wieczorek, Stefan; Wilde, Benjamin; Zwerina, Jochen; Rees, Andrew J.; Clayton, David G.; Smith, Kenneth G.C.

    2013-01-01

    BACKGROUND Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single disease entity and what role ANCA plays in its pathogenesis. We investigated its genetic basis. METHODS A genomewide association study was performed in a discovery cohort of 1233 U.K. patients with ANCA-associated vasculitis and 5884 controls and was replicated in 1454 Northern European case patients and 1666 controls. Quality control, population stratification, and statistical analyses were performed according to standard criteria. RESULTS We found both major-histocompatibility-complex (MHC) and non-MHC associations with ANCA-associated vasculitis and also that granulomatosis with polyangiitis and microscopic polyangiitis were genetically distinct. The strongest genetic associations were with the antigenic specificity of ANCA, not with the clinical syndrome. Anti–proteinase 3 ANCA was associated with HLA-DP and the genes encoding α1-antitrypsin (SERPINA1) and proteinase 3 (PRTN3) (P = 6.2×10−89, P = 5.6×10−12, and P = 2.6×10−7, respectively). Anti–myeloperoxidase ANCA was associated with HLA-DQ (P = 2.1×10−8). CONCLUSIONS This study confirms that the pathogenesis of ANCA-associated vasculitis has a genetic component, shows genetic distinctions between granulomatosis with polyangiitis and microscopic polyangiitis that are associated with ANCA specificity, and suggests that the response against the autoantigen proteinase 3 is a central pathogenic feature of proteinase 3 ANCA–associated vasculitis. These data provide preliminary support for the concept that proteinase 3 ANCA–associated vasculitis and myeloperoxidase ANCA–associated vasculitis are distinct autoimmune syndromes. (Funded by the British Heart Foundation and others.) PMID

  9. Cryofibrinogenemia: a marker of severity of cryoglobulinemic vasculitis.

    Science.gov (United States)

    Michaud, Martin; Moulis, Guillaume; Puissant, Bénédicte; Balardy, Laurent; Huart, Antoine; Gaches, Francis; Cougoul, Pierre; Arlet, Philippe; Blancher, Antoine; Pourrat, Jacques; Sailler, Laurent

    2015-08-01

    Cryofibrinogenemia is frequently associated with cryoglobulinemia. The aim of this study was to determine the characteristics associated with the presence of cryofibrinogenemia in patients with cryoglobulinemic vasculitis. This was a single-center retrospective study that included patients with cryoglobulinemic vasculitis who were tested for cryofibrinogen at a tertiary referral center between January 1, 2011 and December 31, 2012. Twenty-nine patients fulfilled the CryoVas (cryoglobulinemic vasculitis) Survey criteria for cryoglobulinemic vasculitis. Eighteen patients had a detectable cryofibrinogen (CF-positive) and 11 had no detectable cryofibrinogen (CF-negative). Median cryoglobulin levels were 89 ± 129 mg/L in the CF-positive group and 68 ± 82 mg/L in the CF-negative group (P = .32). Median cryofibrinogen level was 70 ± 174 mg/L. Clinical manifestations were similar in both groups. Cancers and hematological disorders were more frequent among CF-positive patients (39% vs 0%, P = .026). Levels of alpha-1 and alpha-2 globulinemia were higher in the CF-positive group. Cryofibrinogenemia ≥ 100 mg/L was associated with cryoglobulinemic vasculitis (odds ratio [OR] 2.86; 95% confidence interval [CI], 1.06-7.73) in cryoglobulinemic patients. Presence of cryofibrinogenemia was associated with use of corticosteroids, immunosuppressants, or plasmapheresis in cryoglobulinemic vasculitis patients (OR 22.7; 95% CI, 2.02-256.44). Our results strongly suggest that presence of cryofibrinogenemia is associated with a more severe phenotype among patients with cryoglobulinemic vasculitis. Copyright © 2015 Elsevier Inc. All rights reserved.

  10. Application of dead-reckoning in the single-base station location and tracking method

    Science.gov (United States)

    Zhang, Ruyun; Fan, Dandan; Xu, Mingyan; Chen, Ming

    2007-11-01

    A location and tracking method utilizing Dead-Reckoning using single base station in cellular networks is proposed in this paper. Extended Kalman Filter (EKF) and the location method utilizing the AOA and the variance ratio of AOA are introduced to smooth the measurement error. The simulation result is illustrated to explain the location and tracking efficiency of the proposed method.

  11. The ANCA Vasculitis Questionnaire (AAV-PRO©)

    Science.gov (United States)

    2017-05-01

    Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) (EGPA); Churg-Strauss Syndrome (CSS); Granulomatosis With Polyangiitis (Wegener's) (GPA); Wegener Granulomatosis (WG); Microscopic Polyangiitis (MPA); ANCA-Associated Vasculitis (AAV); Vasculitis

  12. Vasculitis associated with connective tissue diseases.

    Science.gov (United States)

    Cozzani, E; Gasparini, G; Papini, M; Burlando, M; Drago, F; Parodi, A

    2015-04-01

    Vasculitis in connective tissue disease (CTD) is quite rare, it is reported in approximately 10% of patients with CTD; systemic lupus erythematosus (SLE) shows the highest association rate. Vessels of any size may be involved, but mainly small vessels vasculitis is reported. At present the classification of these vasculitis is unsatisfactory. According to the 2012 revised International Chapel Hill Consensus Conference, vasculitides secondary to CTD are a well identified entity and are classified under the category of "vasculitis associated with systemic disease". However only lupus vasculitis and rheumatoid vasculitis are explicitly listed, while the remaining are generically included under the heading "others". Petechiae, purpura, gangrene and ulcers are the most frequent cutaneous manifestations that should investigated in order to rule out potentially dangerous systemic involvement, especially if cryoglobulinemic or necrotizing vasculitis are suspected. This review will focus on the cutaneous involvement in CTD associated vasculitis.

  13. Solving Minimal Covering Location Problems with Single and Multiple Node Coverage

    Directory of Open Access Journals (Sweden)

    Darko DRAKULIĆ

    2016-12-01

    Full Text Available Location science represents a very attractiveresearch field in combinatorial optimization and it is in expansion in last five decades. The main objective of location problems is determining the best position for facilities in a given set of nodes.Location science includes techniques for modelling problemsand methods for solving them. This paper presents results of solving two types of minimal covering location problems, with single and multiple node coverage, by using CPLEX optimizer and Particle Swarm Optimization method.

  14. Genetically distinct subsets within ANCA-associated vasculitis.

    LENUS (Irish Health Repository)

    Lyons, Paul A

    2012-07-19

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener\\'s granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single disease entity and what role ANCA plays in its pathogenesis. We investigated its genetic basis.

  15. Cerebral vasculitis associated with cocaine abuse

    International Nuclear Information System (INIS)

    Kaye, B.R.; Fainstat, M.

    1987-01-01

    A case of cerebral vasculitis in a previously healthy 22-year-old man with a history of cocaine abuse is described. Cerebral angiograms showed evidence of vasculitis. A search for possible causes other than cocaine produced no results. The authors include cocaine with methamphetamines, heroin, and ephedrine as illicit drugs that can cause cerebral vasculitis

  16. Co-Located Collaborative Learning Video Game with Single Display Groupware

    Science.gov (United States)

    Infante, Cristian; Weitz, Juan; Reyes, Tomas; Nussbaum, Miguel; Gomez, Florencia; Radovic, Darinka

    2010-01-01

    Role Game is a co-located CSCL video game played by three students sitting at one machine sharing a single screen, each with their own input device. Inspired by video console games, Role Game enables students to learn by doing, acquiring social abilities and mastering subject matter in a context of co-located collaboration. After describing the…

  17. Recent advances in childhood vasculitis.

    Science.gov (United States)

    Ozen, Seza; Acar-Ozen, Nazire Pinar

    2017-09-01

    The review aims to summarize the recent findings in vasculitis that may have an impact in our understanding or management of these diseases. We are learning more about monogenic diseases that closely mimic the pediatric vasculitides. Deficiency of adenosine deaminase 2 can present with a polyarteritis nodosa (PAN)-like picture and should be included in the differential of all pediatric cases of PAN with a family history or in cases with early stroke, or in cases resistant to conventional therapy. Mutations in tumor necrosis factor α-induced protein 3 results in a disease that can present as Behçet disease called haploinsufficiency of A20. In fact, these patients would also fulfill the existing criteria for PAN and Behçet disease, respectively. Additional advances in Behçet disease pathogenesis come from a large genetic study of Turkish Behçet disease using data obtained from genotyping using the Immunochip. This confirmed the HLA-B-51 locus as the most significant association and identified new risk loci. Large Iranian and Japanese cohorts were used as replication cohorts. Best treatment of pediatric vasculitis remains a challenge as we continue to lack controlled studies. There are new reports in treatment on Henoch-Schönlein purpura/Immunoglobulin A vasculitis which is one of our most frequent childhood vasculitides. Small series of new treatments for central nervous system vasculitis and Takayasu disease will also be summarized. Diagnostic criteria have been reassessed in pediatric Behçet disease as well as adult and childhood forms of antineutrophil cytoplasmic antibodies-positive vasculitis. The new pathways defined in monogenic diseases may help us better understand the pathogenesis and may help us design more targeted therapy. Although pediatric cases are being increasingly recognized, the relative rarity of the diseases presents an obstacle for studies. Thus, we can reach conclusive results for their management through multicenter studies only.

  18. Feasibility of single-incision laparoscopic surgery for appendicitis in abnormal anatomical locations: A single surgeon′s initial experience

    Directory of Open Access Journals (Sweden)

    Sanoop K Zachariah

    2013-01-01

    Full Text Available Background: Single-incision laparoscopic surgery is considered as a more technically demanding procedure than the standard laparoscopic surgery. Based on an initial and early experience, single-incision laparoscopic appendectomy (LA was found to be technically advantageous for dealing with appendicitis in unusual anatomical locations. This study aims to highlight the technical advantages of single-incision laparoscopic surgery in dealing with the abnormally located appendixes and furthermore report a case of acute appendicitis occurring in a sub-gastric position, which is probably the first such case to be reported in English literature. Materials and Methods: A retrospective analysis of the first 10 cases of single-incision LA which were performed by a single surgeon is presented here. Results: There were seven females and three males. The mean age of the patients was 30.6 (range 18-52 years, mean BMI was 22.7 (range 17-28 kg/m 2 and the mean operative time was 85.5 (range 45-150 min. The mean postoperative stay was 3.6 (range 1-7 days. The commonest position of the appendix was retro-caecal (50% followed by pelvic (30%. In three cases the appendix was found to be in abnormal locations namely sub-hepatic, sub-gastric and deep pelvic or para-vesical or para-rectal. All these cases could be managed with this technique without any conversions Conclusion: Single-incision laparoscopic surgery appears to be a feasible and safe technique for dealing with appendicitis in rare anatomical locations. Appendectomy may be a suitable procedure for the initial training in single-incision laparoscopic surgery.

  19. Epitope analysis of anti-myeloperoxidase antibodies in patients with ANCA-associated vasculitis.

    Directory of Open Access Journals (Sweden)

    Shen-Ju Gou

    Full Text Available OBJECTIVE: Increasing evidences have suggested the pathogenic role of anti-neutrophil cytoplasmic antibodies (ANCA directing myeloperoxidase (MPO in ANCA-associated vasculitis (AAV. The current study aimed to analyze the association between the linear epitopes of MPO-ANCA and clinicopathological features of patients with AAV. METHODS: Six recombinant linear fragments, covering the whole length amino acid sequence of a single chain of MPO, were produced from E.coli. Sera from 77 patients with AAV were collected at presentation. 13 out of the 77 patients had co-existence of serum anti-GBM antibodies. Ten patients also had sequential sera during follow up. The epitope specificities were detected by enzyme-linked immunosorbent assay using the recombinant fragments as solid phase ligands. RESULTS: Sera from 45 of the 77 (58.4% patients with AAV showed a positive reaction to one or more linear fragments of the MPO chain. The Birmingham Vasculitis Activity Scores and the sera creatinine were significantly higher in patients with positive binding to the light chain fragment than that in patients without the binding. The epitopes recognized by MPO-ANCA from patients with co-existence of serum anti-GBM antibodies were mainly located in the N-terminus of the heavy chain. In 5 out of the 6 patients, whose sera in relapse recognize linear fragments, the reactivity to linear fragments in relapse was similar to that of initial onset. CONCLUSION: The epitope specificities of MPO-ANCA were associated with disease activity and some clinicopathological features in patients with ANCA-associated vasculitis.

  20. Meningococal Septicemia with Cutaneous Vasculitis

    Directory of Open Access Journals (Sweden)

    Radha Mittal

    1989-01-01

    Full Text Available Two cases-had meningococcal septicemia with vasculitis of skin. Out 9 year old female developed generalised ,asymptomatic, purpuric atrophic plaques on the limbs, trunk and face on the fourth day of high fever. The second patient was 20 year old male who developed purplish plaques. Nodules, and bullae mainly on the extensor surface of the hands and feet on the tenth day, of high fever. Bullae had clear fluid and bluish-black peripheral rim

  1. Scabietic vasculitis: Report of 2 cases.

    Science.gov (United States)

    Clevy, C; Brajon, D; Combes, E; Benzaquen, M; Dales, J-P; Koeppel, M-C; Berbis, P

    2017-05-01

    The infectious causes of cutaneous vasculitis are well known and include streptococcal infections among others. Cases resulting from parasitic infection are less frequent. Scabies, which is currently on the increase, has only been reported in a few isolated cases. Herein, we report two noteworthy cases of profuse scabies complicated by cutaneous vasculitis. Case 1: a 90-year-old woman, residing in a nursing home, was admitted to our dermatology department complaining of pruritus, present for one month, predominantly on the inside of the thighs and on the buttocks, associated with purpuric lesions on the lower limbs. A skin biopsy revealed leukocytoclastic vasculitis. A diagnosis of scabies was based on severe pruritus and hypereosinophilia and was confirmed by microscopic examination of the parasitology sample and the skin biopsy sample. Despite thorough investigation, no other cause of vasculitis could be found. Complete regression of the skin lesions was achieved with scabies treatment only, without any specific treatment for the vasculitis. Case 2: a 74-year-old man, living in a nursing home, was hospitalized for purpuric papules on the lower limbs, present for one month. Physical examination revealed linear patterns in the interdigital spaces associated with scabies evident on dermoscopic examination. The skin biopsy revealed signs of vasculitis. As in our first case, no aetiology of vasculitis was found and a favorable outcome was achieved by means of scabies treatment alone with no specific treatment for vasculitis. Both of our patients presented scabies and vasculitis. In view of the absence of other causes of vasculitis and of the complete regression of lesions due to vasculitis without recurrence achieved with the scabies treatment alone, a diagnosis was made of scabietic vasculitis, probably as a result of cutaneous hypersensitivity reaction to humeral mediators. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  2. Pregnancy Outcomes Among Patients With Vasculitis

    Science.gov (United States)

    Clowse, Megan E. B.; Richeson, Rachel L.; Pieper, Carl; Merkel, Peter A.

    2015-01-01

    Objective Pregnancy outcomes of patients with vasculitis are unknown, but are of great concern to patients and physicians. Through an online survey, this study assessed pregnancy outcomes among patients with vasculitis. Methods Participants in the Vasculitis Clinical Research Consortium Patient Contact Registry were invited to respond to an anonymous, internet-based survey that included questions about pregnancy outcomes, the timing of pregnancy relative to a diagnosis of vasculitis, and medication use. Results A total of 350 women and 113 men completed the survey. After a diagnosis of vasculitis, 74 pregnancies were reported by women and 18 conceptions were reported by men. The rate of pregnancy loss was higher among women who conceived after a diagnosis of vasculitis compared to those who conceived prior to diagnosis (33.8% versus 22.4%; P = 0.04). Among women, the rate of preterm births increased significantly for pregnancies conceived after a diagnosis of vasculitis relative to those conceived before diagnosis (23.3% versus 11.4%; P = 0.03). Only 18% of women reported worsening of vasculitis during pregnancy, but those who experienced increased vasculitis activity were more likely to deliver preterm. Exposure to cyclophosphamide or prednisone did not appear to impact pregnancy outcomes; however, the number of pregnancies among women taking these medications was small. Among the pregnancies conceived by men with vasculitis, the timing of diagnosis had no significant effect on the rate of pregnancy loss. Conclusion Women who conceived after a diagnosis of vasculitis had a higher rate of pregnancy loss than those who conceived prior to diagnosis. Vasculitis did not worsen during the majority of pregnancies conceived after diagnosis. PMID:23401494

  3. [Primary childhood vasculitis new classification criteria

    DEFF Research Database (Denmark)

    Herlin, T.; Nielsen, Susan

    2008-01-01

    Primary vasculitis is seen in both adults and children, but some of the diseases like Kawasaki disease occur primarily in children. The Chapel Hill Classification Criteria for primary vasculitis refers to the size of vessels but has not been validated in children. Recently, new criteria for the c......Primary vasculitis is seen in both adults and children, but some of the diseases like Kawasaki disease occur primarily in children. The Chapel Hill Classification Criteria for primary vasculitis refers to the size of vessels but has not been validated in children. Recently, new criteria...

  4. Educational Needs of Patients With Systemic Vasculitis

    Science.gov (United States)

    2014-07-11

    Behcet's Disease; Churg-Strauss Syndrome; Vasculitis, Central Nervous System; Giant Cell Arteritis; Wegener Granulomatosis; Henoch-Schoenlein Purpura; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu's Arteritis

  5. Goodpasture's syndrome associated with pulmonary eosinophilic vasculitis.

    Science.gov (United States)

    Komadina, K H; Houk, R W; Vicks, S L; Desrosier, K F; Ridley, D J; Boswell, R N

    1988-08-01

    Lung hemorrhage and antiglomerular basement membrane (anti-GBM) antibody mediated nephritis define Goodpasture's syndrome. We present the case of a 19-year-old Caucasian woman with unique clinical findings of Goodpasture's syndrome. Our patient initially presented with leukocytoclastic vasculitis of the skin followed by the development of nephritis and lung hemorrhage. An open lung biopsy done prior to diagnosing anti-GBM antibody disease demonstrated an intense eosinophilic vasculitis. Skin vasculitis has only been rarely reported, and to our knowledge this is the first reported case of pulmonary eosinophilic vasculitis associated with Goodpasture's syndrome.

  6. Outcome of Renal Transplant in Recipients With Vasculitis.

    Science.gov (United States)

    Barbouch, Samia; Hajji, Meriam; Aoudia, Raja; Ounissi, Monther; Zammouri, Asma; Goucha, Rym; Ben Hamida, Fathi; Bacha, Mohammed Mongi; Abderrahim, Ezzedine; Ben Abdallah, Taieb

    2017-02-01

    End-stage renal disease develops in a high percentage of patients with vasculitis, in whom kidney transplant has become a therapeutic option. However, limited data are available on the prognosis and outcomes after kidney transplant in these patients. We aimed to compare the long-term graft survival and graft function in 8 renal transplant recipients with vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis, Goodpasture syndrome, and Henoch-Schonlein purpura) with the other kidney recipients at a single center. We conducted a retrospective study of patients followed for chronic renal failure associated with vasculitis before renal transplant. We excluded patients with no biopsy-proven nephropathy. There was no difference in the occurrence of metabolic and cardiovascular complications in our case group compared with the other graft recipients. Infections were frequent and included cytomegalovirus and urinary tract infection. The rates of bacterial and viral infection were equivalent in our population. The incidence of allograft loss was estimated at 1.8%, less than that seen in our entire transplant population. The presence of vasculitis was not significantly related to renal failure (P = .07). Extrarenal relapse occurred in 1 patient with microscopic polyangiitis. Antineutrophil cytoplasmic antibody levels in patients with granulomatosis with polyangiitis and microscopic polyangiitis did not seem to influence the renal outcome (P = .08). Circulating antineutrophil cytoplasmic antibodies were associated with the development of vascular lesions in the graft but were not significantly correlated with graft survival (P = .07). This study supports the theory that renal transplant is an effective treatment option for patients with end-stage renal disease secondary to vasculitis. These patients fare similarly to, if not better than, other patients.

  7. Orientation-and-Location Controlled Single-Grain TFTs on Glass Substrate

    NARCIS (Netherlands)

    Chen, T.

    2011-01-01

    Location controlled single-grain TFT by ?-Czochralski process has attracted a lot of interest due to its high field effect mobility which is comparable to SOI. However, the major challenge is the random orientation of the grains due to the lack of seed layer to control the orientation. This will

  8. An iterated tabu search heuristic for the Single Source Capacitated Facility Location Problem

    DEFF Research Database (Denmark)

    Ho, Sin C.

    2015-01-01

    This paper discusses the Single Source Capacitated Facility Location Problem (SSCFLP) where the problem consists in determining a subset of capacitated facilities to be opened in order to satisfy the customers’ demands such that total costs are minimized. The paper presents an iterated tabu searc...... competitive with other metaheuristic approaches for solving the SSCFLP....

  9. Multi-Level Wavelet Shannon Entropy-Based Method for Single-Sensor Fault Location

    Directory of Open Access Journals (Sweden)

    Qiaoning Yang

    2015-10-01

    Full Text Available In actual application, sensors are prone to failure because of harsh environments, battery drain, and sensor aging. Sensor fault location is an important step for follow-up sensor fault detection. In this paper, two new multi-level wavelet Shannon entropies (multi-level wavelet time Shannon entropy and multi-level wavelet time-energy Shannon entropy are defined. They take full advantage of sensor fault frequency distribution and energy distribution across multi-subband in wavelet domain. Based on the multi-level wavelet Shannon entropy, a method is proposed for single sensor fault location. The method firstly uses a criterion of maximum energy-to-Shannon entropy ratio to select the appropriate wavelet base for signal analysis. Then multi-level wavelet time Shannon entropy and multi-level wavelet time-energy Shannon entropy are used to locate the fault. The method is validated using practical chemical gas concentration data from a gas sensor array. Compared with wavelet time Shannon entropy and wavelet energy Shannon entropy, the experimental results demonstrate that the proposed method can achieve accurate location of a single sensor fault and has good anti-noise ability. The proposed method is feasible and effective for single-sensor fault location.

  10. A hybrid algorithm for stochastic single-source capacitated facility location problem with service level requirements

    Directory of Open Access Journals (Sweden)

    Hosseinali Salemi

    2016-04-01

    Full Text Available Facility location models are observed in many diverse areas such as communication networks, transportation, and distribution systems planning. They play significant role in supply chain and operations management and are one of the main well-known topics in strategic agenda of contemporary manufacturing and service companies accompanied by long-lasting effects. We define a new approach for solving stochastic single source capacitated facility location problem (SSSCFLP. Customers with stochastic demand are assigned to set of capacitated facilities that are selected to serve them. It is demonstrated that problem can be transformed to deterministic Single Source Capacitated Facility Location Problem (SSCFLP for Poisson demand distribution. A hybrid algorithm which combines Lagrangian heuristic with adjusted mixture of Ant colony and Genetic optimization is proposed to find lower and upper bounds for this problem. Computational results of various instances with distinct properties indicate that proposed solving approach is efficient.

  11. [Primary childhood vasculitis new classification criteria

    DEFF Research Database (Denmark)

    Herlin, T.; Nielsen, Susan

    2008-01-01

    Primary vasculitis is seen in both adults and children, but some of the diseases like Kawasaki disease occur primarily in children. The Chapel Hill Classification Criteria for primary vasculitis refers to the size of vessels but has not been validated in children. Recently, new criteria...

  12. Single tracking location methods suppress speckle noise in shear wave velocity estimation.

    Science.gov (United States)

    Elegbe, Etana C; McAleavey, Stephen A

    2013-04-01

    In ultrasound-based elastography methods, the estimation of shear wave velocity typically involves the tracking of speckle motion due to an applied force. The errors in the estimates of tissue displacement, and thus shear wave velocity, are generally attributed to electronic noise and decorrelation due to physical processes. We present our preliminary findings on another source of error, namely, speckle-induced bias in phase estimation. We find that methods that involve tracking in a single location, as opposed to multiple locations, are less sensitive to this source of error since the measurement is differential in nature and cancels out speckle-induced phase errors.

  13. Enfrentamiento de las vasculitis primarias

    OpenAIRE

    Dr. D. Santiago Rivero

    2012-01-01

    Las Vasculitis Primarias son enfermedades poco frecuentes, potencialmente fatales, sin causa etiológica conocida, que pueden comprometer a vasos sanguíneos de distinto tamaño, produciéndoles un proceso inflamatorio en la pared vascular, que conduce a la estrechez u obstrucción del vaso afectado, con consecuente isquemia o necrosis del tejido que irrigan. Sus manifestaciones clínicas pueden ser muy variadas, y frecuentemente inespecíficas. Sin embargo hay algunas que hacen sospechar el diagnós...

  14. Identifying the Location of a Single Protein along the DNA Strand Using Solid-State Nanopores.

    Science.gov (United States)

    Yu, Jae-Seok; Lim, Min-Cheol; Huynh, Duyen Thi Ngoc; Kim, Hyung-Jun; Kim, Hyun-Mi; Kim, Young-Rok; Kim, Ki-Bum

    2015-05-26

    Solid-state nanopore has been widely studied as an effective tool to detect and analyze small biomolecules, such as DNA, RNA, and proteins, at a single molecule level. In this study, we demonstrate a rapid identification of the location of zinc finger protein (ZFP), which is bound to a specific locus along the length of a double-stranded DNA (dsDNA) to a single protein resolution using a low noise solid-state nanopore. When ZFP labeled DNAs were driven through a nanopore by an externally applied electric field, characteristic ionic current signals arising from the passage of the DNA/ZFP complex and bare DNA were detected, which enabled us to identify the locations of ZFP binding site. We examined two DNAs with ZFP binding sites at different positions and found that the location of the additional current drop derived from the DNA/ZFP complex is well-matched with a theoretical one along the length of the DNA molecule. These results suggest that the protein binding site on DNA can be mapped or that genetic information can be read at a single molecule level using solid-state nanopores.

  15. Thee-Dimensional Single-Track-Location Shear Wave Elasticity Imaging.

    Science.gov (United States)

    Hollender, Peter; Lipman, Samantha L; Trahey, Gregg E

    2017-12-01

    Conventional multiple-track-location shear wave elasticity imaging (MTL-SWEI) is a powerful tool for noninvasively estimating tissue elasticity. The resolution and noise levels of MTL-SWEI systems, however, are limited by ultrasound speckle. Single-track-location SWEI (STL-SWEI) is a novel variant which fixes the position of the tracking beam and modulates the push location to effectively cancel out the effects of speckle-induced bias. We present here a 3-D STL-SWEI system, which provides full suppression of lateral and elevation speckle bias for high-resolution volumetric elasticity imaging, and requires no spatial smoothing to make accurate measurements of shear wave speed. We demonstrate and analyze the system's performance in homogeneous and layered elasticity phantoms.

  16. Behcet's disease with cerebral vasculitis

    International Nuclear Information System (INIS)

    Scardamaglia, L.; Desmond, P.M.; Gonzales, M.F.; Bendrups, A.; Brodtmann, A.

    2001-01-01

    The case presented illustrates the diagnostic dilemma off neurological involvement in Behcet's disease and other inflammatory diseases. 'Psychiatric' symptoms were present for 2 years without abnormalities on SPECT or MRI and without CSF pleocytosis. Even at the time of fitting, no CSF abnormalities were observed. The preceding psychiatric presentations may have been due to cerebral vasculitis that was exacerbated by withdrawal of steroids. Magnetic resonance imaging is currently the most sensitive imaging modality. Lesions are usually in the brainstem, cerebellum, basal ganglia region or periventricular white matter, and the pons and the mesencephalon are commonly affected. In our patient there was no diencephalic or brainstem involvement. The inflammatory process can appear as a very large lesion, with gadolinium enhancement and significant mass effect, as in our patient. Brain magnetic resonance imaging. Postgadolinium-diethylenetriamine pentaacetic acid, axial image shows two large lesions in the right frontal lobe, with the larger, posterior lesion demonstrating vivid ring enhancement. A central nodule is isodense, with the cerebral white matter within the larger lesion. Surrounding low T 1 signal involves the hemispheric white matter without cortical extension and is consistent with vasogenic oedema. Minor mass effect is demonstrated with bowing of the anterior falx cerebri to the left. Biopsy shows prominent fibrinoid necrosis in small calibre postcapillary venules and cerebral white matter. There are surrounding acute and chronic inflammatory cells and nuclear debris, consistent with vasculitis

  17. Vasculitis after blood pressure monitoring.

    Science.gov (United States)

    Ventura-Ribes, Olga; Machancoses, Francisco H; Rosel Remírez, Jesús F

    2016-01-01

    Description of appearance of ecchymosis on an arm, simultaneously with a classical Leukocytoclastic Vasculitis, the proposal of alternative utilities of measuring blood pressure, and the study of side effects to that measure. Case 80-year-old male came to ER with dyspnea, heart failure, predialysis renal failure with hyperkalemia and hemodynamic instability. During his stay he developed a skin lesion that looks like palpable purpura, from the lower limit of the blood pressure cuff to the distal area of the hand that not disappeared with vitropression, and pruritus. During admission the arm injury was extended to all members, both upper and lower. The study concluded with diagnosis of Leukocytoclastic Vasculitis given the presence of eosinophils, that which suggested probable drug etiology to an antibiotic that had been taken since seven days prior to admission to ER. The need for serial monitoring of blood pressure, and the duration of such monitoring in unstable patients considering the side effects of those techniques was questioned. In addition, the study of other utilities of measuring blood pressure. Copyright © 2015 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  18. Measurement of damage in systemic vasculitis: a comparison of the Vasculitis Damage Index with the Combined Damage Assessment Index

    DEFF Research Database (Denmark)

    Suppiah, Ravi; Flossman, Oliver; Mukhtyar, Chetan

    2011-01-01

    To compare the Vasculitis Damage Index (VDI) with the Combined Damage Assessment Index (CDA) as measures of damage from vasculitis.......To compare the Vasculitis Damage Index (VDI) with the Combined Damage Assessment Index (CDA) as measures of damage from vasculitis....

  19. Accurate single-observer passive coherent location estimation based on TDOA and DOA

    Directory of Open Access Journals (Sweden)

    Li Jing

    2014-08-01

    Full Text Available This paper investigates the problem of target position estimation with a single-observer passive coherent location (PCL system. An approach that combines angle with time difference of arrival (ATDOA is used to estimate the location of a target. Compared with the TDOA-only method which needs two steps, the proposed method estimates the target position more directly. The constrained total least squares (CTLS technique is applied in this approach. It achieves the Cramer–Rao lower bound (CRLB when the parameter measurements are subject to small Gaussian-distributed errors. Performance analysis and the CRLB of this approach are also studied. Theory verifies that the ATDOA method gets a lower CRLB than the TDOA-only method with the same TDOA measuring error. It can also be seen that the position of the target affects estimating precision. At the same time, the locations of transmitters affect the precision and its gradient direction. Compared with the TDOA, the ATDOA method can obtain more precise target position estimation. Furthermore, the proposed method accomplishes target position estimation with a single transmitter, while the TDOA-only method needs at least four transmitters to get the target position. Furthermore, the transmitters’ position errors also affect precision of estimation regularly.

  20. Vasculitis

    Science.gov (United States)

    ... American College of Rheumatology Committee on Communications and Marketing. This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical ...

  1. Single-phased Fault Location on Transmission Lines Using Unsynchronized Voltages

    Directory of Open Access Journals (Sweden)

    ISTRATE, M.

    2009-10-01

    Full Text Available The increased accuracy into the fault's detection and location makes it easier for maintenance, this being the reason to develop new possibilities for a precise estimation of the fault location. In the field literature, many methods for fault location using voltages and currents measurements at one or both terminals of power grids' lines are presented. The double-end synchronized data algorithms are very precise, but the current transformers can limit the accuracy of these estimations. The paper presents an algorithm to estimate the location of the single-phased faults which uses only voltage measurements at both terminals of the transmission lines by eliminating the error due to current transformers and without introducing the restriction of perfect data synchronization. In such conditions, the algorithm can be used with the actual equipment of the most power grids, the installation of phasor measurement units with GPS system synchronized timer not being compulsory. Only the positive sequence of line parameters and sources are used, thus, eliminating the incertitude in zero sequence parameter estimation. The algorithm is tested using the results of EMTP-ATP simulations, after the validation of the ATP models on the basis of registered results in a real power grid.

  2. An improved cut-and-solve algorithm for the single-source capacitated facility location problem

    DEFF Research Database (Denmark)

    Gadegaard, Sune Lauth; Klose, Andreas; Nielsen, Lars Relund

    2017-01-01

    In this paper, we present an improved cut-and-solve algorithm for the single-source capacitated facility location problem. The algorithm consists of three phases. The first phase strengthens the integer program by a cutting plane algorithm to obtain a tight lower bound. The second phase uses a two......-level local branching heuristic to find an upper bound, and if optimality has not yet been established, the third phase uses the cut-and-solve framework to close the optimality gap. Extensive computational results are reported, showing that the proposed algorithm runs 10–80 times faster on average compared...

  3. Medium and large vessel vasculitis

    OpenAIRE

    BRANDT, Hebert Roberto Clivati; ARNONE, Marcelo; VALENTE, Neusa Yuriko Sakai; SOTTO, Mirian Nacagami; CRIADO, Paulo Ricardo

    2009-01-01

    As vasculites são constituídas por um grande grupo de síndromes caracterizadas por inflamação e necrose da parede dos vasos sangüíneos, resultando em estreitamento ou oclusão do lúmen. A distribuição dos vasos envolvidos varia consideravelmente e serve de base para a classificação das síndromes vasculíticas: grandes vasos (arterite de Takayasu, arterite temporal); pequenos e médios vasos (poliarterite nodosa, síndrome de Churg-Strauss, granulomatose de Wegener, vasculites nas doenças reumátic...

  4. A probabilistic framework for single-station location of seismicity on Earth and Mars

    Science.gov (United States)

    Böse, M.; Clinton, J. F.; Ceylan, S.; Euchner, F.; van Driel, M.; Khan, A.; Giardini, D.; Lognonné, P.; Banerdt, W. B.

    2017-01-01

    Locating the source of seismic energy from a single three-component seismic station is associated with large uncertainties, originating from challenges in identifying seismic phases, as well as inevitable pick and model uncertainties. The challenge is even higher for planets such as Mars, where interior structure is a priori largely unknown. In this study, we address the single-station location problem by developing a probabilistic framework that combines location estimates from multiple algorithms to estimate the probability density function (PDF) for epicentral distance, back azimuth, and origin time. Each algorithm uses independent and complementary information in the seismic signals. Together, the algorithms allow locating seismicity ranging from local to teleseismic quakes. Distances and origin times of large regional and teleseismic events (M > 5.5) are estimated from observed and theoretical body- and multi-orbit surface-wave travel times. The latter are picked from the maxima in the waveform envelopes in various frequency bands. For smaller events at local and regional distances, only first arrival picks of body waves are used, possibly in combination with fundamental Rayleigh R1 waveform maxima where detectable; depth phases, such as pP or PmP, help constrain source depth and improve distance estimates. Back azimuth is determined from the polarization of the Rayleigh- and/or P-wave phases. When seismic signals are good enough for multiple approaches to be used, estimates from the various methods are combined through the product of their PDFs, resulting in an improved event location and reduced uncertainty range estimate compared to the results obtained from each algorithm independently. To verify our approach, we use both earthquake recordings from existing Earth stations and synthetic Martian seismograms. The Mars synthetics are generated with a full-waveform scheme (AxiSEM) using spherically-symmetric seismic velocity, density and attenuation models of

  5. A case of small vessel vasculitis

    Directory of Open Access Journals (Sweden)

    Madhulika Mahashabde

    2014-01-01

    We are reporting a case of un-specified small vessel vasculitis, which was diagnosed on the basis of positive perinuclear anti neutrophil cytoplasmic antibodies (ANCA P MPO done by Enzyme Linked Immunosorbent Assay (ELISA.

  6. What matters for patients with vasculitis?

    Science.gov (United States)

    Novakovich, Elaine; Grayson, Peter C

    2015-06-01

    Advances in clinical care for patients with vasculitis have improved survival rates and created new challenges related to the ongoing management of chronic disease. Lack of curative therapies, burden of disease, treatment-related side effects, and fear of relapse contribute to patient-perceived reduction in quality of life. Patient-held beliefs about disease and priorities may differ substantially from the beliefs of their health care providers, and research paradigms are shifting to reflect more emphasis on understanding vasculitis from the patient's perspective. Efforts are ongoing to develop disease outcome measures in vasculitis that better represent the patient experience. Health care providers who care for patients with vasculitis should be sensitive to the substantial burdens of disease commonly experienced by patients living with the disease and should strive to provide comprehensive care directed towards the medical and biopsychological needs of these patients. Published by Elsevier Masson SAS.

  7. Hansen's disease mimicking a systemic vasculitis.

    Science.gov (United States)

    Sampaio, L; Silva, L; Terroso, G; Pimenta, S; Brandão, F; Pinto, J; Prisca, A; Brito, J; Ventura, F

    2011-01-01

    Hansen's disease, caused by Mycobacterium leprae, classically presents with cutaneous and neurological manifestations. Rheumatologic manifestations present in 1 to 5% of the patients, and include arthritis, arthralgias, Charcot arthropathy, erythema nodosum and vasculitis. We report a case of a 86 year old woman with polyarthritis, subcutaneous nodules and leg ulcers whose differential diagnosis included primary vasculitis and diffuse connective tissue diseases and ended to be leprosy in a non endemic country.

  8. Pathophysiological Relationship between Infections and Systemic Vasculitis

    Directory of Open Access Journals (Sweden)

    Carolina Muñoz-Grajales

    2015-01-01

    Full Text Available The development of autoimmune disorders requires a combination of genetic, immunological, and environmental factors. Infectious agents, such as viruses and bacteria, can trigger autoimmunity through different mechanisms, and for systemic vasculitis in particular, microbial agents have been suggested to be involved in its pathogenesis. Although the exact mechanisms have not been fully elucidated, different theories have been postulated. This review considers the role of infections in the etiology of primary vasculitis, emphasizing their related immunological events.

  9. ANCA-associated vasculitis and malignancy

    DEFF Research Database (Denmark)

    Mahr, Alfred; Heijl, Caroline; Le Guenno, Guillaume

    2013-01-01

    In this review, we summarise the current understanding of the potential link between cancer and anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), including granulomatosis with polyangiitis (Wegener's; GPA) and microscopic polyangiitis (MPA). As is true for many autoimmune or infla......In this review, we summarise the current understanding of the potential link between cancer and anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), including granulomatosis with polyangiitis (Wegener's; GPA) and microscopic polyangiitis (MPA). As is true for many autoimmune...

  10. Performance analysis of high frequency single-site-location antenna arrays using numerical electromagnetic modeling

    Science.gov (United States)

    Schiantarelli, Harry T.

    1990-09-01

    Electronic support measures (ESM) systems play an increasingly important role in modern warfare and can influence the outcome of a military engagement. The application of ESM can be extended to anti-guerrilla and anti-drug operations where law enforcement agencies can exploit the fact that their presence is inducing the outlaw to depend more on radio communications to coordinate their activities. When a propagation path of no more than one reflection at the ionosphere (1-hop) can be assumed, position of an HF emitter can be determined by a single observing site using vertical triangulation, provided that the height of the ionosphere at the point where the radio wave is reflected, can be determined. This technique is known as high frequency direction finding single-site-location (HFDF SSL). This thesis analyzes the HFDF SSL error in measuring the direction of arrival of the signal, how this error is generated by the antenna array and its effect on emitter location. The characteristics of the two antenna arrays used by a specific HFDF SSL system that implements the phase-interferometer techniques were studied using electromagnetic modeling.

  11. Single Tracking Location Acoustic Radiation Force Impulse Viscoelasticity Estimation (STL-VE): A Method for Measuring Tissue Viscoelastic Parameters

    OpenAIRE

    Langdon, Jonathan H; Elegbe, Etana; McAleavey, Stephen A

    2015-01-01

    Single Tracking Location (STL) Shear wave Elasticity Imaging (SWEI) is a method for detecting elastic differences between tissues. It has the advantage of intrinsic speckle bias suppression compared to Multiple Tracking Location (MTL) variants of SWEI. However, the assumption of a linear model leads to an overestimation of the shear modulus in viscoelastic media. A new reconstruction technique denoted Single Tracking Location Viscosity Estimation (STL-VE) is introduced to correct for this ove...

  12. Illness perceptions and fatigue in systemic vasculitis.

    Science.gov (United States)

    Grayson, Peter C; Amudala, Naomi A; Mcalear, Carol A; Leduc, Renée L; Shereff, Denise; Richesson, Rachel; Fraenkel, Liana; Merkel, Peter A

    2013-11-01

    To compare illness perceptions among patients with different forms of vasculitis, identify risk factors for negative illness perceptions, and determine the association between illness perceptions and fatigue. Participants were recruited from an online vasculitis registry to complete the revised Illness Perception Questionnaire (IPQ-R). The mean scores on each IPQ-R dimension were compared across different types of vasculitis. Cluster analysis and stepwise regression identified predictors of negative illness perception. Fatigue was measured using the general subscale of the Multidimensional Fatigue Inventory (MFI-20). Patient-reported measures of disease activity and IPQ-R dimensions were assessed in relation to MFI-20 scores using linear regression in sequential, additive models with model-fit comparisons. In total, 692 participants with 9 types of vasculitis completed the IPQ-R. For 6 of the 8 IPQ-R dimensions, there were no significant differences in mean scores between the different vasculitides. Scores in the identity and cyclical dimensions were significantly higher in Behçet’s disease compared with other types of vasculitis (13.5 versus 10.7 for identity and 4.0 versus 3.2 for cyclical [P IPQ-R dimensions explained an equivalent proportion of variability in fatigue scores compared with measures of disease activity. Illness perceptions are similar across different types of vasculitis, and younger age is a risk factor for negative illness perceptions. Illness perceptions explain differences in fatigue scores beyond what can be explained by measures of disease activity.

  13. History of primary vasculitis in Latin America.

    Science.gov (United States)

    Iglesias Gammara, Antonio; Coral, Paola; Quintana, Gerardo; Toro, Carlos E; Flores, Luis Felipe; Matteson, Eric L; Restrepo, José Félix

    2010-03-01

    A literature review utilizing Fepafem, Bireme, LiLacs, Scielo Colombia, Scielo Internacional, former MedLine, Pubmed, and BVS Colombia as well as manual searches in the libraries of major Latin American universities was performed to study vasculitis in Latin America. Since 1945, a total of 752 articles have been published by Latin American authors. However, only a minority are devoted to primary vasculitides, and even fewer have been published in indexed journals. Approximately 126 are in OLD, Medline, Pubmed, Bireme, and Scielo. Most publications are from Mexico, followed by Brazil and Colombia. Systematic studies of the epidemiology of primary idiopathic vasculitis are available for a few countries, i.e. Brazil, Mexico, Colombia, Chile, and Peru. Takayasu arteritis and ANCA-associated vasculitis are the best studied forms of vasculitis in Latin America. Interest and expertise in vasculitis is growing in Latin America, as reflected in the increased number of published articles from this region of the world in the last decade. Racial and environmental factors are possibly responsible for the differential expression of various types of primary vasculitis observed in Latin America. With time, the unique features, epidemiology, and better treatment strategies for idiopathic vasculitides in Latin America will emerge.

  14. Single aromatic residue location alters nucleic acid binding and chaperone function of FIV nucleocapsid protein

    Science.gov (United States)

    Wu, Hao; Wang, Wei; Naiyer, Nada; Fichtenbaum, Eric; Qualley, Dominic F.; McCauley, Micah J.; Gorelick, Robert J.; Rouzina, Ioulia; Musier-Forsyth, Karin; Williams, Mark C.

    2014-01-01

    Feline immunodeficiency virus (FIV) is a retrovirus that infects domestic cats, and is an excellent animal model for human immunodeficiency virus type 1 (HIV-1) pathogenesis. The nucleocapsid (NC) protein is critical for replication in both retroviruses. FIV NC has several structural features that differ from HIV-1 NC. While both NC proteins have a single conserved aromatic residue in each of the two zinc fingers, the aromatic residue on the second finger of FIV NC is located on the opposite C-terminal side relative to its location in HIV-1 NC. In addition, whereas HIV-1 NC has a highly charged cationic N-terminal tail and a relatively short C-terminal extension, the opposite is true for FIV NC. To probe the impact of these differences on the nucleic acid (NA) binding and chaperone properties of FIV NC, we carried out ensemble and single-molecule assays with wild-type (WT) and mutant proteins. The ensemble studies show that FIV NC binding to DNA is strongly electrostatic, with a higher effective charge than that observed for HIV-1 NC. The C-terminal basic domain contributes significantly to the NA binding capability of FIV NC. In addition, the non-electrostatic component of DNA binding is much weaker for FIV NC than for HIV-1 NC. Mutation of both aromatic residues in the zinc fingers to Ala (F12A/W44A) further increases the effective charge of FIV NC and reduces its non-electrostatic binding affinity. Interestingly, switching the location of the C-terminal aromatic residue to mimic the HIV-1 NC sequence (N31W/W44A) reduces the effective charge of FIV NC and increases its non-electrostatic binding affinity to values similar to HIV-1 NC. Consistent with the results of these ensemble studies, single-molecule DNA stretching studies show that while WT FIV NC has reduced stacking capability relative to HIV-1 NC, the aromatic switch mutant recovers the ability to intercalate between the DNA bases. Our results demonstrate that altering the position of a single aromatic

  15. A cross-sectional study of the Birmingham Vasculitis Activity Score version 3 in systemic vasculitis.

    LENUS (Irish Health Repository)

    Suppiah, Ravi

    2011-05-01

    Assessment of disease activity in vasculitis can be achieved using the BVAS, a clinical checklist of relevant symptoms, signs and features of active disease. The aim of this study was to revalidate the BVAS version 3 (BVAS v. 3) in a cohort of patients with systemic vasculitis.

  16. Mechanisms of vasculitis : How pauci-immune is ANCA-associated renal vasculitis?

    NARCIS (Netherlands)

    van Paassen, P.; Tervaert, J. W. Cohen; Heeringa, P.

    2007-01-01

    Both the innate and the acquired immune system are involved in the pathophysiology of renal vasculitis. However, anti-neutrophil cytoplasmic antibody (ANCA)-associated renal vasculitis is characterized by a 'pauci-immune' pattern of immunofluorescence during kidney biopsy, indicating the relative

  17. Lattice location of platinum ions implanted into single crystal zirconia and their annealing behaviour

    Energy Technology Data Exchange (ETDEWEB)

    Cao, D.X. [Royal Melbourne Inst. of Tech., VIC (Australia); Sood, D.K. [Academia Sinica, Shanghai, SH (China). Shanghai Inst. of Nuclear Research; Brown, I.G. [Lawrence Berkeley Lab., CA (United States)

    1993-12-31

    Single crystal samples of (100) oriented cubic zirconia stabilised with 9.5 mol % yttria were implanted with platinum ions, using a metal vapour vacuum arc (MEVVA) high current ion implanter, to a nominal dose of 1x10{sup 17} ions/cm{sup 2}. The implanted samples were annealed isothermally in air ambient at 1200 deg C, from 1-24 hours. Rutherford Backscattering Spectrometry and Channeling (RBSC) of 2 MeV He ions are employed to determine depth distributions of ion damage, Pt ions and substitutionality of Pt ions before and after annealing. The damage behaviour, Pt migration and lattice location are discussed in terms of metastable phase formation and solid solubility considerations. 7 refs., 3 figs.

  18. Epigenetics and Vasculitis: a Comprehensive Review.

    Science.gov (United States)

    Renauer, Paul; Coit, Patrick; Sawalha, Amr H

    2016-06-01

    Vasculitides represent a group of relatively rare systemic inflammatory diseases of the blood vessels. Despite recent progress in understanding the genetic basis and the underlying pathogenic mechanisms in vasculitis, the etiology and pathogenesis of vasculitis remain incompletely understood. Epigenetic dysregulation plays an important role in immune-mediated diseases, and the contribution of epigenetic aberrancies in vasculitis is increasingly being recognized. Histone modifications in the PR3 and MPO gene loci might be mechanistically involved in the pathogenesis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Similarly, other studies revealed important epigenetic contribution to other vasculitides, including Kawasaki disease and IgA vasculitis. More recently, genome-wide epigenomic studies have been performed in several vasculitides. A recent genome-wide DNA methylation study uncovered an important role for epigenetic remodeling of cytoskeleton-related genes in the pathogenesis of Behçet's disease and suggested that reversal of some of these DNA methylation changes associates with disease remission. Genome-wide DNA methylation profiling characterized the inflammatory response in temporal artery tissue from patients with giant cell arteritis and showed increased activation of calcineurin/nuclear factor of activated T cells (NFAT) signaling, prompting the suggestion that a specific calcineurin/NFAT inhibitor that is well tolerated and with the added beneficial anti-platelet activity, such as dipyridamole, might be of therapeutic potential in giant cell arteritis. While epigenetic studies in systemic vasculitis are still in their infancy, currently available data clearly indicate that investigating the epigenetic mechanisms underlying these diseases will help to better understand the pathogenesis of vasculitis and provide novel targets for the development of disease biomarkers and new therapies.

  19. Significance of isolated vasculitis in the gynecological tract: what clinicians do with the pathologic diagnosis of vasculitis?

    Science.gov (United States)

    Roma, Andres A; Amador-Ortiz, Catalina; Liapis, Helen

    2014-08-01

    Vasculitides includes a heterogeneous group of disorders with the common histologic findings of vascular wall inflammation. Systemic or localized disease (eg, renal vasculitis) has serious consequences. The incidence of isolated gynecologic vasculitis diagnosed on pathology specimens and its significance is little known. We performed a 20 year retrospective review including 53 cases with vasculitis diagnosis affecting the female genital tract identified in pathology reports. None had prior symptoms or were diagnosed with generalized vasculitis, while one patient had prior diagnosis of fibromyalgia. Most patients presented with abnormal bleeding and were treated for conditions unrelated to vasculitis. The different types of vasculitis were: predominantly lymphocytic (nonspecific) 30 cases, necrotizing 17 cases and granulomatous 6 cases. Only 2 patients had additional serologic tests. None of the patients with isolated gynecologic vasculitis received corticosteroids or additional treatment related to the vasculitis. None of the patients developed systemic vasculitis at follow-up (2 months-19.5 years; mean, 5.5 years). Isolated gynecologic vasculitis diagnosed on pathology slides is rarely associated with systemic vasculitis. Potential isolated gynecologic vasculitis causes include: previous surgical interventions and vascular inflammation secondary to local neoplasm. In almost all cases, clinicians did not perform a thorough laboratory analysis to exclude systemic vasculitis and therapy was not required in any case, suggesting minimal clinical significance. Copyright © 2014 Elsevier Inc. All rights reserved.

  20. Reproductive Health in Men and Women With Vasculitis

    Science.gov (United States)

    2014-06-25

    Giant Cell Arteritis; Takayasu's Arteritis; Polyarteritis Nodosa; Wegener's Granulomatosis; Microscopic Polyangiitis; Churg-Strauss Syndrome; Behcet's Disease; Kawasaki Disease; Henoch-schoenlein Purpura; Vasculitis, Central Nervous System; Drug-induced Necrotizing Vasculitis

  1. Illness Perceptions and Fatigue in Systemic Vasculitis

    Science.gov (United States)

    Grayson, Peter C.; Amudala, Naomi A.; McAlear, Carol A.; Leduc, Renée L.; Shereff, Denise; Richesson, Rachel; Fraenkel, Liana; Merkel, Peter A.

    2014-01-01

    Objective To compare illness perceptions among patients with different forms of vasculitis, identify risk factors for negative illness perceptions, and determine the association between illness perceptions and fatigue. Methods Participants were recruited from an online registry in vasculitis to complete the revised Illness Perception Questionnaire (IPQ-R). Mean scores on each IPQ-R dimension were compared across types of vasculitis. Cluster analysis and stepwise regression identified predictors of negative illness perception. Fatigue was measured using the general subscale of the Multidimensional Fatigue Inventory (MFI). Patient-reported measures of disease activity and IPQ-R dimensions were assessed in relation to MFI scores using linear regression in sequential, additive models with model-fit comparisons. Results 692 participants with 9 forms of vasculitis completed the IPQ-R. For 6 out of 8 IPQ-R dimensions, there were no significant differences in mean scores between the different vasculitides. Scores in identity and cyclical dimensions were significantly higher in Behçet’s disease compared to other types of vasculitis (13.5 vs 10.7; 4.0 vs 3.2, pvasculitis, and younger age is a risk factor for negative illness perceptions. Illness perceptions explain differences in fatigue scores beyond what can be explained by measures of disease activity. PMID:23861259

  2. Renal vasculitis presenting with acute kidney injury.

    Science.gov (United States)

    Villacorta, Javier; Diaz-Crespo, Francisco; Acevedo, Mercedes; Cavero, Teresa; Guerrero, Carmen; Praga, Manuel; Fernandez-Juarez, Gema

    2017-06-01

    Renal failure secondary to ANCA-associated vasculitis represents a clinical and therapeutic challenge. In this study, we aimed to assess the treatment response rates and long-term outcomes of vasculitis patients presenting with renal failure. This retrospective study included 151 patients with renal vasculitis from three hospitals who underwent a renal biopsy between 1997 and 2014. Patients with renal failure which required dialysis at the onset were compared to those presenting with more preserved renal function. The primary end point was treatment response and patient surivival. Patients with severe renal involvement had a lower response to treatment compared to those having preserved renal function (26.6 versus 93.4%; p renal recovery (41.6 versus 12.5%; p = 0.05). A higher incidence of severe infections was observed among patients with severe renal involvement (38.4 versus 18.1%, p = 0.01). The mortality rate was significantly higher among vasculitis patients presenting with renal failure (53.8 versus 22.2%, p = 0.001). Global survival at 1 and 5 years was 60 and 47% in patients requiring dialysis compared with 90 and 80% among those with more preserved renal function (p renal dysfunction represents an independent risk factor for patient survival in renal vasculitis. Patients requiring dialysis associate a lower response rate to immunosuppressive therapy and a higher incidence of severe infections.

  3. Interstital lung disease in ANCA vasculitis.

    Science.gov (United States)

    Alba, Marco A; Flores-Suárez, Luis Felipe; Henderson, Ashley G; Xiao, Hong; Hu, Peiqi; Nachman, Patrick H; Falk, Ronald J; Charles Jennette, J

    2017-07-01

    Anti-neutrophil cytoplasmic antibodies (ANCA) vasculitides are immune-mediated disorders that primarily affect small blood vessels of the airway and kidneys. Lung involvement, one of the hallmarks of microscopic polyangiitis and granulomatosis with polyangiitis, is associated with increased mortality and morbidity. In recent years, several retrospective series and case reports have described the association of interstitial lung disease (ILD) and ANCA vasculitis, particularly those positive for ANCA specific for myeloperoxidase. In the majority of these patients pulmonary fibrosis occurs concurrently or predates the diagnosis of ANCA vasculitis. More importantly, these studies have shown that ILD has an adverse impact on the long-term prognosis of ANCA vasculitis. This review focuses on the main clinical and radiologic features of pulmonary fibrosis associated with anti-neutrophil cytoplasmic antibodies. Major histopathology features, prognosis and therapeutic options are summarized. Copyright © 2017 Elsevier B.V. All rights reserved.

  4. SINGLE-SHELL TANKS LEAK INTEGRITY ELEMENTS/SX FARM LEAK CAUSES AND LOCATIONS - 12127

    Energy Technology Data Exchange (ETDEWEB)

    VENETZ TJ; WASHENFELDER D; JOHNSON J; GIRARDOT C

    2012-01-25

    Washington River Protection Solutions, LLC (WRPS) developed an enhanced single-shell tank (SST) integrity project in 2009. An expert panel on SST integrity was created to provide recommendations supporting the development of the project. One primary recommendation was to expand the leak assessment reports (substitute report or LD-1) to include leak causes and locations. The recommendation has been included in the M-045-9IF Hanford Federal Facility Agreement and Consent Order (Tri-Party Agreement) as one of four targets relating to SST leak integrity. The 241-SX Farm (SX Farm) tanks with leak losses were addressed on an individual tank basis as part of LD-1. Currently, 8 out of 23 SSTs that have been reported to having a liner leak are located in SX Farm. This percentage was the highest compared to other tank farms which is why SX Farm was analyzed first. The SX Farm is comprised of fifteen SSTs built 1953-1954. The tanks are arranged in rows of three tanks each, forming a cascade. Each of the SX Farm tanks has a nominal I-million-gal storage capacity. Of the fifteen tanks in SX Farm, an assessment reported leak losses for the following tanks: 241-SX-107, 241-SX-108, 241-SX-109, 241-SX-111, 241-SX-112, 241-SX-113, 241-SX-114 and 241-SX-115. The method used to identify leak location consisted of reviewing in-tank and ex-tank leak detection information. This provided the basic data identifying where and when the first leaks were detected. In-tank leak detection consisted of liquid level measurement that can be augmented with photographs which can provide an indication of the vertical leak location on the sidewall. Ex-tank leak detection for the leaking tanks consisted of soil radiation data from laterals and drywells near the tank. The in-tank and ex-tank leak detection can provide an indication of the possible leak location radially around and under the tank. Potential leak causes were determined using in-tank and ex-tank information that is not directly related to

  5. Antineutrophil Cytoplasmic Antibodies, Autoimmune Neutropenia, and Vasculitis

    Science.gov (United States)

    Grayson, Peter C.; Sloan, J. Mark; Niles, John L.; Monach, Paul A.; Merkel, Peter A.

    2011-01-01

    Objectives Reports of an association between antineutrophil cytoplasmic antibodies (ANCA) and autoimmune neutropenia have rarely included cases of proven vasculitis. A case of ANCA-associated vasculitis (AAV) with recurrent neutropenia is described and relevant literature on the association between ANCA, neutropenia, and vasculitis is reviewed. Methods Longitudinal clinical assessments and laboratory findings are described in a patient with AAV and recurrent episodes of profound neutropenia from December 2008 – October 2010. A PubMed database search of the medical literature was performed for papers published from 1960 through October 2010 to identify all reported cases of ANCA and neutropenia. Results A 49 year-old man developed recurrent neutropenia, periodic fevers, arthritis, biopsy-proven cutaneous vasculitis, sensorineural hearing loss, epididymitis, and positive tests for ANCA with specificity for antibodies to both proteinase 3 and myeloperoxidase. Antineutrophil membrane antibodies were detected during an acute neutropenic phase and were not detectable in a post-recovery sample, whereas ANCA titers did not seem to correlate with neutropenia. An association between ANCA and neutropenia has been reported in 74 cases from 24 studies in the context of drug/toxin exposure, underlying autoimmune disease, or chronic neutropenia without underlying autoimmune disease. In these cases, the presence of atypical ANCA patterns and other antibodies were common; however, vasculitis was uncommon and when it occurred was usually limited to the skin and in cases of underlying toxin exposure. Conclusions ANCA is associated with autoimmune neutropenia, but systemic vasculitis rarely occurs in association with ANCA and neutropenia. The interaction between neutrophils and ANCA may provide insight into understanding both autoimmune neutropenia and AAV. PMID:21507463

  6. Neuroimaging of cerebral vasculitis; Bildgebung zerebraler Vaskulitiden

    Energy Technology Data Exchange (ETDEWEB)

    Wengenroth, M. [Universitaetsklinikum Schleswig-Holstein, Campus Luebeck, Institut fuer Neuroradiologie, Luebeck (Germany); Klinikum der Ludwig-Maximilians-Universitaet, Institut fuer klinische Radiologie, Muenchen (Germany); Saam, T. [Klinikum der Ludwig-Maximilians-Universitaet, Institut fuer klinische Radiologie, Muenchen (Germany); Haehnel, S. [Universitaetsklinikum Heidelberg, Abteilung Neuroradiologie, Heidelberg (Germany)

    2016-01-15

    Cerebral vasculitis can have a variety of origins. Furthermore, there are no vasculitis-specific symptoms or imaging signs and vasculitis of the CNS can mimic many other neurological diseases, which require different treatment approaches. Thus, the clinical and radiological diagnosis of cerebral vasculitis is challenging. Magnetic resonance imaging (MRI) and MR angiography (MRA) should be the radiological imaging methods of choice to assess the degree of parenchymal damage and to detect vessel wall changes. If the results are unclear digital subtraction angiography (DSA) should be pursued in order to also detect changes in medium sized vessels. Vasculitis of small vessels cannot be detected by vascular imaging and requires brain or leptomeningeal biopsy. In this review we present the current diagnostic approach and a variety of imaging findings in cerebral vasculitis and discuss the main radiological differential diagnoses. (orig.) [German] Die zerebrale Vaskulitis kann viele Ursachen haben. Daher ist die klinische und radiologische Diagnose schwierig, auch weil es etliche vaskulitistypische Symptome und MR-Befunde bei anderen Erkrankungen gibt. Die ZNS-Vaskulitis kann zahlreiche andere neurologische Erkrankungen imitieren, die unterschiedlicher Therapie beduerfen. MRT und MR-Angiographie (MRA) sind die Verfahren der Wahl, mit denen die radiologische Diagnostik begonnen werden sollte, um den Parenchymschaden und den Gefaessbefall zu beurteilen. Bei unklaren Befunden kann eine digitale Subtraktionsangiographie (DSA) ergaenzt werden, um auch mittelgrosse Gefaesse beurteilen zu koennen. Der Befall kleiner Gefaesse kann nur bioptisch nachgewiesen werden. In der folgenden Uebersicht stellen wir das diagnostische Herangehen bei zerebraler Vaskulitis vor, praesentieren zahlreiche Befunde und diskutieren die wichtigsten radiologischen Differenzialdiagnosen. (orig.)

  7. Connective Tissue Disorder-Associated Vasculitis.

    Science.gov (United States)

    Sharma, Aman; Dhooria, Aadhaar; Aggarwal, Ashish; Rathi, Manish; Chandran, Vinod

    2016-06-01

    Vasculitides secondary to connective tissue diseases are classified under the category of 'vasculitis associated with systemic disease' in the revised International Chapel Hill Consensus Conference (CHCC) nomenclature. These secondary vasculitides may affect any of the small, medium or large vessels and usually portend a poor prognosis. Any organ system can be involved and the presentation would vary depending upon that involvement. Treatment depends upon the type and severity of presentation. In this review, we describe secondary vasculitis associated with rheumatoid arthritis, systemic lupus erythematosus, sarcoidosis, relapsing polychondritis, systemic sclerosis, Sjogren's syndrome and idiopathic inflammatory myositis, focusing mainly on recent advances in the past 3 years.

  8. Urticarial Vasculitis in a Teenage Girl

    Directory of Open Access Journals (Sweden)

    Aaron McGuffin MD

    2013-04-01

    Full Text Available This case involves a 13-year-old female who presented to the pediatrician for a routine check-up with complaints of a long history of intermittent diarrhea followed by a severe rash lasting for up to a week afterwards. The mother had described her daughter’s condition to multiple physicians, several whom had seen her during flare-ups. The nonmigratory lesions resembled “hives” with a single lesion lasting for 48 to 72 hours and resolving into what her parent described as a bruise. They often diagnosed her daughter with urticaria and prescribed steroids, which did resolve the acute flare-ups. None of the physicians, however, focused on the disease’s evolution and chronicity in an effort toward diagnosis and prevention. The patient was referred by her pediatrician to a dermatologist who diagnosed the patient with urticarial vasculitis. She was initially started on dapsone 25 mg and was increased over a period of months to a maintenance dose of 100 mg daily. She has had no recurrences in her cutaneous or systemic symptoms on this dose. She is closely monitored by her dermatologist on a regular basis with twice yearly complete blood counts. Several attempts have been made to discontinue the dapsone, resulting in a flare of her gastrointestinal symptoms. This patient suffered with this condition for almost 10 years. This is a reminder that spending extra time to think through a patient’s problem early on may prevent years of suffering for patients and their families.

  9. Vasculitis syndromes : Peripheral neuropathy in AAV--when vasculitis hits a nerve

    NARCIS (Netherlands)

    Rutgers, Abraham; Kallenberg, Cornelis

    Peripheral neuropathy can be a manifestation of small-vessel vasculitides such as antineutrophil cytoplasmic antibody-associated vasculitis. Diagnosing vasculitic neuropathy is, however, difficult in many cases. Early treatment focused on achieving remission of the underlying vasculitic process is

  10. Does drinking location matter? Profiles of risky single-occasion drinking by location and alcohol-related harm among young men

    Directory of Open Access Journals (Sweden)

    Caroline eBähler

    2014-06-01

    Full Text Available In adolescents and young adults, acute consequences like injuries account for a substantial proportion of alcohol-related harm, especially in risky single-occasion (RSO drinkers. The primary aim of the study was to characterize different drinking profiles in RSO drinkers according to drinking locations and their relationship to negative, alcohol-related consequences. The sample consisted of 2746 young men from the Cohort Study on Substance Use Risk Factors (C-SURF who had reported drinking 6 or more drinks on a single occasion at least monthly over the preceding 12 months. Principal component analysis on the frequency and amount of drinking at 11 different locations was conducted, and two distinguishable components emerged: a non-party-dimension (loading high on theatre/cinema, sport clubs, other clubs/societies, restaurants, and sport events and a party-dimension (loading high on someone else’s home, pubs/bars, discos/nightclubs, outdoor public places, special events, and home. Differential impacts of drinking location profiles were observed on severe negative alcohol-related consequences (SAC. Relative to those classified as low or intermediate in both dimensions, no significant difference experiencing SAC was found among those who were classified as high in the non-party-dimension only. However, those who were classified as high in the party-dimension alone or in both dimensions were more likely to experience SAC. These differential effects remained after adjusting for alcohol consumption (volume and RSOD, personality traits, and peer-influence (adjusted OR=0.83 [0.68-1.02], 1.57 [1.27-1.96] and 1.72 [1.23-2.41], respectively, indicating independent effects of drinking location on SAC. The inclusion of sociodemographic factors did not alter this association. The fact that this cluster of party-dimension locations seems to predispose young men to experiencing SAC has important implications for alcohol control policies.

  11. Location, Location, Location!

    Science.gov (United States)

    Ramsdell, Kristin

    2004-01-01

    Of prime importance in real estate, location is also a key element in the appeal of romances. Popular geographic settings and historical periods sell, unpopular ones do not--not always with a logical explanation, as the author discovered when she conducted a survey on this topic last year. (Why, for example, are the French Revolution and the…

  12. Location, location, location

    NARCIS (Netherlands)

    Anderson, S.P.; Goeree, J.K.; Ramer, R.

    1997-01-01

    We analyze the canonical location-then-price duopoly game with general log- concave consumer densities. A unique pure-strategy equilibrium to the two-stage game exists if the density is not "too asymmetric" and not "too concave." These criteria are satisfied by many commonly used densities.

  13. [Diagnosis and therapy of systemic necrotizing vasculitis].

    Science.gov (United States)

    Aksamija-Rizvić, B

    1992-01-01

    Through the presentation of the case of Leucocytoclastic vasculitis of a young man we enlighten the problems of diagnosis, differential diagnosis, ethiology, development of disease, and therapeutic approach. We presented the new knowledge in the patogenesis of changes in the vessel wall and possible correlation with thrombotic thrombocytopenic purpura.

  14. Perspective on future therapy of vasculitis.

    Science.gov (United States)

    Boumpas, D T; Kritikos, H D; Daskalakis, N G

    2000-10-01

    This article summarizes recent advances in the management of various vasculitic syndromes and discusses potential new therapies based on a better understanding of their pathogenesis and natural history. Current efforts for optimization of testing for antineutrophil cytoplasmic antibodies and improvement of diagnostic criteria will certainly have a significant impact on future therapy. Biologic agents such as interferon-alpha are already in use in various vasculitides, whereas others, such as inhibitors of tumor necrosis factor-alpha, are in phase I clinical trials. Agents that selectively inhibit distinct steps in the pathogenesis of vasculitis are in preclinical or early clinical stages of development. Newer (mycophenolate mofetil, leflunamide) or older (methotrexate, azathioprine) immunosuppresive agents are finding new roles in the management of vasculitides. For patients with severe vasculitis, short-term use of cytotoxic agents, such as cyclophosphamide, alone or in combination with biologic agents, may expedite remission, which could then be better maintained with other, less toxic (and less expensive) immunosuppressive agents, such as methotrexate, azathioprine, mycophenolate mofetil, and leflunamide. For patients with mild or moderately severe vasculitis, these latter agents alone may be adequate. New therapeutic studies in vasculitis should better address the impact of therapy on health-related quality of life and its long-term toxicity.

  15. Magnetic resonance angiography in suspected cerebral vasculitis

    Energy Technology Data Exchange (ETDEWEB)

    Demaerel, Philippe; De Ruyter, Nele; Wilms, Guido [Department of Radiology, Universitair Ziekenhuis, KU Leuven, 3000, Leuven (Belgium); Maes, Frederik [Department of Medical Imaging Computing, Universitair Ziekenhuis, KU Leuven, 3000, Leuven (Belgium); Velghe, Beatrijs [Department of Radiology, Ziekenhuis Oost-Limburg, Schiepse Bos 6, 3600, Genk (Belgium)

    2004-06-01

    The purpose of this study was to determine the technical capacity and diagnostic accuracy of 3D time-of-flight magnetic resonance angiography (MRA) in suspected cerebral vasculitis in a retrospective analysis of MRA and digital subtraction angiography (DSA) in 14 young patients with clinical and/or radiological suspicion of cerebral vasculitis. A total of nine arteries were evaluated in each patient. Consensus review of DSA by three observers was the reference standard. The sensitivity for detecting a stenosis varied from 62 to 79% for MRA and from 76 to 94% for DSA, depending on the observer. The specificity for detecting a stenosis varied from 83 to 87% for MRA and from 83 to 97% for DSA. Using the criterion ''more than two stenoses in at least two separate vascular distributions'' to consider the examination as being true positive, the false-positive rates for MRA and DSA were comparable. MRA plays a role as the first angiographical examination in the diagnostic work-up of suspected cerebral vasculitis. When more than two stenoses in at least two separate vascular distributions are depicted on MRA, DSA is not expected to add a significant diagnostic contribution in a patient with suspected cerebral vasculitis. DSA remains necessary when MRA is normal or when less than three stenoses are seen. (orig.)

  16. Pediatric ischemic stroke due to dengue vasculitis.

    Science.gov (United States)

    Nanda, Subrat Kumar; Jayalakshmi, Sita; Mohandas, Surath

    2014-10-01

    Dengue infection is an important arboviral infection in southeast Asia, especially in India. Neurological manifestations of dengue are increasingly recognized. We report an ischemic stroke due to dengue vasculitis in an 8-year-old child. We present a girl with a short febrile illness followed by episodic severe headache, with gradually progressive hemiparesis and visual impairment. Her brain magnetic resonance imaging revealed multiple infarctions in the anterior and posterior circulation. The magnetic resonance angiogram revealed irregular narrowing of bilateral middle cerebral arteries, right anterior cerebral artery, left posterior cerebral, and bilateral vertebral arteries suggestive of vasculitis. Her dengue serology was strongly positive for immunoglobulin M with 68.9 panbio units. The rest of the evaluation for pediatric stroke was unremarkable. She was treated with intravenous followed by oral corticosteroids and recovered totally with resolution of vasculitis on magnetic resonance angiogram over the next 3 months. This child illustrates possible immune-mediated vasculitis caused by dengue infection which is rather a rare presentation in a child who subsequently recovered well. One should consider dengue in childhood strokes in endemic regions. Copyright © 2014 Elsevier Inc. All rights reserved.

  17. Cryoglobulinemic vasculitis preceding diagnosis of Carney Complex

    DEFF Research Database (Denmark)

    Glerup, Mia; Stausbøl-Grøn, Brian; Heuck, Carsten

    2014-01-01

    with classical symptoms of a stroke. MRI revealed small middle cerebral artery infarctions but no signs of schwannomas or vasculitis on the angiography. An echocardiography revealed an obstructive tumor in the left atrium, 5-6 centimeters in diameter, diagnosed as myxoma, which was surgically removed...

  18. Magnetic resonance angiography in suspected cerebral vasculitis

    International Nuclear Information System (INIS)

    Demaerel, Philippe; De Ruyter, Nele; Wilms, Guido; Maes, Frederik; Velghe, Beatrijs

    2004-01-01

    The purpose of this study was to determine the technical capacity and diagnostic accuracy of 3D time-of-flight magnetic resonance angiography (MRA) in suspected cerebral vasculitis in a retrospective analysis of MRA and digital subtraction angiography (DSA) in 14 young patients with clinical and/or radiological suspicion of cerebral vasculitis. A total of nine arteries were evaluated in each patient. Consensus review of DSA by three observers was the reference standard. The sensitivity for detecting a stenosis varied from 62 to 79% for MRA and from 76 to 94% for DSA, depending on the observer. The specificity for detecting a stenosis varied from 83 to 87% for MRA and from 83 to 97% for DSA. Using the criterion ''more than two stenoses in at least two separate vascular distributions'' to consider the examination as being true positive, the false-positive rates for MRA and DSA were comparable. MRA plays a role as the first angiographical examination in the diagnostic work-up of suspected cerebral vasculitis. When more than two stenoses in at least two separate vascular distributions are depicted on MRA, DSA is not expected to add a significant diagnostic contribution in a patient with suspected cerebral vasculitis. DSA remains necessary when MRA is normal or when less than three stenoses are seen. (orig.)

  19. SLG(Single-Line-to-Ground Fault Location in NUGS(Neutral Un-effectively Grounded System

    Directory of Open Access Journals (Sweden)

    Zhang Wenhai

    2018-01-01

    Full Text Available This paper reviews the SLG(Single-Line-to-Ground fault location methods in NUGS(Neutral Un-effectively Grounded System, including ungrounded system, resonant grounded system and high-resistance grounded system which are widely used in Northern Europe and China. This type of fault is hard to detect and location because fault current is the sum of capacitance current of the system which is always small(about tens of amperes. The characteristics of SLG fault in NUGS and the fault location methods are introduced in the paper.

  20. Systemic lupus erythematosus complicated by intestinal vasculitis and pneumatosis intestinalis.

    Science.gov (United States)

    Marinello, Débora Karine; Rafael, Daniane; Paiva, Eduardo Dos Santos; Dominoni, Robson Luiz

    2010-01-01

    Gastrointestinal manifestations in systemic lupus erythematosus (SLE) are not uncommon. Non specific symptoms are often observed, such as abdominal pain, nausea, vomiting and diarrhea. On the other hand, pneumatosis cystoides intestinalis, which is characterized by multiple gas-filled cysts located throughout the intestinal wall, is a rare condition in SLE. We describe a case of a 20-year-old man who was admitted with fever, weight loss, headache and arthralgia and had a diagnosis of systemic lupus erythematosus. During his hospital stay, he developed abdominal symptoms that suggested intestinal vasculitis. The computed tomography of the abdomen showed the double halo sign, or target sign and pneumatosis cystoides intestinalis. The patient presented complete recovery after conservative treatment, with intestinal rest and total parenteral nutrition.

  1. A clinical approach to the diagnosis of retinal vasculitis.

    Science.gov (United States)

    El-Asrar, Ahmed M Abu; Herbort, Carl P; Tabbara, Khalid F

    2010-04-01

    Retinal vasculitis is a sight-threatening inflammatory eye condition that involves the retinal vessels. Detection of retinal vasculitis is made clinically, and is confirmed with the help of fundus fluorescein angiography. Active vascular disease is characterized by exudates around retinal vessels resulting in white sheathing or cuffing of the affected vessels. In this review, a practical approach to the diagnosis of retinal vasculitis is discussed based on ophthalmoscopic and fundus fluorescein angiographic findings.

  2. A classic mimicker of systemic vasculitis.

    Science.gov (United States)

    Moreno-Ariño, Marc; Ortiz-Santamaria, Vera; Deudero Infante, Aída; Ayats Delgado, Montserrat; Novell Teixidó, Francesc

    2016-01-01

    Embolic and constitutional manifestations of intracavitary cardiac tumors are included within the classic mimickers of systemic vasculitis, especially in those in which there are no cardiac manifestations. We present a case report of atrial myxoma in which the patient only presented systemic symptoms and in whom an initial diagnostic approach of systemic vasculitis was made. We also performed a literature search of the cases described. A case report of atrial myxoma with atypical presentation manifested as a systemic disease with no concomitant cardiac symptoms is described. The case report is discussed and 11 cases of atrial myxoma pseudovasculitis described in the literature are reviewed, emphasizing their similarities and differences. Constitutional symptoms and cutaneous manifestations were the most common. Most of the cases showed partial response to glucococorticosteroid treatment, reinforcing the theory of the inflammatory role in its pathogenesis. Mean delayed time to diagnosis was 12.27 months. Atrial myxoma is a systemic vasculitis mimicker, this being difficult to diagnose in the absence of cardiac manifestations. This delay in diagnosis entails serious complications. Copyright © 2015 Elsevier España, S.L.U. y Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  3. Hanford Single-Shell Tank Leak Causes and Locations - 241-B Farm

    Energy Technology Data Exchange (ETDEWEB)

    Girardot, Crystal L. [Washington River Protection Systems, Richland, WA (United States); Harlow, Donald G. [Washington River Protection Systems, Richland, WA (United States)

    2013-07-11

    This document identifies 241-B Tank Farm (B Farm) leak cause and locations for the 100 series leaking tank (241-B-107) identified in RPP-RPT-49089, Hanford B-Farm Leak Inventory Assessments Report. This document satisfies the B Farm portion of the target (T04) in the Hanford Federal Facility Agreement and Consent Order milestone M-045-91F.

  4. Hanford Single-Shell Tank Leak Causes and Locations - 241-B Farm

    International Nuclear Information System (INIS)

    Girardot, Crystal L.; Harlow, Donald G.

    2013-01-01

    This document identifies 241-B Tank Farm (B Farm) leak cause and locations for the 100 series leaking tank (241-B-107) identified in RPP-RPT-49089, Hanford B-Farm Leak Inventory Assessments Report. This document satisfies the B Farm portion of the target (T04) in the Hanford Federal Facility Agreement and Consent Order milestone M-045-91F

  5. Single-Side Two-Location Spotlight Imaging for Building Based on MIMO Through-Wall-Radar

    Directory of Open Access Journals (Sweden)

    Yong Jia

    2016-09-01

    Full Text Available Through-wall-radar imaging is of interest for mapping the wall layout of buildings and for the detection of stationary targets within buildings. In this paper, we present an easy single-side two-location spotlight imaging method for both wall layout mapping and stationary target detection by utilizing multiple-input multiple-output (MIMO through-wall-radar. Rather than imaging for building walls directly, the images of all building corners are generated to speculate wall layout indirectly by successively deploying the MIMO through-wall-radar at two appropriate locations on only one side of the building and then carrying out spotlight imaging with two different squint-views. In addition to the ease of implementation, the single-side two-location squint-view detection also has two other advantages for stationary target imaging. The first one is the fewer multi-path ghosts, and the second one is the smaller region of side-lobe interferences from the corner images in comparison to the wall images. Based on Computer Simulation Technology (CST electromagnetic simulation software, we provide multiple sets of validation results where multiple binary panorama images with clear images of all corners and stationary targets are obtained by combining two single-location images with the use of incoherent additive fusion and two-dimensional cell-averaging constant-false-alarm-rate (2D CA-CFAR detection.

  6. Location and direction specificity in motion direction learning associated with a single-level method of constant stimuli.

    Science.gov (United States)

    Xiong, Ying-Zi; Xie, Xin-Yu; Yu, Cong

    2016-02-01

    Recent studies reported significantly less location specificity in motion direction learning than in previous classical studies. The latter performed training with the method of constant stimuli containing a single level of direction difference. In contrast the former used staircase methods that varied the direction difference trial by trial. We suspect that extensive practice with a single direction difference could allow an observer to use some subtle local cues for direction discrimination. Such local cues may be unavailable at a new stimulus location, leading to higher location specificity. To test this hypothesis, we jittered slightly the directions of a stimulus pair by the same amount while keeping the direction difference constant, so as to disturb the potential local cues. We observed significantly more transfer of learning to untrained locations. The local cue effects may also explain the recent controversies regarding the finding that foveal motion direction learning becomes significantly more transferrable to a new direction with TPE (training-plus-exposure) training. One specific study by Zili Liu and collaborators that challenges this finding also used a single-level direction difference for training. We first replicated their results. But we found that if the directions of the stimulus pair were again jittered while the direction difference was kept constant, motion direction learning transferred significantly more to an orthogonal direction with TPE training. Our results thus demonstrate the importance of using appropriate psychophysical methods in training to reduce local-cue related specificity in perceptual learning. Copyright © 2015 Elsevier Ltd. All rights reserved.

  7. Rare Case of Vasculitis of the Hepatic Artery.

    Science.gov (United States)

    Mali, Padmavathi; Muduganti, Sudheer R; Goldberg, Jerry

    2015-12-01

    Vasculitis is an accumulation of inflammatory leucocytes in the blood vessels with reactive damage to mural structures. Isolated vasculitis of the gastrointestinal tract without systemic involvement is rare. We report a unique case of a female patient who presented with abdominal pain, and was found, on serology, to have elevated inflammatory markers without autoantibodies. A computed tomography scan of the abdomen and pelvis was suggestive of vasculitis of the hepatic artery. To the best of our knowledge, this is the first case, to date, of vasculitis of hepatic artery. © 2015 Marshfield Clinic.

  8. Endoscopic Findings of Upper Gastrointestinal Involvement in Primary Vasculitis.

    Science.gov (United States)

    Gong, Eun Jeong; Kim, Do Hoon; Chun, Joo Hyun; Ahn, Ji Yong; Choi, Kwi-Sook; Jung, Kee Wook; Lee, Jeong Hoon; Choi, Kee Don; Song, Ho June; Lee, Gin Hyug; Jung, Hwoon-Yong; Kim, Jin Ho; Song, In Hye; Kim, Yong-Gil

    2016-07-15

    Gastrointestinal involvement in vasculitis may result in life-threatening complications. However, its variable clinical presentations and endoscopic features, and the rarity of the disease, often result in delayed diagnosis. Clinical characteristics, endoscopic features, and histopathological findings were reviewed from medical records. Of 6,477 patients with vasculitis, 148 were diagnosed as primary vasculitis with upper gastrointestinal involvement. Of these, 21 cases (14.2%) were classified as large-vessel vasculitis, 17 cases (11.5%) as medium-vessel vasculitis, and 110 cases (74.3%) as small-vessel vasculitis. According to the specific diagnosis, IgA vasculitis (Henoch-Schönlein purpura) was the most common diagnosis (56.8%), followed by Takayasu arteritis (14.1%), microscopic polyangiitis (10.1%), and polyarteritis nodosa (6.8%). Gastrointestinal symptoms were present in 113 subjects (76.4%), with abdominal pain (78.8%) the most common symptom. Erosion and ulcers were striking endoscopic features, and the second portion of the duodenum was the most frequently involved site. Biopsy specimens were obtained from 124 patients, and only eight (5.4%) presented histopathological signs of vasculitis. Diagnosis of vasculitis involving the upper gastrointestinal tract is difficult. Because of the widespread use of endoscopy, combining clinical features with endoscopic findings may facilitate making appropriate diagnoses; however, the diagnostic yield of endoscopic biopsy is low.

  9. A Case of Leukocytoclastic Vasculitis Caused by Listeria monocytogenes Bacteremia

    Directory of Open Access Journals (Sweden)

    Daniel R. Bunker

    2016-01-01

    Full Text Available Importance. Infections can cause leukocytoclastic vasculitis. Observations. We report the case of a patient with a left ventricular assist device who presented with acute kidney injury and biopsy proven leukocytoclastic vasculitis. Blood cultures grew Listeria monocytogenes. The patient’s rash improved with treatment of the underlying Listeria infection. Conclusion. Clinicians should be aware that there are a number of broad categories of disease associated with the histologic finding of vasculitis, including infection. It is important to keep in mind the risk factors of a particular patient when formulating a differential diagnosis. This is the first reported case of Listeria bacteremia causing leukocytoclastic vasculitis.

  10. Neglect of bandwidth of Odontocetes echo location clicks biases propagation loss and single hydrophone population estimates

    NARCIS (Netherlands)

    Ainslie, M.A.

    2013-01-01

    Passive acoustic monitoring with a single hydrophone has been suggested as a cost-effective method to monitor population density of echolocating marine mammals, by estimating the distance at which the hydrophone is able to intercept the echolocation clicks and distinguish these from the background.

  11. Hanford Single-Shell Tank Leak Causes and Locations - 241-T Farm

    Energy Technology Data Exchange (ETDEWEB)

    Girardot, Crystal L.; Harlow, Donald G.

    2014-05-15

    This document identifies 241-T Tank Farm (T Farm) leak causes and locations for the 100 series leaking tanks (241-T-106 and 241-T-111) identified in RPP-RPT-55084, Rev. 0, Hanford 241-T Farm Leak Inventory Assessment Report. This document satisfies the T Farm portion of the target (T04) in the Hanford Federal Facility Agreement and Consent Order milestone M-045-91F.

  12. Hanford Single-Shell Tank Leak Causes and Locations - 241-U Farm

    Energy Technology Data Exchange (ETDEWEB)

    Girardot, Crystal L.; Harlow, Donald G.

    2013-12-02

    This document identifies 241-U Tank Farm (U Farm) leak causes and locations for the 100 series leaking tanks (241-U-104, 241-U-110, and 241-U-112) identified in RPP-RPT-50097, Rev. 0, Hanford 241-U Farm Leak Inventory Assessment Report. This document satisfies the U-Farm portion of the target (T04) in the Hanford Federal Facility Agreement and Consent Order milestone M-045-91F.

  13. Hanford Single-Shell Tank Leak Causes and Locations - 241-C Farm

    Energy Technology Data Exchange (ETDEWEB)

    Girardot, Crystal L.; Harlow, Donald G.

    2013-07-30

    This document identifies 241-C Tank Farm (C Farm) leak causes and locations for the 100 series leaking tanks (241-C-101 and 241-C-105) identified in RPP-RPT-33418, Rev. 2, Hanford C-Farm Leak Inventory Assessments Report. This document satisfies the C Farm portion of the target (T04) in the Hanford Federal Facility Agreement and Consent Order milestone M-045-91F.

  14. Hanford Single Shell Tank Leak Causes and Locations - 241-TX Farm

    Energy Technology Data Exchange (ETDEWEB)

    Girardot, C. L.; Harlow, D> G.

    2014-07-22

    This document identifies 241-TX Tank Farm (TX Farm) leak causes and locations for the 100 series leaking tanks (241-TX-107 and 241-TX-114) identified in RPP-RPT-50870, Rev. 0, Hanford 241-TX Farm Leak Inventory Assessment Report. This document satisfies the TX Farm portion of the target (T04) in the Hanford Federal Facility Agreement and Consent Order milestone M-045-91F.

  15. Hanford Single-Shell Tank Leak Causes and Locations - 241-A Farm

    Energy Technology Data Exchange (ETDEWEB)

    Girardot, Crystal L.; Harlow, Donald G.

    2013-09-10

    This document identifies 241-A Tank Farm (A Farm) leak causes and locations for the 100 series leaking tanks (241-A-104 and 241-A-105) identified in RPP-ENV-37956, Hanford A and AX Farm Leak Assessment Report. This document satisfies the A Farm portion of the target (T04) in the Hanford Federal Facility Agreement and Consent Order milestone M-045-91F.

  16. Single Tracking Location Methods Suppress Speckle Noise in Shear Wave Velocity Estimation

    OpenAIRE

    Elegbe, Etana C.; McAleavey, Stephen A.

    2013-01-01

    In ultrasound-based elastography methods, the estimation of shear wave velocity typically involves the tracking of speckle motion due to an applied force. The errors in the estimates of tissue displacement, and thus shear wave velocity, are generally attributed to electronic noise and decorrelation due to physical processes. We present our preliminary findings on another source of error, namely, speckle-induced bias in phase estimation. We find that methods that involve tracking in a single l...

  17. The effect of accelerometer location on the classification of single-site forearm mechanomyograms

    Directory of Open Access Journals (Sweden)

    Sejdić Ervin

    2010-06-01

    Full Text Available Abstract Background Recently, pattern recognition methods have been deployed in the classification of multiple activation states from mechanomyogram (MMG signals for the purpose of controlling switching interfaces. Given the propagative properties of MMG signals, it has been suggested that MMG classification should be robust to changes in sensor placement. Nonetheless, this purported robustness remains speculative to date. This study sought to quantify the change in classification accuracy, if any, when a classifier trained with MMG signals from the muscle belly, is subsequently tested with MMG signals from a nearby location. Methods An arrangement of 5 accelerometers was attached to the flexor carpi radialis muscle of 12 able-bodied participants; a reference accelerometer was located over the muscle belly, two peripheral accelerometers were positioned along the muscle's transverse axis and two more were aligned to the muscle's longitudinal axis. Participants performed three classes of muscle activity: wrist flexion, wrist extension and semi-pronation. A collection of time, frequency and time-frequency features were considered and reduced by genetic feature selection. The classifier, trained using features from the reference accelerometer, was tested with signals from the longitudinally and transversally displaced accelerometers. Results Classification degradation due to accelerometer displacement was significant for all participants, and showed no consistent trend with the direction of displacement. Further, the displaced accelerometer signals showed task-dependent de-correlations with respect to the reference accelerometer. Conclusions These results indicate that MMG signal features vary with spatial location and that accelerometer displacements of only 1-2 cm cause sufficient feature drift to significantly diminish classification accuracy. This finding emphasizes the importance of consistent sensor placement between MMG classifier training

  18. Hanford Single-Shell Tank Leak Causes and Locations - 241-BY and 241-TY Farm

    Energy Technology Data Exchange (ETDEWEB)

    Girardot, Crystal L.; Harlow, Donald G.

    2014-09-04

    This document identifies 241-BY Tank Farm (BY Farm) and 241-TY Tank Farm (TY Farm) lead causes and locations for the 100 series leaking tanks (241-BY-103, 241-TY-103, 241-TY-104, 241-TY-105 and 241-TY-106) identified in RPP-RPT-43704, Hanford BY Farm Leak Assessments Report, and in RPP-RPT-42296, Hanford TY Farm Leak Assessments Report. This document satisfies the BY and TY Farm portion of the target (T04) in the Hanford Federal Facility Agreement and Consent Order milestone M-045-91F.

  19. Hanford Single-Shell Tank Leak Causes and Locations - 241-BY and 241-TY Farm

    Energy Technology Data Exchange (ETDEWEB)

    Girardot, Crystal L.; Harlow, Donald G.

    2013-11-19

    This document identifies 241-BY Tank Farm (BY Farm) and 241-TY Tank Farm (TY Farm) leak causes and locations for the 100 series leaking tanks (241-BY-103, 241-TY-103, 241-TY-104, 241-TY-105, and 241-TY-106) identified in RPP-RPT-43704, Hanford BY Farm Leak Assessments Report, and in RPP-RPT-42296, Hanford TY Farm Leak Assessments Report. This document satisfies the BY and TY Farm portion of the target (T04) in Hanford Federal Facility Agreement and Consent Order milestone M-045-91F.

  20. Hanford Single-Shell Tank Leak Causes and Locations - 241-SX Farm

    Energy Technology Data Exchange (ETDEWEB)

    Girardot, Crystal L. [Washington River Protection Solutions (United States); Harlow, Donald G. [Washington River Protection Solutions (United States)

    2014-01-08

    This document identifies 241-SX Tank Farm (SX Farm) leak causes and locations for the 100 series leaking tanks (241-SX-107, 241-SX-108, 241-SX-109, 241-SX-111, 241-SX-112, 241-SX-113, 241-SX-114, and 241-SX-115) identified in RPP-ENV-39658, Rev. 0, Hanford SX-Farm Leak Assessments Report. This document satisfies the SX Farm portion of the target (T04) in the Hanford Federal Facility Agreement and Consent Order milestone M-045-91F.

  1. Detecting and locating light atoms from high-resolution STEM images: The quest for a single optimal design.

    Science.gov (United States)

    Gonnissen, J; De Backer, A; den Dekker, A J; Sijbers, J; Van Aert, S

    2016-11-01

    In the present paper, the optimal detector design is investigated for both detecting and locating light atoms from high resolution scanning transmission electron microscopy (HR STEM) images. The principles of detection theory are used to quantify the probability of error for the detection of light atoms from HR STEM images. To determine the optimal experiment design for locating light atoms, use is made of the so-called Cramér-Rao Lower Bound (CRLB). It is investigated if a single optimal design can be found for both the detection and location problem of light atoms. Furthermore, the incoming electron dose is optimised for both research goals and it is shown that picometre range precision is feasible for the estimation of the atom positions when using an appropriate incoming electron dose under the optimal detector settings to detect light atoms. Copyright © 2016 Elsevier B.V. All rights reserved.

  2. The impact of vasculitis on patients’ social participation and friendships

    Science.gov (United States)

    Carpenter, Delesha M.; Meador, Amy E.; Elstad, Emily A.; Hogan, Susan L.; DeVellis, Robert F.

    2013-01-01

    Objectives Our objective is to explore how vasculitis, affects patients’ friendships and social participation. Methods Vasculitis patients (n=221) completed an online questionnaire that asked if, and how, relationships with friends have changed since receiving a vasculitis diagnosis. Participants’ written responses were imported into Atlas.ti, and two independent researchers used both structured and unstructured coding to identify themes. After reaching 100% consensus on the themes present in each participant’s responses, the coders determined how themes were interrelated across participants. Results Over half of patients (52%) expressed that vasculitis negatively impacted their friendships and 25% noted a negative impact on their social participation. At limes, this negative impact was related to structural changes in patients’ social networks due to loss of friendships. Reduced social participation was also associated with friends’ inability to understand vasculitis and its effects, vasculitis-related fatigue, and lifestyle changes such as not being able to drink alcohol and avoiding infection-prone events. Additionally, patients withdrew from social engagements due to fatigue or because of physical symptoms and side effects. Conclusion The unique circumstances associated with a rare chronic illness like vasculitis can create significant barriers to friendships, including loss of these relationships. Interventions designed to help patients cope with the social impact of vasculitis are implicated, especially if they increase patients’ ability to engage in dialogue about their illness with their friends. PMID:22325346

  3. The impact of vasculitis on patients' social participation and friendships.

    Science.gov (United States)

    Carpenter, Delesha M; Meador, Amy E; Elstad, Emily A; Hogan, Susan L; DeVellis, Robert F

    2012-01-01

    Our objective is to explore how vasculitis affects patients' friendships and social participation. Vasculitis patients (n=221) completed an online questionnaire that asked if, and how, relationships with friends have changed since receiving a vasculitis diagnosis. Participants' written responses were imported into Atlas.ti, and two independent researchers used both structured and unstructured coding to identify themes. After reaching 100% consensus on the themes present in each participant's responses, the coders determined how themes were interrelated across participants. Over half of patients (52%) expressed that vasculitis negatively impacted their friendships and 25% noted a negative impact on their social participation. At times, this negative impact was related to structural changes in patients' social networks due to loss of friendships. Reduced social participation was also associated with friends' inability to understand vasculitis and its effects, vasculitis-related fatigue, and lifestyle changes such as not being able to drink alcohol and avoiding infection-prone events. Additionally, patients withdrew from social engagements due to fatigue or because of physical symptoms and side effects. The unique circumstances associated with a rare chronic illness like vasculitis can create significant barriers to friendships, including loss of these relationships. Interventions designed to help patients cope with the social impact of vasculitis are implicated, especially if they increase patients' ability to engage in dialogue about their illness with their friends.

  4. Systemic Vasculitis During the Course of Systemic Sclerosis

    Science.gov (United States)

    Quéméneur, Thomas; Mouthon, Luc; Cacoub, Patrice; Meyer, Olivier; Michon-Pasturel, Ulrique; Vanhille, Philippe; Hatron, Pierre-Yves; Guillevin, Loïc; Hachulla, Eric

    2013-01-01

    Abstract Although the presence of antineutrophil cytoplasmic antibodies (ANCA) has been reported in patients with systemic sclerosis (SSc), the association of SSc and systemic vasculitis has rarely been described. We obtained information on cases of systemic vasculitis associated with SSc in France from the French Vasculitis Study Group and all members of the French Research Group on Systemic Sclerosis. We identified 12 patients with systemic vasculitis associated with SSc: 9 with ANCA-associated systemic vasculitis (AASV) and 3 with mixed cryoglobulinemia vasculitis (MCV). In all AASV patients, SSc was of the limited type. The main complication of SSc was pulmonary fibrosis. Only 2 patients underwent a D-penicillamine regimen before the occurrence of AASV. The characteristics of AASV were microscopic polyangiitis (n = 7) and renal limited vasculitis (n = 2). Anti-myeloperoxidase antibodies were found in 8 of the 9 patients. The Five Factor Score was above 1 in 3 of the 9 patients. Of the 3 patients with MCV, Sjögren syndrome was confirmed in 2. We compared our findings with the results of a literature review (42 previously reported cases of AASV with SSc). Although rare, vasculitis is a complication of SSc. AASV is the most frequent type, and its diagnosis can be challenging when the kidney is injured. Better awareness of this rare association could facilitate earlier diagnosis and appropriate management to reduce damage. PMID:23263715

  5. Cerebral vasculitis and its simulators. Diagnostic and interventional radiology

    International Nuclear Information System (INIS)

    Ishimaru, Hideki; Nakajima, Kazuaki; Matsuoka, Yohjiro; Sakamoto, Ayami; Sakugawa, Takayuki; Morikawa, Minoru; Inuzuka, Michiko; Kimura, Seigo

    2009-01-01

    Cerebral vasculitis, although rare in general, is an important cause of cerebral ischemia, because the treatment strategy is different from that of usual cerebral infarction. On the other hand, a variety of common and uncommon nonvasculitic disorders may mimic vasculitis angiographically or clinically. It is also important to distinguish these vasculitis simulators from true vasculitis in order to avoid the unnecessary and harmful side effects of corticosteroid and immunosuppressant, the mainstay of drug therapy for vasculitis. The diagnosis is often difficult. Angiography is expected to demonstrate the underlying vascular pathology; however, many vasculitides affect small arteries beyond the spatial resolution of conventional angiography. The most important role of the diagnostic imaging is the identification of brain lesions consistent with cerebral vasculitis. Multiple lesions in more than one vascular territory should raise this suspicion, although no specific pattern for this entity exists. In large and medium-sized vessel vasculitis, MRI can directly demonstrate mural thickening and enhancement, which is considered to specific for active inflammation. We propose here a classification for cerebral vasculitis and simulators according to the size of the affected brain vessels, which would help us to make a differential diagnosis. We also review the endovascular techniques for this entity, and its indications. (author)

  6. Petechiae and vasculitis in asymptomatic primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Diederichsen, H; Sørensen, P G; Mickley, H

    1985-01-01

    vasculitis in all three patients. Direct immunofluorescent studies showed deposits of IgM, C3 and fibrinogen in and around the walls of the small vessels of the skin of two and C3 as well as fibrinogen in one patient. It is suggested that leukocytoclastic vasculitis may be a microscopic feature...

  7. Pathogenesis of ANCA-Associated Vasculitis, an Update

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.

    2011-01-01

    Clinical observations, including a report of neonatal vasculitis occurring in a child born from a mother with anti-neutrophil cytoplasmic antibody directed to myeloperoxidase (MPO-ANCA)-associated vasculitis, suggest a pathogenic role for ANCA. Such a role is supported by in vitro experimental data

  8. Surgical Complications of Hemorrhagic Vasculitis in a Child

    Directory of Open Access Journals (Sweden)

    E. Y. Dyakonova

    2015-01-01

    Full Text Available Hemorrhagic vasculitis is a hematological disorder, which is often accompanied by abdominal pain syndrome and blood in stool, which is why it requires differential diagnosis with acute surgical pathology. The article presents clinical follow-up of a patient with hemorrhagic vasculitis complicated by intestinal intussusception and developed mesenteric thrombosis, which required emergency surgical intervention. 

  9. 1D layered velocity models and microseismic event locations: synthetic examples for a case with a single linear receiver array

    Science.gov (United States)

    Akram, Jubran; Eaton, David W.

    2017-10-01

    We discuss various aspects of 1D velocity-model building for application to microseismic data analysis. We generate simple synthetic example data using a widely used single linear array geometry. The synthetic data contain 30 sources with known locations for a reference model based on previous studies of the Barnett shale. We investigate several key factors that should be considered, including selection of the calibration technique, inclusion of a priori information such as lateral heterogeneity and parameter ranges, and choice of algorithm for travel time computations. For the source-receiver geometry considered here, hypocenter location errors (±6 m in X and ±12 m in Z) can result from differently calibrated models only and without including the errors in picked arrival times and polarization estimates. We find that the errors in hypocenter locations are reduced (±3 m in X and ±6 m in Z) when a model calibrated with multiple shots simultaneously is used. Using four different models (vertical fault, dipping layers, channels, and these effects combined), we demonstrate that systematic errors in hypocenter locations can result when a 1D layered model is used in lieu of a laterally heterogeneous subsurface. Finally, we show that event locations from a velocity model calibrated using direct-arrival times are more stable than from a model calibrated with first-arrival times.

  10. Imaging of systemic vasculitis in childhood

    International Nuclear Information System (INIS)

    Soliman, Magdy; Laxer, Ronald; Yeung, Rae; Manson, David; Doria, Andrea S.

    2015-01-01

    The term ''systemic vasculitis'' encompasses a diverse set of diseases linked by the presence of blood-vessel inflammation that are often associated with critical complications. These diseases are uncommon in childhood and are frequently subjected to a delayed diagnosis. Although the diagnosis and treatment may be similar for adult and childhood systemic vasculitides, the prevalence and classification vary according to the age group under investigation. For example, Kawasaki disease affects children while it is rarely encountered in adults. In 2006, the European League Against Rheumatism (EULAR) and the Pediatric Rheumatology European Society (PReS) proposed a classification system for childhood vasculitis adopting the system devised in the Chapel Hill Consensus Conference in 1993, which categorizes vasculitides according to the predominant size of the involved blood vessels into small, medium and large vessel diseases. Currently, medical imaging has a pivotal role in the diagnosis of vasculitis given recent developments in the imaging of blood vessels. For example, early diagnosis of coronary artery aneurysms, a serious complication of Kawasaki disease, is now possible by magnetic resonance imaging (MRI) of the heart and multidetector computed tomography (MDCT); positron emission tomography/CT (PET/CT) helps to assess active vascular inflammation in Takayasu arteritis. Our review offers a unique approach using the integration of the proposed classification criteria for common systemic childhood vasculitides with their most frequent imaging findings, along with differential diagnoses and an algorithm for diagnosis based on common findings. It should help radiologists and clinicians reach an early diagnosis, therefore facilitating the ultimate goal of proper management of affected children. (orig.)

  11. Imaging of systemic vasculitis in childhood

    Energy Technology Data Exchange (ETDEWEB)

    Soliman, Magdy [The Hospital for Sick Children, Department of Medical Imaging, Toronto, ON (Canada); Cairo University, Department of Medical Imaging, Cairo (Egypt); Laxer, Ronald; Yeung, Rae [The Hospital for Sick Children, Department of Rheumatology, Toronto, ON (Canada); Manson, David; Doria, Andrea S. [The Hospital for Sick Children, Department of Medical Imaging, Toronto, ON (Canada)

    2015-08-15

    The term ''systemic vasculitis'' encompasses a diverse set of diseases linked by the presence of blood-vessel inflammation that are often associated with critical complications. These diseases are uncommon in childhood and are frequently subjected to a delayed diagnosis. Although the diagnosis and treatment may be similar for adult and childhood systemic vasculitides, the prevalence and classification vary according to the age group under investigation. For example, Kawasaki disease affects children while it is rarely encountered in adults. In 2006, the European League Against Rheumatism (EULAR) and the Pediatric Rheumatology European Society (PReS) proposed a classification system for childhood vasculitis adopting the system devised in the Chapel Hill Consensus Conference in 1993, which categorizes vasculitides according to the predominant size of the involved blood vessels into small, medium and large vessel diseases. Currently, medical imaging has a pivotal role in the diagnosis of vasculitis given recent developments in the imaging of blood vessels. For example, early diagnosis of coronary artery aneurysms, a serious complication of Kawasaki disease, is now possible by magnetic resonance imaging (MRI) of the heart and multidetector computed tomography (MDCT); positron emission tomography/CT (PET/CT) helps to assess active vascular inflammation in Takayasu arteritis. Our review offers a unique approach using the integration of the proposed classification criteria for common systemic childhood vasculitides with their most frequent imaging findings, along with differential diagnoses and an algorithm for diagnosis based on common findings. It should help radiologists and clinicians reach an early diagnosis, therefore facilitating the ultimate goal of proper management of affected children. (orig.)

  12. Managing refractory cryoglobulinemic vasculitis: challenges and solutions

    Directory of Open Access Journals (Sweden)

    Ostojic P

    2017-05-01

    Full Text Available Predrag Ostojic, Ivan R Jeremic Institute of Rheumatology, School of Medicine, University of Belgrade, Belgrade, Serbia Abstract: Cryoglobulinemia is thought to be a rare condition. It may be an isolated disorder or secondary to a particular disease. According to immunoglobulin composition, cryoglobulinemia is classified into three types. In mixed cryoglobulinemia (types II and III, vascular deposition of cryoglobulin-containing immune complexes and complement may induce a clinical syndrome, characterized by systemic vasculitis and inflammation – cryoglobulinemic vasculitis (CryoVas. Most common clinical manifestations in CryoVas are skin lesions (orthostatic purpura and ulcers, weakness, peripheral neuropathy, Raynaud’s phenomenon, sicca syndrome, membranoproliferative glomerulonephritis, and arthralgia and seldom arthritis. In patients with mixed cryoglobulinemia, prevalence of anti-hepatitis C virus (HCV antibodies and/or HCV RNA, detected by polymerase chain reaction (PCR, is reported to be up to 90%, indicating a significant role of HCV in the development of this condition. The goals of therapy for mixed cryoglobulinemia include immunoglobulin level reduction and antigen elimination. CryoVas not associated with HCV infection should be treated according to treatment recommendations for small-vessel vasculitides. CryoVas associated with chronic HCV infection should be treated with antivirals along with immunosuppressive drugs, with or without plasmapheresis, depending on disease severity and organ involvement. Patients who do not respond to first-line therapy may achieve remission when treatment with rituximab is started as second-line therapy. In HCV-related CryoVas, antiviral therapy should be given along with rituximab in order to achieve complete or partial remission. Moreover, rituximab has proven to be a glucocorticoid-sparing medication. Other potential therapies for refractory CryoVas include mycophenolate mofetil and belimumab

  13. Cryoglobulinemic vasculitis in a patient with CREST syndrome.

    Science.gov (United States)

    Hurst, Rebecca L; Berianu, Florentina; Ginsburg, William W; Klein, Christopher J; Englestad, Janean K; Kennelly, Kathleen D

    2014-10-01

    Cryoglobulinemic vasculitis is a rare entity. Although it has been reported in diffuse systemic sclerosis, it has not been reported in calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia (CREST) syndrome. We report a patient with cryoglobulinemic vasculitis with CREST syndrome who did not have typical clinical features of vasculitis. This 58-year-old woman presented with mild generalized weakness and a diagnosis of CREST syndrome, which included Raynaud's syndrome, dysphagia and telangiectasias. She was positive for serum cryoglobulins, which led to a sural nerve biopsy. The biopsy results were consistent with cryoglobulinemic vasculitis. Cryoglobulinemic vasculitis has not been previously reported in CREST syndrome to our knowledge. Additionally, the patient also had limited clinical symptoms. Our patient displays the importance of checking for cryoglobulins and obtaining a nerve biopsy when the serum is positive. Both of these diagnostic tests were integral for directing appropriate treatment for this patient. Copyright © 2014 Elsevier Ltd. All rights reserved.

  14. pANCA-vasculitis associated with rectal adenocarcinoma.

    Science.gov (United States)

    Hommel, C; Rihova, Z; Mokaddem, F; Libotte, B

    2014-12-01

    We report the case of a 69-year-old male patient who was admitted for fever, dry cough, recurrent sinusitis with epistaxis, anorexia with weight loss of 20 kg over a 3-month period, myalgia, and mononeuritis multiplex. He was diagnosed with pANCA/anti-MPO associated vasculitis and rectal adenocarcinoma. The tumor was treated by surgical resection. Recurrence of vasculitis occurred during steroid tapering which prompted us to add Mycophenolate mofetyl. A complete remission was achieved. We conclude that in the present case the vasculitis was an independent disease, not a paraneoplastic phenomenon. We discuss the value of different ANCA serologies for diagnostics and follow-up, the epidemiology of vasculitis associated with malignancy, and the concept of vasculitis as a paraneoplastic syndrome.

  15. Renal paraneoplastic vasculitis complicating lung adenocarcinoma

    Directory of Open Access Journals (Sweden)

    Besma Ben Dhaou

    2014-01-01

    Full Text Available Renal paraneoplastic vasculitis (RNPV is rare. It can be revealed by glomerulonephritis, microaneurysms or renal failure. RPNV may precede the onset of the primary tumor, and treatment and prognosis depend on the etiology (primary tumor. A 54-year-old man who had a primary lung adenocarcinoma was admitted for nephrotic syndrome. The investigations revealed RNPV. The patient was treated with corticosteroids at high dose and cyclophosphamide with improvement of the renal condition; however, the patient died from worsening of his pulmonary neoplasia.

  16. Effect of advection on variations in zooplankton at a single location near Cabo Nazca, Peru

    Energy Technology Data Exchange (ETDEWEB)

    Smith, S L; Brink, K H; Santander, H; Cowles, T J; Huyer, A

    1980-04-01

    Temporal variations in the biomass and species composition of zooplankton at a single midshelf station in an upwelling area off Peru can be explained to a large extent by onshore-offshore advection in the upper 20 m of the water column. During periods of strong or sustained near-surface onshore flow, peaks in biomass of zooplankton were observed at midshelf and typically oceanic species of copepod were collected. In periods of offshore flow at the surface, a copepod capable of migrating into oxygen-depleted layers deeper than 30 m was collected. A simple translocation model of advection applied to the cross-shelf distribution of Paracalanus parvus suggests that the fluctuations in P. pavus observed in the midshelf time-series were closely related to onshore-offshore flow in the upper 20 m. Fluctuations in abundance of the numerically dominant copepod, Acartia tonsa, were apparently affected by near surface flow also. The population age-structure suggests that A. tonsa was growing at maximal rates, due in part to its positive feeding response to the dinoflagellate/diatom assemblage of phytoplankton.

  17. New var reconstruction algorithm exposes high var sequence diversity in a single geographic location in Mali.

    Science.gov (United States)

    Dara, Antoine; Drábek, Elliott F; Travassos, Mark A; Moser, Kara A; Delcher, Arthur L; Su, Qi; Hostelley, Timothy; Coulibaly, Drissa; Daou, Modibo; Dembele, Ahmadou; Diarra, Issa; Kone, Abdoulaye K; Kouriba, Bourema; Laurens, Matthew B; Niangaly, Amadou; Traore, Karim; Tolo, Youssouf; Fraser, Claire M; Thera, Mahamadou A; Djimde, Abdoulaye A; Doumbo, Ogobara K; Plowe, Christopher V; Silva, Joana C

    2017-03-28

    Encoded by the var gene family, highly variable Plasmodium falciparum erythrocyte membrane protein-1 (PfEMP1) proteins mediate tissue-specific cytoadherence of infected erythrocytes, resulting in immune evasion and severe malaria disease. Sequencing and assembling the 40-60 var gene complement for individual infections has been notoriously difficult, impeding molecular epidemiological studies and the assessment of particular var elements as subunit vaccine candidates. We developed and validated a novel algorithm, Exon-Targeted Hybrid Assembly (ETHA), to perform targeted assembly of var gene sequences, based on a combination of Pacific Biosciences and Illumina data. Using ETHA, we characterized the repertoire of var genes in 12 samples from uncomplicated malaria infections in children from a single Malian village and showed them to be as genetically diverse as vars from isolates from around the globe. The gene var2csa, a member of the var family associated with placental malaria pathogenesis, was present in each genome, as were vars previously associated with severe malaria. ETHA, a tool to discover novel var sequences from clinical samples, will aid the understanding of malaria pathogenesis and inform the design of malaria vaccines based on PfEMP1. ETHA is available at: https://sourceforge.net/projects/etha/ .

  18. A FLUID PRESSURE-LOADED SINGLE CRACK LOCATED IN A ROCK MASSIF PROPAGATION TRAJECTORY CALCULATION

    Directory of Open Access Journals (Sweden)

    Cherdantsev N.V.

    2017-12-01

    Full Text Available The task solving results of a single crack filled with liquid under pressure propagation in a rock mass are presented. The rock mass is loaded with an unequal component gravitational field of stresses and represents a homogeneous elastic medium. The causes of the crack occurrence and its loading by internal pressure are not considered. In the task set, the rock mass is under conditions of a flat deformed state. In this paper, the effect of the opening on the stressed state of the rock mass and on crack propagation trajectory is not considered. The task is solved within the framework of classical concepts of the state of a crack, its stable and unstable growth in an infinite plate of brittle material, based on the theories of Griffiths - Irwin. Based on the results of the studies carried out, crack propagation trajectories are constructed for a number of the crack to the horizon inclination angle values, the characteristics associated with the strength of the enclosing rocks. An analysis is given of the critical pressures change during the crack intergrowth

  19. Vasculitis Associated With Tumor Necrosis Factor-α Inhibitors

    Science.gov (United States)

    Sokumbi, Olayemi; Wetter, David A.; Makol, Ashima; Warrington, Kenneth J.

    2012-01-01

    Objective To describe the clinical characteristics, histopathologic features, and outcomes of patients in whom vasculitis developed in association with use of tumor necrosis factor-α (TNF-α) inhibitors. Patients and Methods This is a retrospective review of patients evaluated at Mayo Clinic, Rochester, Minnesota, from January 1, 1998, through March 31, 2011, with a diagnosis of vasculitis induced by anti–TNF-α therapy. Results Of 8 patients with vasculitis associated with anti–TNF-α therapy (mean age, 48.5 years), 6 (75%) were female. Four (50%) had rheumatoid arthritis, 1 (13%) had Crohn disease, and 3 (38%) had ulcerative colitis. Five (63%) were treated with infliximab, 2 (25%) with etanercept, and 1 (13%) with adalimumab. The mean duration of treatment before development of vasculitis was 34.5 months. The skin was the predominant organ affected (5 patients [63%]), with the most common cutaneous lesion being palpable purpura (4 of 5 [80%]). Two organs involved in systemic vasculitis were the peripheral nervous system (4 patients [50%]) and kidney (1 patient [13%]). All cases of vasculitis were histopathologically confirmed. Seven of 8 patients improved with discontinuation of therapy (mean time to resolution, 6.9 months) and adjuvant treatment (all 8 received prednisone; another agent was also used in 7); rechallenge with anti–TNF-α therapy was not attempted in any patient. At last follow-up, no patients had experienced a recurrence of vasculitis after therapy discontinuation. Conclusion Cutaneous small-vessel vasculitis was the most common finding, but systemic vasculitis, including peripheral nerve and renal vasculitis, was also frequently observed. PMID:22795634

  20. Cutaneous Manifestations of ANCA-Associated Small Vessels Vasculitis.

    Science.gov (United States)

    Marzano, Angelo Valerio; Raimondo, Maria Gabriella; Berti, Emilio; Meroni, Pier Luigi; Ingegnoli, Francesca

    2017-12-01

    Skin lesions are frequent manifestations of underlying systemic conditions, including systemic autoimmune vasculitis. In particular, anti-neutrophil cytoplasmic antibodies (ANCA) are associated with distinct forms of vasculitis characterized by inflammatory cell infiltration of the walls of small and medium-sized vessels leading to vascular destruction and tissue necrosis. ANCA-associated vasculitis is rare and systemic diseases, which can be classified based on different distribution of vascular inflammation and presence or absence of granulomatosis and asthma. Despite their diversities, ANCA-associated vasculitis, namely microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis, can all display a broad variety of cutaneous manifestations, which can appear during the course of the disease or even as first sign at the time of onset. Different skin manifestations might coexist in the same patient and occur in different occasions during the course of the vasculitis. Thus, a deep knowledge of the spectrum of skin lesions as part of ANCA-associated vasculitis is mandatory for a correct diagnostic process, whenever cutaneous vasculitis is suspected. Due to this broad variety of manifestations, the diagnosis of skin involvement in ANCA-associated vasculitis is very challenging and it must be supported by a detailed medical history, accurate physical examination, specific histopathological analysis of skin biopsy and the presence of ANCA serology. In this review, we focus on the cutaneous manifestations that can develop in the context of ANCA-associated vasculitis, detailing the clinical features, the histopathological aspects as well as the direct immunofluorescence studies for each of the three conditions. Moreover, we acknowledged the differential diagnoses that must be ruled out in the diagnostic process and the main therapeutic approaches available for treatment of ANCA-associated vasculitis.

  1. Single tracking location acoustic radiation force impulse viscoelasticity estimation (STL-VE): A method for measuring tissue viscoelastic parameters.

    Science.gov (United States)

    Langdon, Jonathan H; Elegbe, Etana; McAleavey, Stephen A

    2015-07-01

    Single tracking location (STL) shear wave elasticity imaging (SWEI) is a method for detecting elastic differences between tissues. It has the advantage of intrinsic speckle bias suppression compared with multiple tracking location variants of SWEI. However, the assumption of a linear model leads to an overestimation of the shear modulus in viscoelastic media. A new reconstruction technique denoted single tracking location viscosity estimation (STL-VE) is introduced to correct for this overestimation. This technique utilizes the same raw data generated in STL-SWEI imaging. Here, the STL-VE technique is developed by way of a maximum likelihood estimation for general viscoelastic materials. The method is then implemented for the particular case of the Kelvin-Voigt Model. Using simulation data, the STL-VE technique is demonstrated and the performance of the estimator is characterized. Finally, the STL-VE method is used to estimate the viscoelastic parameters of ex vivo bovine liver. We find good agreement between the STL-VE results and the simulation parameters as well as between the liver shear wave data and the modeled data fit.

  2. Locating Damage Using Integrated Global-Local Approach with Wireless Sensing System and Single-Chip Impedance Measurement Device

    Directory of Open Access Journals (Sweden)

    Tzu-Hsuan Lin

    2014-01-01

    Full Text Available This study developed an integrated global-local approach for locating damage on building structures. A damage detection approach with a novel embedded frequency response function damage index (NEFDI was proposed and embedded in the Imote2.NET-based wireless structural health monitoring (SHM system to locate global damage. Local damage is then identified using an electromechanical impedance- (EMI- based damage detection method. The electromechanical impedance was measured using a single-chip impedance measurement device which has the advantages of small size, low cost, and portability. The feasibility of the proposed damage detection scheme was studied with reference to a numerical example of a six-storey shear plane frame structure and a small-scale experimental steel frame. Numerical and experimental analysis using the integrated global-local SHM approach reveals that, after NEFDI indicates the approximate location of a damaged area, the EMI-based damage detection approach can then identify the detailed damage location in the structure of the building.

  3. Locating damage using integrated global-local approach with wireless sensing system and single-chip impedance measurement device.

    Science.gov (United States)

    Lin, Tzu-Hsuan; Lu, Yung-Chi; Hung, Shih-Lin

    2014-01-01

    This study developed an integrated global-local approach for locating damage on building structures. A damage detection approach with a novel embedded frequency response function damage index (NEFDI) was proposed and embedded in the Imote2.NET-based wireless structural health monitoring (SHM) system to locate global damage. Local damage is then identified using an electromechanical impedance- (EMI-) based damage detection method. The electromechanical impedance was measured using a single-chip impedance measurement device which has the advantages of small size, low cost, and portability. The feasibility of the proposed damage detection scheme was studied with reference to a numerical example of a six-storey shear plane frame structure and a small-scale experimental steel frame. Numerical and experimental analysis using the integrated global-local SHM approach reveals that, after NEFDI indicates the approximate location of a damaged area, the EMI-based damage detection approach can then identify the detailed damage location in the structure of the building.

  4. Plasma exchange for renal vasculitis and idiopathic rapidly progressive glomerulonephritis: a meta-analysis

    DEFF Research Database (Denmark)

    Walsh, Michael; Catapano, Fausta; Szpirt, Wladimir

    2011-01-01

    Plasma exchange may be effective adjunctive treatment for renal vasculitis. We performed a systematic review and meta-analysis of randomized controlled trials of plasma exchange for renal vasculitis.......Plasma exchange may be effective adjunctive treatment for renal vasculitis. We performed a systematic review and meta-analysis of randomized controlled trials of plasma exchange for renal vasculitis....

  5. Single- and Multiple- Track Location Shear Wave and Acoustic Radiation Force Impulse Imaging: Matched Comparison of Contrast, CNR, and Resolution

    Science.gov (United States)

    Hollender, Peter J.; Rosenzweig, Stephen J.; Nightingale, Kathryn R.; Trahey, Gregg E.

    2014-01-01

    Acoustic radiation force impulse (ARFI) imaging and shear wave elasticity imaging (SWEI) use the dynamic response of tissue to impulsive mechanical stimulus to characterize local elasticity. A variant of conventional, multiple track location SWEI (MTL-SWEI), denoted single track location SWEI (STL-SWEI) offers the promise of creating speckle-free shear wave images. This work compares the three imaging modalities using a high push and track beam density combined acquisition sequence to image inclusions of different sizes and contrasts. STL-SWEI is shown to have significantly higher CNR than MTL-SWEI, allowing for operation at higher resolution. ARFI and STL-SWEI perform similarly in the larger inclusions, with STL-SWEI providing better visualization of small targets ≤2.5 mm in diameter. The processing of each modality introduces different trade-offs between smoothness and resolution of edges and structures; these are discussed in detail. PMID:25701531

  6. Hypocomplementemic Urticarial Vasculitis Syndrome With Crescentic Glomerulonephritis.

    Science.gov (United States)

    Salim, Sohail Abdul; Yousuf, Tauqeer; Patel, Asha; Fülöp, Tibor; Agarwal, Mohit

    2018-02-01

    Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disease characterized by multiple organ system involvement, including renal disease, with low complement levels. We report the case of a 31-year-old woman who presented with nonspecific symptoms including fatigue, diarrhea, macular rash and abdominal pain with acute renal failure leading to end-stage kidney disease. Laboratory results showed hematuria, nephrotic range proteinuria, worsening creatinine and low C1q levels. Left kidney biopsy showed proliferative glomerulonephritis with crescent formation. She was treated with 6 months of intravenous cyclophosphamide, followed by 2 doses of intravenous rituximab (1g each), thereafter maintained on mycophenolate mofetil and glucocorticoid-based therapy. She experienced a full recovery of renal function after 12 months of dialysis dependence. Hypocomplementemic urticarial vasculitis syndrome with crescentic glomerulonephritis is a rare disease with only 5 other reported cases in literature. In our case, we document a delayed but excellent renal recovery during a 2-year follow-up. Copyright © 2018 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

  7. Comparison of femur tunnel aperture location in patients undergoing transtibial and anatomical single-bundle anterior cruciate ligament reconstruction.

    Science.gov (United States)

    Lee, Dae-Hee; Kim, Hyun-Jung; Ahn, Hyeong-Sik; Bin, Seong-Il

    2016-12-01

    Although three-dimensional computed tomography (3D-CT) has been used to compare femoral tunnel position following transtibial and anatomical anterior cruciate ligament (ACL) reconstruction, no consensus has been reached on which technique results in a more anatomical position because methods of quantifying femoral tunnel position on 3D-CT have not been consistent. This meta-analysis was therefore performed to compare femoral tunnel location following transtibial and anatomical ACL reconstruction, in both the low-to-high and deep-to-shallow directions. This meta-analysis included all studies that used 3D-CT to compare femoral tunnel location, using quadrant or anatomical coordinate axis methods, following transtibial and anatomical (AM portal or OI) single-bundle ACL reconstruction. Six studies were included in the meta-analysis. Femoral tunnel location was 18 % higher in the low-to-high direction, but was not significant in the deep-to-shallow direction, using the transtibial technique than the anatomical methods, when measured using the anatomical coordinate axis method. When measured using the quadrant method, however, femoral tunnel positions were significantly higher (21 %) and shallower (6 %) with transtibial than anatomical methods of ACL reconstruction. The anatomical ACL reconstruction techniques led to a lower femoral tunnel aperture location than the transtibial technique, suggesting the superiority of anatomical techniques for creating new femoral tunnels during revision ACL reconstruction in femoral tunnel aperture location in the low-to-high direction. However, the mean difference in the deep-to-shallow direction differed by method of measurement. Meta-analysis, Level II.

  8. Predictors of medication non-adherence for vasculitis patients

    Science.gov (United States)

    Hogan, Susan L.; DeVellis, Robert F.

    2013-01-01

    The primary purpose of this article is to document whether demographic, clinical, regimen-related, intrapersonal, and interpersonal factors predict medication non-adherence for vasculitis patients. A secondary purpose is to explore whether adherence varies by medication type and whether patients experienced drug-related side effects. Vasculitis patients (n=228) completed online baseline and 3-month follow-up surveys. Demographic (age, gender, education, race, marital status, and insurance status), clinical (perceived vasculitis severity, disease duration, vasculitis type, and relapse/remission status), regimen-related (experience of side effects), intrapersonal (depressive symptoms), and interpersonal (adherence-related support from family and friends) factors were measured at baseline. Medication non-adherence was assessed at follow-up using the Vasculitis Self-Management Survey medication adherence sub-scale (α=0.89). Variables that significantly correlated (pvasculitis medication types, patients who experienced side effects were less adherent than patients who did not experience side effects. Multiple factors are associated with medication non-adherence for vasculitis patients. Providers should discuss medication adherence and drug-related side effects with vasculitis patients. Providers may want to particularly target younger patients and patients with clinical signs of depression. PMID:23314654

  9. [Secondary vasculitis to infection by Fasciola hepatica].

    Science.gov (United States)

    Málaga, Germán; Taco-Palma, Rubén; Cáceres-Pizarro, Jaime; Lazo, María de los Angeles; Castaneda-Guarderas, Ana; Ticse, Ray

    2012-01-01

    There is a case of a 38 year-old male patient coming from an area where hepatic fascioliasis is endemic in Peru. He was hospitalized because he showed 4 weeks of symptoms like fever, intense myalgias, erythematous and painful injuries on limb extensions. The electromyography and nerve conduction velocity showed a global inflammatory myopathy. A skin biopsy showed polyarteritis nodosa-type vasculitis. During the evaluation process prior to the immunosuppressive therapy, hepatic Fasciola eggs were found in the parasitological examination of stools. The fascioliasis diagnosis was confirmed by fas2-ELISA: 0.46 (VN <0.20). Clinical symptoms started to subside after treatment with ticlabendazol. Contact with the patient was maintained for a year and there was no evidence of disease recurrence, and he was asymptomatic.

  10. Cutaneous necrotizing vasculitis. Relation to systemic disease.

    Science.gov (United States)

    Lotti, T M; Comacchi, C; Ghersetich, I

    1999-01-01

    Cutaneous necrotizing vasculitis (CNV) is a complex multisystem disease generally involving the skin and mucous membranes, often accompanied by renal, gastrointestinal, pericardial, neurological, and articular signs and symptoms. CNV may be idiopatical or occur in association with a drug, infection, or underlying disease. CNV has been shown in patients with chronic infections (viral, bacterial, protozoa, helminthic), serum sickness, a variety of collagen vascular diseases (systemic lupus erythematous, Sjögren's syndrome, rheumatoid arthritis, Behçet's disease) hyperglobulinemic states, cryoglobulinemia, bowel bypass syndrome, ulcerative colitis, cystic fibrosis, primary biliary cirrhosis and HIV infection. Association with malignancies is not frequent. Lymphoproliferative disorders (Hodgkin's disease, mycosis fungoides, lymphosarcoma, adult T-cell leukemia, multiple mieloma) and solid tumors (lung cancer, colon carcinoma, renal, prostate, head and neck cancer and breast cancer) may be associated with CNV. Whenever possible, treatment is directed at the elimination of the cause. In other cases after adequate laboratory screening local and systemic therapy are recommended.

  11. Churg-Strauss syndrome and hemorragic vasculitis

    Directory of Open Access Journals (Sweden)

    Rui Moreira Marques

    2011-12-01

    Full Text Available Churg-Strauss syndrome (CSS is a rare syndrome characterized by sinusitis, asthma and peripheral eosinophilia. This vasculitic syndrome affects medium and small-sized vessels, the lung being the most commonly affected organ, followed by the skin. The authors report a case of a 59-year-old male with a past history of asthma and allergic rhinitis. He presented necrohemorragic lesions in the distal phalanx of the 2nd, 3rd and 4th fingers of the lefthand and petechial lesions in the plant of both feet, accompanied by asthenia, anorexia and weight loss. The analytical study revealed leukocytosis with eosinophilia, elevated inflammatory parameters and p-ANCA positive antibodies. The diagnosis of CSS was established based on clinical and histopathological data. Cutaneous manifestations of hemorragic vasculitis are rare in CSS syndrome but can be the first manifestation of the disease. The recognition of this presentation is important for the early diagnosis and treatment of this syndrome.

  12. Vasculitis inducida por metimazol: Reporte de caso

    Directory of Open Access Journals (Sweden)

    Miguel Pinto

    2011-07-01

    Full Text Available Se reporta el caso de una paciente con enfermedad de Graves, que presentó vasculitis asociada al uso de metimazol. Mujer de 14 años, que acudió a consulta por presentar intolerancia al calor, tremor distal y palpitaciones. El examen físico mostró bocio difuso, y el perfil tiroideo, TSH suprimida y hormonas tiroideas elevadas. Los anticuerpos antiperoxidasa tiroidea fueron positivos. Se inició tratamiento con metimazol y beta bloqueadores. Después de 20 días, la paciente regresó por presentar malestar general, fiebre, poliartralgia, lesiones cutáneas maculopapulares y edema de miembros inferiores. Los anticuerpos antinucleares fueron negativos y los anticuerpos anticitoplasma de los neutrófilos (ANCA, positivos. Se suspendió el metimazol y se inició prednisona. Después de 10 días de tratamiento, las molestias desaparecieron y la paciente recibió I 131.Las vasculitis asociadas al uso de tionamidas son poco frecuentes, no dependen de la dosis y están asociadas a la presencia de anticuerpos tipo ANCA. Clásicamente, afectan a los vasos pequeños de la piel; sin embargo, también pueden afectar los riñones y pulmones. El cuadro clínico se caracteriza por artralgias y mialgias. En algunos casos puede ocurrir insuficiencia renal de grado variable. En la mayoría de casos, el cuadro remite con la suspensión de la droga; pero, en algunos se requiere el uso de glucocorticoides o inmunosupresores.(Rev Med Hered 2011;22:147-150.

  13. Single-dose methotrexate treatment for ectopic pregnancy and pregnancy of unknown location and progesterone as a predictor of success.

    Science.gov (United States)

    Wu, Joyce; Ludlow, Joanne P; De Vries, Bradley; Black, Kirsten; Beale, Philip

    2014-10-01

    The use of single-dose intramuscular administration of methotrexate in the treatment of ectopic pregnancies (EP) is a well-established practice. This study evaluates its use at a novel dose of 40 mg/m(2) body surface area (BSA). To evaluate the efficacy and safety of single-dose methotrexate treatment 40 mg/m(2) for tubal EP and persistent pregnancies of unknown location (PUL) and determine whether serum progesterone is a predictor of treatment success. Retrospective cohort study of patients receiving intramuscular methotrexate 40 mg/m(2) for the treatment of EP or PUL at Royal Prince Alfred Hospital over five years. One hundred and eighteen women received single-dose methotrexate with an overall success of 84%. Surgical intervention was needed in 16.6%. Pretreatment beta-hCG level and ectopic diagnosis were independent variables predictive of the need for surgery (P = 0.003 and 0.02, respectively). Serum progesterone level was not predictive of the need for a second dose or surgery. The sensitivity and specificity at pretreatment beta-hCG of 1202 IU/L were 84% and 74%, respectively. Commonly reported side effects included nausea, abdominal pain and heavy vaginal bleeding. Significant treatment-related adverse effects were rare. Single-dose IM methotrexate at a novel dose of 40 mg/m(2) is a safe and effective treatment for selected EP and persistent PUL. The risk of surgery was positively correlated to serum beta-hCG level and the diagnosis of EP. Progesterone was not a risk factor for surgery. Further studies are required to confirm the efficacy of this dose regimen and explore the safety of expectant management as an alternative to methotrexate treatment. © 2014 The Royal Australian and New Zealand College of Obstetricians and Gynaecologists.

  14. Could we abandon cyclophosphamide in systemic vasculitis and lupus nephritis?

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.

    Cyclophosphamide has greatly improved prognosis in antineutrophil cytoplasmic autoantibody-associated vasculitis (AAV) and proliferative lupus nephritis (LN). However, the side effects of long-term cyclophosphamide treatment are considerable prompting a search for alternatives to cyclophosphamide.

  15. Refractory disease in antineutrophil cytoplasmic antibodies associated vasculitis

    NARCIS (Netherlands)

    Rutgers, Abraham; Kallenberg, Cornelis

    Purpose of review Induction treatment of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) is not always successful and nonresponding patients are considered refractory. Recent findings Refractory disease should be subdefined to the treatment that was received.

  16. Propylthiouracil-Induced Vasculitis With Antineutrophil Cytoplasmic Antibody.

    Science.gov (United States)

    Criado, Paulo Ricardo; Grizzo Peres Martins, Ana Claudia; Gaviolli, Camila Fatima; Alavi, Afsaneh

    2015-06-01

    Propylthiouracil (PTU)-associated vasculitis is a potentially life-threatening disease with a recent increase in the reported cases in the medical literature. This increase may suggest that some earlier cases have been unrecognized or assigned to an alternative nosology category. Although the skin can be the only organ affected by PTU-associated vasculitis, there are many reports with multiple-system involvement. Classically, the symptoms appear under a tetrad of fever, sore throat, arthralgia, and skin lesions. Cutaneous lesions in reported cases of PTU vasculitis have most commonly consisted of retiform acral, purpuric plaques, or nodules. We report a case of perinuclear antineutrophil cytoplasmic antibody-associated vasculitis developed during treatment with PTU for Grave's disease. © The Author(s) 2014.

  17. A Unique Case of Systemic Lupus Erythematosus Pelvic Vasculitis

    Directory of Open Access Journals (Sweden)

    Pamela Traisak

    2016-01-01

    Full Text Available The clinical presentation of Systemic Lupus Erythematosus (SLE is diverse and vasculitis can be a potential manifestation. Cutaneous lesions involving small vessels are the most frequent presentation. However, medium and large vessel vasculitis may present with life-threatening visceral manifestations. We present a unique case of pelvic vasculitis mimicking a pelvic mass as an initial presentation of SLE. There are case reports of systemic vasculitis involving the female genital tract with giant cell arteritis (GCA, polyarteritis nodosa (PAN, and granulomatous with polyangiitis and microscopic polyangiitis (GPA/MPA, among others, but only a few cases attributed to SLE. Awareness of this condition and a prompt diagnosis are warranted as this is a severe and potentially life-threatening condition.

  18. A pragmatic approach to vasculitis in the gastrointestinal tract.

    Science.gov (United States)

    Chetty, Runjan; Serra, Stefano

    2017-06-01

    Although vasculitis involving the gastrointestinal tract (GIT) is an uncommon occurrence, occasionally vasculitis can present as haemorrhagic infarction or ischaemia for which a length of bowel is removed. Invariably, the appropriate clinical history is not forthcoming, or vasculitis is not clinically suspected. The purpose of this overview is to provide the practising gastrointestinal (GI) pathologist with a framework to recognise and diagnose vasculitides within the GIT. The classification may be approached by aetiological agent or size of vessel involved; an international consensus group now favours the latter approach. The symptoms that systemic and/or localised vasculitis may cause in the GIT are protean and non-specific. As a result, pathologists examining resection specimens for unexplained haemorrhagic infarction or ischaemia should be aware that vasculitis may be a potential cause. Several well-known systemic vasculitides such as polyarteritis nodosa, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis or Churg-Strauss syndrome and granulomatosis with polyangiitis or Wegener's granulomatosis can occur in the GIT. The latter three constitute the antineutrophil cytoplasmic antibody-positive vasculitides. In addition, the so-called solitary organ vasculitis (SOV) can occur in the GIT as the harbinger of later onset systemic vasculitis, and be the cause of the GIT symptoms. In addition, SOV can occur incidentally and coexist with GIT disease such as gallstones or polyps, and there may be no manifestations of systemic vasculitis for years, or not at all. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  19. Testicular lymphocytic vasculitis treated with prednisolone and azathioprine.

    Science.gov (United States)

    Kanzawa, Yohei; Imai, Yukihiro; Mizuno, Yasushi; Nishioka, Hiroaki

    2017-07-01

    Testicular vasculitis is a rare condition and little is known about its morphological features. Herein, we report a case of testicular lymphocytic vasculitis, which is rarely documented, in an elderly man. He presented with left testicular swelling and fever, but without any signs of other organ involvement. He was effectively treated with prednisolone and azathioprine. This case report offers information related to the disease course and the importance of biopsy.

  20. Long-term patient survival in ANCA-associated vasculitis

    DEFF Research Database (Denmark)

    Flossmann, Oliver; Berden, Annelies; de Groot, Kirsten

    2011-01-01

    Wegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain.......Wegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain....

  1. Acrally distributed dermatoses: Vascular dermatoses (purpura and vasculitis).

    Science.gov (United States)

    Kazandjieva, Jana; Antonov, Dimitar; Kamarashev, Jivko; Tsankov, Nikolai

    Purpuric lesions appear in acral distribution in a variety of conditions and often provide clues to the clinical diagnosis. Purpuric means "hemorrhagic"-that is, the lesions do not blanch from pressure. This review focuses on dermatoses that produce hemorrhagic lesions in acral distribution from the large groups of the vasculitic diseases and their mimics. Cutaneous small vessel vasculitis is confined to the skin, involves mainly postcapillary venules, and has the hallmark manifestation of palpable purpura. Henoch-Schönlein purpura is an immune complex-mediated systemic vasculitis of the small vessels with manifestations from the skin, joints, kidneys, and gastrointestinal system. Only cases where the immune complexes contain immunoglobulin A type are classified as Henoch-Schönlein purpura. Cryoglobulinemic vasculitis is induced by the deposition of cold-precipitated immune complexes in the small vessels. Urticarial vasculitis comprises a spectrum of conditions with the characteristic course of chronic urticaria, with wheals that persist longer than 24 hours, leave hyperpigmentation, and have leukocytoclastic vasculitis on histologic examination. Polyarteritis nodosa is a rare multisystem, segmental necrotizing vasculitis of mainly the medium-sized vessels. Pigmented purpuric dermatoses are chronic benign dermatoses characterized by petechiae, purpura, and increased skin pigmentation. The hallmark of pigmented purpuric dermatoses is their orange-brown, speckled, cayenne pepper-like discoloration. Copyright © 2016 Elsevier Inc. All rights reserved.

  2. Vasculitis as an adverse event following immunization - Systematic literature review.

    Science.gov (United States)

    Bonetto, Caterina; Trotta, Francesco; Felicetti, Patrizia; Alarcón, Graciela S; Santuccio, Carmela; Bachtiar, Novilia Sjafri; Brauchli Pernus, Yolanda; Chandler, Rebecca; Girolomoni, Giampiero; Hadden, Robert D M; Kucuku, Merita; Ozen, Seza; Pahud, Barbara; Top, Karina; Varricchio, Frederick; Wise, Robert P; Zanoni, Giovanna; Živković, Saša; Bonhoeffer, Jan

    2016-12-12

    Several types of vasculitis have been observed and reported in temporal association with the administration of various vaccines. A systematic review of current evidence is lacking. This systematic literature review aimed to assess available evidence and current reporting practice of vasculitides as adverse events following immunization (AEFI). We reviewed the literature from 1st January 1994 to 30th June 2014. This review comprises randomized controlled trials, observational studies, case series, case reports, reviews and comments regardless of vaccine and target population. The initial search resulted in the identification of 6656 articles. Of these, 157 articles were assessed for eligibility and 75 studies were considered for analysis, including 6 retrospective/observational studies, 2 randomized controlled trials, 7 reviews, 11 case series, 46 case reports and 3 comments. Most of the larger, higher quality studies found no causal association between vaccination and subsequent development of vasculitis, including several studies on Kawasaki disease and Henoch-Schönlein purpura (IgA vasculitis). Smaller case series reported a few cases of vasculitis following BCG and vaccines against influenza and hepatitis. Only 24% of the articles reported using a case definition of vasculitis. Existing literature does not allow establishing a causative link between vaccination and vasculitides. Further investigations were strengthened by the use of standardized case definitions and methods for data collection, analysis and presentation to improve data comparability and interpretation of vasculitis cases following immunization. Published by Elsevier Ltd.

  3. Isolated vasculitis of the CNS; Isolierte Vaskulitis des ZNS

    Energy Technology Data Exchange (ETDEWEB)

    Block, F. [RWTH Aachen (Germany). Neurologische Klinik; Reith, W. [Universitaet des Saarlandes, Homburg/Saar (Germany). Radiologische Klinik

    2000-11-01

    Vasculitis is a rare cause for disease of the CNS. The isolated vasculitis of the CNS is restricted to the CNS whereas other forms of vasculitis affect various organs including the CNS. Headache, encephalopathy, focal deficits and epileptic seizures are the major symptoms suggestive for vasculitis. One major criterion of the isolated vasculitis of the CNS is the lack of evidence for other vasculitis forms or for pathology of other organs. Angiography displays multifocal segmental stenosis of intracranial vessels. MRI demonstrates multiple lesions which in part show enhancement after gadolinium. A definite diagnosis can only be made on the grounds of biopsy from leptomeninges and parenchyma. Therapy consists of corticosteroids and cyclophosphamid. (orig.) [German] Vaskulitiden sind eine seltene Ursache fuer Erkrankungen des ZNS. Die Vaskulitiden lassen sich in primaere und sekundaere einteilen, von denen sich die ueberwiegende Mehrzahl an verschiedenen Organsystemen einschliesslich dem ZNS manifestieren kann. Die isolierte ZNS-Vaskulitis ist auf das ZNS beschraenkt, bei ihr stehen klinisch-neurologisch wie bei den anderen Vaskulitisformen Kopfschmerzen, Enzephalopathie, fokale Defizite und epileptische Anfaelle im Vordergrund. Ein Kriterium der isolierten ZNS-Vaskulitis ist der klinische und laborchemische Ausschluss anderer Vaskulitiden bzw. der Beteiligung anderer Organsysteme. Multiple Kaliberspruenge intrakranieller Arterien in der zerebralen Angiographie und multiple, kleine, z.T. kontrastmittelaufnehmende Laesionen in der MRT des Schaedels sind vaskulitistypische Befunde, die allerdings auch bei anderen Vaskulitiden zu finden sind. Einzig beweisend ist eine Hirnhaut- und Hirnparenchymbiopsie. Besonders vor dem Hintergrund der therapeutischen Option, Immunsuppression mit Kortison und Cyclophosphamid, ist eine moeglichst genaue Diagnose erforderlich. (orig.)

  4. Optimal location of a single distal interlocking screw in intramedullary nailing of distal third femoral shaft fractures.

    Science.gov (United States)

    George, C J; Lindsey, R W; Noble, P C; Alexander, J W; Kamaric, E

    1998-05-01

    This biomechanical study was done to determine the effect of the level of a single distal screw in a static intramedullary (IM) femoral nail on the stability of fixation of a fracture in the distal third of the femur. Fifteen composite fiberglass femora were osteotomized transversely in the distal third of the femur. A Grosse-Kempf nail was implanted into the femurs, which were divided into three groups of five specimens. Single screw distal nail locking was varied distal to the osteotomy site for each group at 2.5, 5.0, and 7.5 centimeters, respectively. All instrumented femurs were mounted on a servohydraulic testing machine and fitted with transducers to measure axial, rotational, and bending displacements. Specimens were cyclically loaded (one hertz) in simultaneous torsion (moment: +/- 10 newton-meters) and axial compression (amplitude: 2,000 newtons) for 500 cycles with a 250-pound abductor force. Data from linear and rotational transducers were sampled at 100 hertz for five cycles before cycling, every 100 cycles of loading, and immediately after cycling. Custom computer software was developed to convert transducer signals into static and dynamic measurements of axial motion (in millimeters), rotation (in degrees), and angulation (in degrees). Osteotomy site dynamic rotation increased significantly in specimens locked at 7.5 centimeters when compared with the 2.5-centimeter group. There was minimal difference between the stability of the 5.0-centimeter and 7.5-centimeter groups. There was no significant change in position at the fracture site before or after cyclic loading with respect to axial shortening, rotation, or bending. Both dynamic axial and angular displacements were also unaffected by screw position. The location of a single distal interlocking screw in static IM nail fixation of distal third femur fractures can significantly affect rotational stability but not axial or angular fixation.

  5. Severe Small-Vessel Vasculitis Temporally Associated With Administration of Ustekinumab.

    Science.gov (United States)

    MacArthur, Kelly M; Merkel, Peter A; Van Voorhees, Abby S; Nguyen, Jennifer; Rosenbach, Misha

    2016-03-01

    Vasculitis may be caused by infection, medications, systemic diseases, malignancy, or occur as an idiopathic condition. In cases of drug-induced vasculitis, it is essential to identify and discontinue the culprit medication. As novel agents are approved through clinical trials, some rare events, including vasculitis, may not become apparent until wider use, and rigorous post-marketing surveillance for new medications is important. Physicians should consider drug-induced vasculitis on the differential for all new vasculitis diagnoses, and if the potential triggering medication is a novel medication, it is essential to rigorously investigate the potential for emerging cases of medication-associated vasculitis in all available scientific literature.

  6. [The diagnostic value of human chorionic gonadotrophin ratio compared to single measurements of S-human chorionic gonadotrophin on the outcome of pregnancy of unknown location].

    Science.gov (United States)

    Majeed, Huda Galib; Lyngsø, Julie; Bor, Pinar

    2014-10-13

    Pregnancy of unknown location is defined by a positive pregnancy test, without visualizing of the intrauterine or extrauterine pregnancy by transvaginal sonography. We present the advantages of using human chorionic gonadotrophin (hCG) ratio instead of single measurements of S-hCG for predicting the outcomes of pregnancies of unknown location.

  7. Thoracic vasculitis presenting as surgical problems.

    LENUS (Irish Health Repository)

    Jansen, Michael

    2010-01-01

    We present four patients with vasculitis manifesting with unusual clinical or pathological features, generating surgical problems. Two cases presented with pulmonary hypertension, with investigations and radiological evidence prompting clinical suspicion of pulmonary thrombo-embolic disease. First case, with an antecedant history of Wegener\\'s granulomatosis (WG), demonstrated following "embolectomy", WG involving the large pulmonary elastic arteries. The second case of inoperable "pulmonary thrombo-embolic disease" was subsequently found at limited post mortem to have giant cell arteritis, which affected widespread small peripheral pulmonary arterial vessels. The other two cases were of aortitis occurring in the background of immune-mediated disease, which had been treated with aggressive immunosuppression regimens. The first of these was a case of Cogan\\'s syndrome complicated by descending aortitis, a rarely reported phenomenon, with co-existent acute endocarditis of the aortic valve leaflets. Most cases of endocarditis in this context occur secondary to and in continuity with ascending aortitis. That this case, and a case of ascending aortitis occurring in the context of relapsing polychondritis occurred in the face of aggressive immunosuppression with an apparent clinical response, underscores the need to not accept a clinical picture at face value. This has implications for clinical management, particularly in the follow-up of surgical prosthetic devices such as grafts which may be used in these cases. All four cases emphasise the continued importance of histology and the post-mortem examination in elucidating previously undetected or unsuspected disease.

  8. CT findings at lupus mesenteric vasculitis

    International Nuclear Information System (INIS)

    Ko, S.F.; Lee, T.Y.; Cheng, T.T.; Ng, S.H.; Lai, H.M.; Cheng, Y.F.; Tsai, C.C.

    1997-01-01

    Purpose: To describe the spectrum of early CT findings of lupus mesenteric vasculitis (LMV) and to assess the utility of CT in the management of this uncommon entity. Methods: Abdominal CT was performed within 1-4 days (average 2.2 days) of the onset of severe abdominal pain and tenderness in 15 women with systemic lupus erythematosus. Prompt high-dose i.v. corticosteroid in 11 patients after the CT diagnosis of LMV was made. CT was performed after abdominal symptoms subsided. Results: Eleven cases revealed CT features suggestive of LMV including conspicuous prominence of mesentric vessels with palisade pattern or comb-like appearance (CT comb sign) supplying focal or diffuse dilated bowel loops (n=11), ascites with slightly increased peritoneal enhancement (n=11), small bowel wall thickening (n=10) with double halo or target sign (n=8). Follow-up CT before high-dose steroid therapy revealed complete or marked resolution of the abnormal CT findings. Conclusion: CT is helpful for confirming the diagnosis of LMV, especially the comb sign which may be an early sign. Bowel ischemia due to LMV is less ominous than previously expected, and the abnormal CT findings were reversible when early diagnosis and prompt i.v. steroid therapy could be achieved. (orig.)

  9. CT findings at lupus mesenteric vasculitis

    Energy Technology Data Exchange (ETDEWEB)

    Ko, S.F. [Chang Gung Medical College and Memorial Hospital, Dept. of Radiology, Kaohsiung Hsien (Taiwan); Lee, T.Y. [Chang Gung Medical College and Memorial Hospital, Dept. of Radiology, Kaohsiung Hsien (Taiwan); Cheng, T.T. [Chang Gung Medical College and Memorial Hospital, Dept. of Rheumatology, Kaohsiung Hsien (Taiwan); Ng, S.H. [Chang Gung Medical College and Memorial Hospital, Dept. of Radiology, Kaohsiung Hsien (Taiwan); Lai, H.M. [Chang Gung Medical College and Memorial Hospital, Dept. of Rheumatology, Kaohsiung Hsien (Taiwan); Cheng, Y.F. [Chang Gung Medical College and Memorial Hospital, Dept. of Radiology, Kaohsiung Hsien (Taiwan); Tsai, C.C. [Chang Gung Medical College and Memorial Hospital, Dept. of Radiology, Kaohsiung Hsien (Taiwan)

    1997-01-01

    Purpose: To describe the spectrum of early CT findings of lupus mesenteric vasculitis (LMV) and to assess the utility of CT in the management of this uncommon entity. Methods: Abdominal CT was performed within 1-4 days (average 2.2 days) of the onset of severe abdominal pain and tenderness in 15 women with systemic lupus erythematosus. Prompt high-dose i.v. corticosteroid in 11 patients after the CT diagnosis of LMV was made. CT was performed after abdominal symptoms subsided. Results: Eleven cases revealed CT features suggestive of LMV including conspicuous prominence of mesentric vessels with palisade pattern or comb-like appearance (CT comb sign) supplying focal or diffuse dilated bowel loops (n=11), ascites with slightly increased peritoneal enhancement (n=11), small bowel wall thickening (n=10) with double halo or target sign (n=8). Follow-up CT before high-dose steroid therapy revealed complete or marked resolution of the abnormal CT findings. Conclusion: CT is helpful for confirming the diagnosis of LMV, especially the comb sign which may be an early sign. Bowel ischemia due to LMV is less ominous than previously expected, and the abnormal CT findings were reversible when early diagnosis and prompt i.v. steroid therapy could be achieved. (orig.).

  10. Location, location, location: Extracting location value from house prices

    OpenAIRE

    Kolbe, Jens; Schulz, Rainer; Wersing, Martin; Werwatz, Axel

    2013-01-01

    The price for a single-family house depends both on the characteristics of the building and on its location. We propose a novel semiparametric method to extract location values from house prices. After splitting house prices into building and land components, location values are estimated with adaptive weight smoothing. The adaptive estimator requires neither strong smoothness assumptions nor local symmetry. We apply the method to house transactions from Berlin, Germany. The estimated surface...

  11. The efficacy of novel therapeutic modalities of isolated ocular vasculitis vs ocular vasculitis as a systemic disease

    Directory of Open Access Journals (Sweden)

    Filip M. Radotić

    2012-02-01

    Full Text Available Aim To evaluate the efficacy of new therapeutic modalities withthe observation period of three years on patients with isolated ocular vasculitis in comparison with ocular vasculitis as a systemicdisease. Methods The effectiveness of the therapy was assessed based on the changes in visual acuity and degree of ocular inflammation (mild, medium, moderate, severe with the following parameters: vitreous body cloudiness, blood vessels layering, macula oedema, blood vessels occlusion and new vascularisation. Results New therapeutic modalities resulted in reduction in the number of patients with severe inflammation in the group of isolated ocular vasculitis from 8(13.5% to 7(12.2% after three years, while the number of patients with mild inflammation increased from 13(20.7% to 18 (29.3% in the same group (p>0.05. The number of patients with severe ocular inflammation in a group of ocular vasculitis as systemic disease increased from 3(16.2% to 4(21.6%, because of the presence of patients with Behçet’s disease. The number of patients with visual acuity less than 0.1 decreased from 11(17% to 8(13.4% in a group of patients with ocular vasculitis as systemic disease, which was associated with the presence of Behçet’s disease too (p>0.05. Conclusion Although the effect of new therapeutic modalities did not result in statistically significant improvement in visual acuity and reducing inflammation, systemic and intravitreal corticosteroids with steroid-sparing immunomodulatory therapy represents effective strategies in forms of isolated ocular vasculitis and ocular vasculitis as systemic disease.

  12. Radiologic studies in two outbreaks of isolated vasculitis in the central nervous system

    International Nuclear Information System (INIS)

    Robertson, H.J.; Perez, M.; Tilton, A.H.; Garcia, C.; McGarry, P.

    1989-01-01

    Cerebral vasculitis is only occasionally diagnosed with angiography. Two outbreaks of isolated central nervous system vasculitis permitted a comparison of the accuracy of diagnostic radiologic studies. Two new radiologic features and methods of diagnosis are discussed

  13. CLINICAL MANIFESTATIONS OF VASCULITIS AT THE ONSET OF MULTIPLE MYELOMA

    Directory of Open Access Journals (Sweden)

    V I Vasil'ev

    2010-01-01

    Full Text Available The paper describes two patients (a 50-year woman and a 72-year man in whom the clinical manifestations of ulceronecrotic vasculitis had long preceded before the diagnosis of multiple myeloma was made. In the former, monoclonal cryoglobulinemia type I induced ulcerative lesions with the development of dry toe gangrene and paraproteinemic renal lesion. In the latter, ulceronecrotic vasculitis with the development of dry toe gangrene was a manifestation of monoclonal paraproteinemia without signs of cryoglobulinemia. Both patients were found to have monoclonal blood secretion (РIgGλ и РIgGκ and urine Bence Jones protein (BJλ+BJκ, BJκ in the absence of immunological markers of vasculitis developing in patients with rheumatic diseases. Immunochemical study of serum/urine and, when monoclonal secretion of PIg+BJ is detected, further examination for plasma cell dyscrasia should be performed in all cases of vascular disorders (cold allergy, Raynaud's syndrome, purpura, ulcers of cruses, and gangrene of distal phalanxes of the hands/feet in patients with atypical vasculitis. The timely immunochemical study of blood and urine will make it possible to diagnose plasma cell dyscrasia (different types of myelomic disease, Waldenstrцm macroglobulinemia, primary amyloidosis at the early stage of the disease and to rule out unjustifiably diagnosed vasculitis in patients with atypical vascular lesions.

  14. [Large vessel vasculitis with myelodysplastic syndrome: A rare association].

    Science.gov (United States)

    Galland, J; Kawski, H; Guichard, J-F; Maurier, F

    2017-07-01

    The vasculitis can be the consequence of malignancy: most often hematologic rather than solid tumors. The association between large vessels vasculitis and myelodysplastic syndrome is rare. A 55-year-old man experienced asthenia, fever, polyarthritis and inflammatory syndrome. Haematological investigations found a type 2 refractory anemia with excess blasts (RAEB-2) with discovery of severe anemia (Hb: 7,8g/dl) and thrombopenia (platelets: 40,000/mm 3 ). Radiological examinations found thoracic aortitis and carotid vasculitis. Treatment in the form of steroids and azacitidine was instituted. The lack of control of both RAEB-2 and vasculitis was responsible for the death of the patient. Myelodysplastic syndrome and large vessels vasculitis is a rare but serious association disease. The lack of efficiency of corticosteroids seems to be common. Prognosis depends on the haematological treatment effectiveness. Copyright © 2016 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  15. Equine pastern vasculitis: a clinical and histopathological study.

    Science.gov (United States)

    Psalla, Dimitra; Rüfenacht, Silvia; Stoffel, Michael H; Chiers, Koen; Gaschen, Véronique; Doherr, Marcus G; Gerber, Vincent; Welle, Monika M

    2013-11-01

    Equine pastern vasculitis is clinically challenging and the underlying aetiopathogenesis is unclear. The aims of this retrospective study were to establish histopathological criteria for pastern vasculitis, to look for an underlying cause, to investigate whether the histopathological lesions are associated with a distinct clinical picture, to assess if and how the clinical picture varies, and to determine the treatment response. Skin biopsies and clinical data from 20 horses with a diagnosis of vasculitis of the distal extremities were investigated and histology was compared to biopsies from healthy horses. It was concluded that intramural inflammatory cells, leukocytoclasia with nuclear dust, thickening and oedema of the vessel walls, and microhaemorrhages are highly specific histological findings in equine pastern vasculitis. Based on the feedback from the clinicians, the lesions were mostly seen on the lateral and medial aspects of un-pigmented legs. Lesions in white skin were characterised by exudation and crusts, whereas those in pigmented skin were alopecic and characterised by scaling. The response to treatment was poor and the prognosis guarded. No association was found between any of the histopathological findings and a distinct clinical picture. An underlying cause of equine pastern vasculitis could not be identified. Considering the large number of confounding factors, the causative agents are difficult to identify, but may involve drugs or a hypersensitivity reactions to yet unknown antigens. Copyright © 2013 Elsevier Ltd. All rights reserved.

  16. Single- and multiple-track-location shear wave and acoustic radiation force impulse imaging: matched comparison of contrast, contrast-to-noise ratio and resolution.

    Science.gov (United States)

    Hollender, Peter J; Rosenzweig, Stephen J; Nightingale, Kathryn R; Trahey, Gregg E

    2015-04-01

    Acoustic radiation force impulse imaging and shear wave elasticity imaging (SWEI) use the dynamic response of tissue to impulsive mechanical stimulus to characterize local elasticity. A variant of conventional, multiple-track-location SWEI, denoted single-track-location SWEI, offers the promise of creating speckle-free shear wave images. This work compares the three imaging modalities using a high push and track beam density combined acquisition sequence to image inclusions of different sizes and contrasts. Single-track-location SWEI is found to have a significantly higher contrast-to-noise ratio than multiple-track-location SWEI, allowing for operation at higher resolution. Acoustic radiation force impulse imaging and single-track-location SWEI perform similarly in the larger inclusions, with single-track-location SWEI providing better visualization of small targets ≤ 2.5 mm in diameter. The processing of each modality introduces different trade-offs between smoothness and resolution of edges and structures; these are discussed in detail. Copyright © 2015 World Federation for Ultrasound in Medicine & Biology. Published by Elsevier Inc. All rights reserved.

  17. Myositis, Vasculitis, Hepatic Dysfunction in Adult-Onset Still's Disease

    Directory of Open Access Journals (Sweden)

    Hidekatsu Yanai

    2009-01-01

    Full Text Available Although hepatic dysfunction is common in adult-onset Still's disease (AOSD, sometimes it is difficult to differentiate hepatic dysfunction due to AOSD itself from drug-induced hepatic dysfunction. Further, myalgia often occurs in patients with AOSD; however, AOSD patients complicated with myositis are rare. We report a 43-year-old Japanese man with AOSD who developed myositis and hepatic dysfunction which were deteriorated by multiple nonsteroidal antiinflammatory drugs (NSAIDs and were dramatically ameliorated by a low-dose steroid therapy. A skin biopsy of salmon pink rash which is characteristic for AOSD showed leukocytoclastic vasculitis, and the markers for vasculitis, plasma von Willebrand factor, and vascular endothelial growth factor levels were elevated in this patient, suggesting an association between AOSD and systemic vasculitis.

  18. Drug-induced leukocytoclastic vasculitis: tigecycline a rare cause

    Directory of Open Access Journals (Sweden)

    Kalpana Bhairavarasu

    2015-01-01

    Full Text Available Drug-induced leukocytoclastic vasculitis is an inflammation of blood vessels triggered by various drugs. It presents with a localized skin rash but may involve the internal organ systems, including the gastrointestinal tract, kidneys, lungs, central nervous system, and joints. The clinical recognition of drug-induced vasculitis is very important because continued use of the culprit drug can be organ or life threatening. The prognosis is excellent if the disease is limited to the skin and diagnosed promptly. The use of tigecycline has recently increased due to resistance patterns of bacteria, and it is important to recognize this potential adverse effect of this drug and to diagnose and treat the patient early to achieve a favorable outcome. To best of our knowledge, we report the first case of tigecycline-induced leukocytoclastic vasculitis.

  19. Hypertrophic cardiomyopathy secondary to hepatitis C virus-related vasculitis.

    Science.gov (United States)

    Cavalli, Giulio; Berti, Alvise; Fragasso, Gabriele; De Cobelli, Francesco

    2016-12-01

    : Almost invariably associated with chronic HCV infection, cryoglobulinemic vasculitis is a small-vessel vasculitis commonly affecting the skin, kidneys, and peripheral nervous system. Cardiac involvement, possibly due to cardiac microcirculation involvement, is an utterly rare and severe complication. We describe a case of hypertrophic cardiomyopathy secondary to cryoglobulinemic vasculitis. Evaluation with transthoracic cardiac ultrasound and cardiac MRI evidenced severe left ventricular hypertrophy and diffuse hypokinesia, a marked decrease in left ventricular ejection fraction, and a subtle late enhancement of inferior and lateral left ventricular walls. Upon clinical stabilization, the patient received treatment with anti-CD20 monoclonal antibody rituximab. Clinical and radiological follow-up with cardiac ultrasound and cardiac MRI documented a dramatic and sustained clinical improvement, with marked reduction of left ventricular hypertrophy, resolution of late enhancement, recovery of left ventricular contractility and function.

  20. Active necrotizing cerebral vasculitis in systemic lupus erythematosus.

    Science.gov (United States)

    Goel, Deepa; Reddy, S Rajashekhar; Sundaram, Challa; Prayaga, Aruna K; Rajasekhar, Liza; Narsimulu, Gumdal

    2007-12-01

    Systemic lupus erythematosus (SLE) is a multisystemic disease with varied clinical manifestations. Focal cortical brain infarcts and CNS infections are the most common neuropathological features reported in most studies. This report describes a 32-year-old woman who had repeated episodes of strokes over 5 years. In view of polyarthritis, oral ulcers, presence of high titres of serum antinuclear antibodies, high titres of double-stranded DNA and strokes, she was treated as SLE. Despite prolonged immunosuppressive therapy with azathioprine and pulse cyclophosphamide, she succumbed to a brainstem stroke. Complete body autopsy showed multiple cerebral cortical and brainstem infarcts with fibrinoid necrosis of the vessel wall. Renal infarction with healed vasculitis and systemic vasculitis involving small vessels was seen. Extensive thrombosis was remarkable by its absence. Active necrotizing vasculitis of cerebral and renal vessels is a rare complication of SLE, which contributed to a fatal outcome in this patient.

  1. Preliminary classification criteria for the cryoglobulinaemic vasculitis

    Science.gov (United States)

    De Vita, S; Soldano, F; Isola, M; Monti, G; Gabrielli, A; Tzioufas, A; Ferri, C; Ferraccioli, G F; Quartuccio, L; Corazza, L; De Marchi, G; Casals, M Ramos; Voulgarelis, M; Lenzi, M; Saccardo, F; Fraticelli, P; Mascia, M T; Sansonno, D; Cacoub, P; Tomsic, M; Tavoni, A; Pietrogrande, M; Zignego, A L; Scarpato, S; Mazzaro, C; Pioltelli, P; Steinfeld, S; Lamprecht, P; Bombardieri, S; Galli, M

    2011-01-01

    Background To develop preliminary classification criteria for the cryoglobulinaemic syndrome or cryoglobulinaemic vasculitis (CV). Methods Study part I developed a questionnaire for CV to be included in the formal, second part (study part II). Positivity of serum cryoglobulins was defined by experts as an essential condition for CV classification. In study part II, a core set of classification items (questionnaire, clinical and laboratory items, as agreed) was tested in three groups of patients and controls—that is, group A (new patients with the CV), group B (controls with serum cryoglobulins but lacking CV) and group C (controls without serum cryoglobulins but with features which can be observed in CV). Results In study part I (188 cases, 284 controls), a positive response to at least two of three selected questions showed a sensitivity of 81.9% and a specificity of 83.5% for CV. This questionnaire was employed and validated in study part II, which included 272 patients in group A and 228 controls in group B. The final classification criteria for CV, by pooling data from group A and group B, required the positivity of questionnaire plus clinical, questionnaire plus laboratory, or clinical plus laboratory items, or all the three, providing a sensitivity of 88.5% and a specificity of 93.6% for CV. By comparing data in group A versus group C (425 controls), the same classification criteria showed a sensitivity 88.5% and a specificity 97.0% for CV. Conclusion Classification criteria for CV were developed, and now need validation. PMID:21571735

  2. Urinary Soluble CD163 in Active Renal Vasculitis.

    Science.gov (United States)

    O'Reilly, Vincent P; Wong, Limy; Kennedy, Claire; Elliot, Louise A; O'Meachair, Shane; Coughlan, Alice Marie; O'Brien, Eoin C; Ryan, Michelle M; Sandoval, Diego; Connolly, Emma; Dekkema, Gerjan J; Lau, Jiaying; Abdulahad, Wayel H; Sanders, Jan-Stephan F; Heeringa, Peter; Buckley, Colm; O'Brien, Cathal; Finn, Stephen; Cohen, Clemens D; Lindemeyer, Maja T; Hickey, Fionnuala B; O'Hara, Paul V; Feighery, Conleth; Moran, Sarah M; Mellotte, George; Clarkson, Michael R; Dorman, Anthony J; Murray, Patrick T; Little, Mark A

    2016-09-01

    A specific biomarker that can separate active renal vasculitis from other causes of renal dysfunction is lacking, with a kidney biopsy often being required. Soluble CD163 (sCD163), shed by monocytes and macrophages, has been reported as a potential biomarker in diseases associated with excessive macrophage activation. Thus, we hypothesized that urinary sCD163 shed by crescent macrophages correlates with active glomerular inflammation. We detected sCD163 in rat urine early in the disease course of experimental vasculitis. Moreover, microdissected glomeruli from patients with small vessel vasculitis (SVV) had markedly higher levels of CD163 mRNA than did those from patients with lupus nephritis, diabetic nephropathy, or nephrotic syndrome. Both glomeruli and interstitium of patients with SVV strongly expressed CD163 protein. In 479 individuals, including patients with SVV, disease controls, and healthy controls, serum levels of sCD163 did not differ between the groups. However, in an inception cohort, including 177 patients with SVV, patients with active renal vasculitis had markedly higher urinary sCD163 levels than did patients in remission, disease controls, or healthy controls. Analyses in both internal and external validation cohorts confirmed these results. Setting a derived optimum cutoff for urinary sCD163 of 0.3 ng/mmol creatinine for detection of active renal vasculitis resulted in a sensitivity of 83%, specificity of 96%, and a positive likelihood ratio of 20.8. These data indicate that urinary sCD163 level associates very tightly with active renal vasculitis, and assessing this level may be a noninvasive method for diagnosing renal flare in the setting of a known diagnosis of SVV. Copyright © 2016 by the American Society of Nephrology.

  3. Ischemic Retinal Vasculitis Associated with Cataract Surgery and Intracameral Vancomycin

    Directory of Open Access Journals (Sweden)

    Lucas T. Lenci

    2015-01-01

    Full Text Available Recently, there have been reports suggesting that intracameral vancomycin has been associated with retinal vasculitis; some have described this phenomenon as postoperative hemorrhagic occlusive retinal vasculitis. We present a case of a 65-year-old woman who underwent uncomplicated phacoemulsification and posterior chamber intraocular lens implantation followed by intracameral antibiotic prophylaxis. Unlike prior reports, this report demonstrates a case of mild visual reduction and minimal inflammation with subtle but complete unilateral peripheral retinal ischemia associated with cataract surgery and intracameral vancomycin, suggesting a spectrum of toxicity that may be underrecognized.

  4. Petechiae and vasculitis in asymptomatic primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Diederichsen, H; Sørensen, P G; Mickley, H

    1985-01-01

    Recurrent petechiae of the lower legs and signs of asymptomatic primary biliary cirrhosis have been found in three women. Large immune complexes were identified in the serum of three and cryoglobulin/cryofibrinogen in two. Histological examination of skin biopsies revealed a leukocytoclastic...... vasculitis in all three patients. Direct immunofluorescent studies showed deposits of IgM, C3 and fibrinogen in and around the walls of the small vessels of the skin of two and C3 as well as fibrinogen in one patient. It is suggested that leukocytoclastic vasculitis may be a microscopic feature...... of the systemic involvement in asymptomatic primary biliary cirrhosis, and petechiae the clinical manifestation....

  5. Large vessel vasculitis and spondyloarthritis: coincidence or associated diseases?

    Science.gov (United States)

    Ernst, D; Baerlecken, N T; Schmidt, R E; Witte, T

    2014-01-01

    Although cardiac complications have been reported in established spondyloarthritis (SpA), little is known about peripheral axial SpA in large vessel vasculitis (LVV). The aim of this study was to assess the prevalence of SpA in patients with newly diagnosed LVV. Retrospective single-centre analysis of all newly diagnosed LVV patients was performed between January 2011 and December 2012. Vasculitides were confirmed on thoracic magnetic resonance imaging (MRI) or 18-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT). Patients completed a standardized questionnaire incorporating the Berlin criteria to assess inflammatory back pain. Existing scans were reassessed for sacroiliitis and ferritin antibodies measured in all patients. Fifteen patients exhibiting new LVV were identified. Diagnosis was confirmed using MRI in nine patients and FDG-PET/CT in six. Six patients (40%) fulfilled American College of Rheumatology (ACR) criteria for giant cell arteritis (GCA) and polymyalgia rheumatica (PMR), and nine PMR only. Four patients fulfilled the Berlin criteria for inflammatory back pain, with three demonstrating sacroiliitis on imaging. All remaining patients demonstrated no sacroiliitis. One further patient with LVV lacking features of inflammatory back pain had known psoriatic arthritis (PsA). Patients with coexisting SpA were younger (mean age 57 years vs. 66 years) and had higher C-reactive protein (CRP) levels (200 mg/L vs. 85 mg/L) at presentation. Four SpA patients and seven out of nine patients with isolated LVV had ferritin antibodies. We have demonstrated a higher than anticipated prevalence of SpA in LVV, given the reported 0.5-1% prevalence in the general population. Coexisting SpA should be considered in LVV patients exhibiting inflammatory back pain despite steroid initiation. Ferritin antibodies demonstrated a similarly high prevalence in aortitis and SpA as reported previously in untreated GCA and PMR.

  6. Drug-Induced Vasculitis: New Insights and a Changing Lineup of Suspects.

    Science.gov (United States)

    Grau, Rafael G

    2015-12-01

    An increasing number of therapeutic agents have been associated with a vasculitic syndrome. This usually involves small vessels, primarily capillaries, venules, and arterioles in leukocytoclastic vasculitis, small-vessel disease similar to an antineutrophil cytoplasmic antibody-related vasculitis, or mid-sized muscular arteries in a polyarteritis-like picture. Antineutrophil cytoplasmic antibodies are present in many cases of vasculitis regardless of the size of the vessel involved. Monoclonal antibodies used to treat many autoimmune disorders have become the most common agents associated with drug-induced vasculitis. Important advances in epigenetics, genetics, and neutrophil apoptosis are providing new insights into the pathogenesis of both drug-induced vasculitis and idiopathic vasculitis. Although management has not changed significantly in the past few years where withdrawal of the offending agent is the primary intervention, increasing awareness of drug-induced vasculitis can lead to earlier diagnosis and prevention of severe organ damage and fatalities.

  7. Acute type II cryoglobulinaemic vasculitis mimicking atherosclerotic peripheral vascular disease.

    LENUS (Irish Health Repository)

    Saeed, A

    2012-01-31

    Atherosclerotic peripheral vascular disease is a common presenting cause for digital ischaemia in life long smokers. Acute severe Type II Cryoglobulinaemic vasculitis is a rare yet important cause, which may present with similar clinical features and which if undiagnosed may be rapidly fatal. Following the instigation of therapy with intravenous methylprednisolone and cyclophosphamide this patient made an excellent recovery.

  8. Animal models of antineutrophil cytoplasm antibody-associated vasculitis.

    LENUS (Irish Health Repository)

    Salama, Alan D

    2012-01-01

    To provide an update on the experimental models that have been developed recapitulating clinical antineutrophil cytoplasm antibody (ANCA) associated vasculitis. The application of the models in the study of pathogenesis, and the therapeutic implications of this, are covered in the article by van Timmeren and Heeringa in this issue.

  9. Anca associated vasculitis : occurrence, prediction, prevention, and outcome of relapses

    NARCIS (Netherlands)

    Boomsma, Maarten Michiel

    2001-01-01

    During follow-up, relapses of disease activity occur in the majority of patients with ANCA associated vasculitis. The general objective brought together in this thesis was to further elucidate the characteristics and consequences of these relapses. Investigated items are the occurrence, the

  10. Prednisone and vardenafil hydrochloride for refractory levamisole-induced vasculitis.

    Science.gov (United States)

    Mandrell, Joshua; Kranc, Christina L

    2016-08-01

    Levamisole is an immunomodulatory drug that was previously used to treat various medical conditions, including parasitic infections, nephrotic syndrome, and colorectal cancer. Over the last few years, increasing amounts of levamisole have been used as an adulterant in cocaine. Levamisole-cut cocaine has become a concern because it is known to cause a necrotizing purpuric rash, autoantibody production, and life-threatening leukopenia. Mixed histologic findings of vasculitis and thrombosis are characteristic of levamisole-induced purpura. The recommended management of levamisole-induced vasculitis currently involves withdrawal of the culprit along with supportive treatment. We describe a patient with levamisole-induced vasculitis who continued to develop skin lesions despite self-reported cocaine cessation. Complete resolution of cutaneous disease occurred with the addition of oral prednisone and vardenafil hydrochloride, suggesting the possibility of a new treatment option in patients with refractory disease. In addition, we review the clinical presentation, disease course, diagnostic approach, laboratory findings, histology, and management of levamisole-induced vasculitis. The harmful effects of levamisole-cut cocaine are serious enough that public alerts have been issued to increase awareness. Clinicians should consider the possibility of levamisole exposure in cocaine users presenting with any combination of fever, neutropenia, and necrotic skin lesions, especially in acral areas including the ears.

  11. Assessment of disease activity in large-vessel vasculitis

    DEFF Research Database (Denmark)

    Aydin, Sibel Z.; Direskeneli, Haner; Merkel, Peter A.

    2017-01-01

    Objective. To arrive at consensus for candidate outcomes for disease activity assessment in largevessel vasculitis (LVV) in clinical trials. Methods.A Delphi survey including 99 items was circulated among international experts for 3 rounds. Results. Fifty-seven items were accepted for both giant...

  12. [Necrotic leg ulcer revealing vasculitis induced by vitamin K antagonists].

    Science.gov (United States)

    Chabli, H; Hocar, O; Akhdari, N; Amal, S; Hakkou, M; Hamdaoui, A

    2015-12-01

    Vitamin K antagonists are widely used in thromboembolic diseases. Hemorrhagic complications related to drug overdose represent their main side effect. We report a rare side effect, a severe and unexpected type of skin vasculitis - necrotic leg ulcer - induced by vitamin K antagonist. A 63-year-old female with a history of diabetes developed hyperalgesic necrotic ulcerations on the lower limbs one month after starting an acenocoumarol-based treatment for ischemic heart disease. Histological examination revealed lymphocytic vasculitis with fibrinoid necrosis. Etiological explorations searching for vasculitis were negative. In the absence of a precise etiology, drug-induced ulcer was suspected. Low molecular weight heparin was prescribed to replace acenocoumarol. The lesions slowly resolved with topical treatment. The chronological criteria and the negativity of etiological explorations allowed the diagnosis of vitamin K antagonist-induced necrotic skin ulcer. Clinicians should be aware of this rare complication induced by oral anticoagulants because of its practical therapeutic implications. This is the first case of necrotic leg ulcer induced by acenocoumarol corresponding histologically to necrotising lymphocytic vasculitis. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  13. Vasculitis related to viral and other microbial agents.

    Science.gov (United States)

    Teng, Gim Gee; Chatham, W Winn

    2015-04-01

    Vasculitis due to infection may occur as a consequence of the inflammation of vessel walls due to direct or contiguous infection, type II or immune complex-mediated reaction, cell-mediated hypersensitivity, or inflammation due to immune dysregulation triggered by bacterial toxin and/or superantigen production. As immunosuppressive therapy administered in the absence of antimicrobial therapy may increase morbidity and fail to effect the resolution of infection-associated vascular inflammation, it is important to consider infectious entities as potential inciting factors in vasculitis syndromes. The causality between infection and vasculitis has been established in hepatitis B-associated polyarteritis nodosa (HBV-PAN) and hepatitis C-associated (cryoglobulinemic) vasculitis (HCV-CV). The review summarizes the recent literature on the pathophysiological mechanisms and the approaches to the management of HBV-PAN and HCV-CV. Roles of other viral and microbial infections, which either manifest as vasculitic syndromes or are implicated in the pathogenesis of primary vasculitides, are also discussed. Copyright © 2015. Published by Elsevier Ltd.

  14. Motoneuronal location of the external urethral and anal sphincters : A single and double labeling study in the male and female golden hamster

    NARCIS (Netherlands)

    Gerrits, Peter O.; Sie, Judith A.M.L.; Holstege, Gerrit

    1997-01-01

    The location of external urethral (EUS) and anal sphincter (EAS) motoneurons was investigated in the golden hamster using the retrograde tracers horseradish peroxidase and cholera toxin B-subunit. Single and double labeling studies revealed that the motoneurons of the EUS and EAS were present in the

  15. Long- term outcome of paediatric patients with ANCA vasculitis

    Directory of Open Access Journals (Sweden)

    Pusey Charles D

    2011-06-01

    Full Text Available Abstract Background Primary systemic vasculitis presenting in childhood is an uncommon but serious condition. As these patients transfer to adult clinics for continuing care, defining long term outcomes with emphasis on disease and treatment- related morbidity and mortality is important. The aim of this study is to describe the long- term clinical course of paediatric patients with ANCA vasculitis. Methods The adult patients in our vasculitis clinics who had presented in childhood, with a follow up time of greater than 10 years were included. We also reviewed the literature for articles describing the clinical outcome of paediatric patients with ANCA vasculitis. Results We describe the clinical course of 8 adults who presented in childhood with ANCA vasculitis. 7 patients had Wegener's granulomatosis and 1 had microscopic polyangiitis. The median age at presentation was 11.5 years, and follow up time ranged form 11 to 30 years. Induction therapy for all patients was steroids and/or cyclophosphamide. Maintenance therapy was with azathioprine or mycophenolate mofetil. Biological agents were used in 3 patients for relapsed disease in adulthood only. Seven patients achieved complete remission. All patients experienced disease relapse, with a median of 4 episodes. Kidney function was generally well preserved, with median eGFR 76 ml/min. Only one patient developed end-stage renal failure and one patient died after 25 years of disease. Treatment-related morbidity rates were high; 7 suffered from infections, 4 were infertile, 2 had skeletal complications, and 1 developed malignancy. Conclusion Close long- term follow up of paediatric patients with ANCA vasculitis is imperative, as this patient cohort is likely to live long enough to develop significant treatment and disease- related morbidities. Prospective cohort studies with novel therapies including paediatric patients are crucial to help us determine the best approach to managing this complex group

  16. Mitoxantrone Therapy for Acute Posterior Multifocal Placoid Pigment Epitheliopathy with Cerebral Vasculitis

    Directory of Open Access Journals (Sweden)

    Hélène Massé

    2009-01-01

    Full Text Available Purpose. To report favorable outcome of a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE associated with cerebral vasculitis after treatment with immunosuppressive therapy by mitoxantrone. Design. Single case report. Method. A 22-year-old man presented with acute isolated bilateral loss of vision revealing APMPPE. Corticosteroid therapy was initiated and visual acuity gradually improved. Seventeen days later, visual function deteriorated again, associated with flu-like syndrome and severe headaches. A relapse of APMPPE was diagnosed, complicated with lymphocytic meningitis and cerebral ischemia. Intravenous therapy with mitoxantrone was performed in combination with methylprednisolone. Results. Headaches disappeared in a few days whereas visual acuity gradually improved and stabilized at 20/40 in the right eye and 20/32 in the left eye. No adverse event was observed. Clinical improvement was confirmed by magnetic resonance imaging. Conclusion. Cerebral vasculitis is the most severe complication of the extraocular manifestations of APMPEE. This diagnosis should be evoked when severe headaches or behavior disorder are associated with APMPEE.

  17. Static and fatigue tensile properties of cross-ply laminates containing vascules for self-healing applications

    International Nuclear Information System (INIS)

    Luterbacher, R; Trask, R S; Bond, I P

    2016-01-01

    The effect of including hollow channels (vascules) within cross-ply laminates on static tensile properties and fatigue performance is investigated. No change in mechanical properties or damage formation is observed when a single vascule is included in the 0/90 interface, representing 0.5% of the cross sectional area within the specimen. During tensile loading, matrix cracks develop in the 90° layers leading to a reduction of stiffness and strength (defined as the loss of linearity) and a healing agent is injected through the vascules in order to heal them and mitigate the caused degradation. Two different healing agents, a commercial low viscosity epoxy resin (RT151, Resintech) and a toughened epoxy blend (bespoke, in-house formulation) have been used to successfully recover stiffness under static loading conditions. The RT151 system recovered 75% of the initial failure strength, whereas the toughened epoxy blend achieved a recovery of 67%. Under fatigue conditions, post healing, a rapid decay of stiffness was observed as the healed damage re-opened within the first 2500 cycles. This was caused by the high fatigue loading intensity, which was near the static failure strength of the healing resin. However, the potential for ameliorating (via self-healing or autonomous repair) more diffuse transverse matrix damage via a vascular network has been shown. (paper)

  18. Long-Term Maintenance Therapy Using Rituximab-Induced Continuous B-Cell Depletion in Patients with ANCA Vasculitis

    Science.gov (United States)

    Pendergraft, William F.; Cortazar, Frank B.; Wenger, Julia; Murphy, Andrew P.; Rhee, Eugene P.; Laliberte, Karen A.; Niles, John L.

    2014-01-01

    Background and objectives Remission in the majority of ANCA vasculitis patients is not sustained after a single course of rituximab, and risk of relapse warrants development of a successful strategy to ensure durable remission. Design, setting, participants, & measurements A retrospective analysis of ANCA vasculitis patients who underwent maintenance therapy using rituximab-induced continuous B-cell depletion for up to 7 years was performed. Maintenance therapy with rituximab was initiated after achieving remission or converting from other prior maintenance therapy. Continuous B-cell depletion was achieved in all patients by scheduled rituximab administration every 4 months. Disease activity, serologic parameters, adverse events, and survival were examined. Results In the study, 172 patients (mean age=60 years, 55% women, 57% myeloperoxidase–ANCA) treated from April of 2006 to March of 2013 underwent continuous B-cell depletion with rituximab. Median remission maintenance follow-up time was 2.1 years. Complete remission (Birmingham Vasculitis Activity Score [BVAS]=0) was achieved in all patients. Major relapse (BVAS≥3) occurred in 5% of patients and was associated with weaning of other immunosuppression drugs. Remission was reinduced in all patients. Survival mirrored survival of a general age-, sex-, and ethnicity-matched United States population. Conclusion This analysis provides evidence for long-term disease control using continuous B-cell depletion. This treatment strategy in ANCA vasculitis patients also seems to result in survival rates comparable with rates in a matched reference population. These findings suggest that prospective remission maintenance treatment trials using continuous B-cell depletion are warranted. PMID:24626432

  19. Elimination of Multiple Estimation for Fault Location in Radial Power Systems by Using Fundamental Single-End Measurements

    NARCIS (Netherlands)

    Morales-Espana, G.; Mora-Floréz, J.; Vargas-Torres, H.

    2009-01-01

    This paper presents a conceptual approach for eliminating the multiple estimation problem of impedance-based fault location methods applied to power distribution systems, using the available measurements of current and voltage fundamentals at the power substation. Three test systems are used to

  20. Detecting and locating light atoms from high-resolution STEM images : The quest for a single optimal design

    NARCIS (Netherlands)

    Gonnissen, J; De Backer, A; den Dekker, A.J.; Sijbers, J.; Van Aert, S.

    2016-01-01

    In the present paper, the optimal detector design is investigated for both detecting and locating light atoms from high resolution scanning transmission electron microscopy (HR STEM) images. The principles of detection theory are used to quantify the probability of error for the detection of

  1. Cocaine-induced vasculitis: is this a new trend?

    Directory of Open Access Journals (Sweden)

    García Pérez MR

    2013-10-01

    Full Text Available Miraida Reneé García Pérez,1 Vanessa L Ortiz-González,1 Maria Betancourt,1 Rogelio Mercado21Department of Internal Medicine, San Juan City Hospital, 2Department of Dermatology, University of Puerto Rico School of Medicine, San Juan, Puerto RicoAbstract: Cocaine-induced vasculitis is a rare complication found in drug abusers. It occurs due to cocaine adulterated with levamisole. Levamisole was once used as a chemotherapy and immunomodulator for different conditions. One of the side effects of this medication is necrotizing vasculitis which has been reported in the US and Puerto Rico. Here we present another case of cocaine induced vasculitis in Puerto Rico. We describe a 43-year-old female with past medical history of bronchial asthma, migraine, and crack smoking who presented to the emergency room due to blood in her urine for 5 days. She also reported fever, chills, and fatigue. At the physical exam she had a right knee ulcer with swelling erythema, warmth, and pain. Also, she had retiform purpuric plaque lesions in her ears, bilaterally. Eroded plaques with elevated borders at left foot and finger dorsum were also present. Laboratory workup was positive for cocaine. The patient showed leucopenia and microcytic anemia with a normal absolute neutrophil count in her cell blood count. Blood cultures, urine cultures, and ulcer cultures were negative. Urinalysis was positive for proteinuria and hematuria. Also, the patient had positive perinuclear anti-neutrophil cytoplasmic antibody, cytoplasmic anti-neutrophil cytoplasmic antibody, and antinuclear antibody tests and elastase specificity. She showed negative anticardiolipin and lupus anticoagulant antibodies. Her complement levels were decreased. The punch biopsy of her ear showed superficial thrombosis of superficial vascular plexus with perivascular lymphocytic infiltrates and deeper sections showed epidermal necrosis and necrotizing vasculitis. She was started on a high dose of steroids, but

  2. Spatiotemporal analysis of single-trial EEG of emotional pictures based on independent component analysis and source location

    Science.gov (United States)

    Liu, Jiangang; Tian, Jie

    2007-03-01

    The present study combined the Independent Component Analysis (ICA) and low-resolution brain electromagnetic tomography (LORETA) algorithms to identify the spatial distribution and time course of single-trial EEG record differences between neural responses to emotional stimuli vs. the neutral. Single-trial multichannel (129-sensor) EEG records were collected from 21 healthy, right-handed subjects viewing the emotion emotional (pleasant/unpleasant) and neutral pictures selected from International Affective Picture System (IAPS). For each subject, the single-trial EEG records of each emotional pictures were concatenated with the neutral, and a three-step analysis was applied to each of them in the same way. First, the ICA was performed to decompose each concatenated single-trial EEG records into temporally independent and spatially fixed components, namely independent components (ICs). The IC associated with artifacts were isolated. Second, the clustering analysis classified, across subjects, the temporally and spatially similar ICs into the same clusters, in which nonparametric permutation test for Global Field Power (GFP) of IC projection scalp maps identified significantly different temporal segments of each emotional condition vs. neutral. Third, the brain regions accounted for those significant segments were localized spatially with LORETA analysis. In each cluster, a voxel-by-voxel randomization test identified significantly different brain regions between each emotional condition vs. the neutral. Compared to the neutral, both emotional pictures elicited activation in the visual, temporal, ventromedial and dorsomedial prefrontal cortex and anterior cingulated gyrus. In addition, the pleasant pictures activated the left middle prefrontal cortex and the posterior precuneus, while the unpleasant pictures activated the right orbitofrontal cortex, posterior cingulated gyrus and somatosensory region. Our results were well consistent with other functional imaging

  3. Small vessel vasculitis History, classification, etiology, histopathology, clinic, diagnosis and treatment

    International Nuclear Information System (INIS)

    Iglesias Gamarra, Antonio; Matteson, Eric L; Restrepo, Jose Felix

    2007-01-01

    Small-vessel vasculitis is a convenient descriptor for a wide range of diseases characterized by vascular inflammation of the venules, capillaries, and/or arterioles with pleomorphic clinical manifestations. The classical clinical phenotype is leucocytoclastic vasculitis with palpable purpura, but manifestations vary widely depending upon the organs involved. Histopathologic examination in leucocytoclastic vasculitis reveals angiocentric segmental inflammation, fibrinoid necrosis, and a neutrophilic infiltrate around the blood vessel walls with erythrocyte extravasation. The etiology of small-vessel vasculitis is unknown in many cases, but in others, drugs, post viral syndromes, malignancy, primary vasculitis such as microscopic polyarteritis, and connective tissue disorders are associated, The diagnosis of small- vessel vasculitis relies on a thorough history and physical examination, as well as relevant antibody testing including antinuclear antibody and anti neutrophil cytoplasmic antibody, hepatitis B and C serologies, assessment of complement, immunoglobulins, blood count, serum creatinine liver function tests, urinalysis, radiographic imaging and biopsy. The treatment is based primarily on corticosteroid and immunosuppressive agents

  4. Structurafand metabolic peculiarities of erythrocytes in patients with systemic vasculitis

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    M M Faslyev

    2004-01-01

    Full Text Available Obyective. To assess of functional , structural and metabolic changes of RBC in patients with systemic vasculitis. To estimate influence of RBC abnormalities on blood viscosity and hemostasis. Materials and methods. Blood samples of 75 pis with Henoch-Schonlein Purpura (HSP and 15 pts with microscopic polyarteritis (MPA were tested. The levels of ATP transporting enzymes and lipid peroxide oxidation (LPO in erythrocyte's membranes were assessed. The degree of erythrocyte membrane stability was estimated by test of osmotic and acidic membrane resistance. Results. Suppression of Na +, K +, Ca + activated ATP transporting enzyme in pts with MPA and renal form of HSP was found. This group of patients was also characterized by LPO activation, decreased osmotic and their acidic resistance of erythrocytes and their marked hyperaggregation. Conclusion. The assessment of structural and functional changes of erythrocytes helps to estimate abnormalities of blood viscosity and wo adjust management and predict prognosis in pts with systemic vasculitis.

  5. Correlation of angiography and MR imaging in cerebral vasculitis

    International Nuclear Information System (INIS)

    Cloft, H.J.; Phillips, C.D.; Dix, J.E.; McNulty, B.C.; Kallmes, D.F.; Zagardo, M.T.

    1999-01-01

    Purpose: MR imaging and cerebral angiography were correlated in patients with primary angiitis of the central nervous system (PACNS) to assess the relative roles of these imaging modalities in the diagnosis. Material and Methods: In 9 patients, MR imaging and angiography were compared with regard to the relative involvement of each major vascular territory. Vascular territories assessed were the anterior, middle, and posterior cerebral arteries, and the posterior fossa. Results: All patients had angiographic findings consistent with vasculitis in multiple vascular territories. MR findings ranged from normal to diffusely abnormal. One patient had a completely normal MR investigation. Of 50 territories affected by vasculitis on angiography, 17 (34%) were normal on MR. Conclusion: Relative to cerebral angiography, MR imaging is a poor indicator of the presence or absence of PACNS. Angiography is indicated when clinical suspicion of PACNS is strong, regardless of the findings on MR. (orig.)

  6. Central Nervous System Vasculitis: Still More Questions than Answers

    Science.gov (United States)

    Alba, Marco A; Espígol-Frigolé, Georgina; Prieto-González, Sergio; Tavera-Bahillo, Itziar; García-Martínez, Ana; Butjosa, Montserrat; Hernández-Rodríguez, José; Cid, Maria C

    2011-01-01

    The central nervous system (CNS) may be involved by a variety of inflammatory diseases of blood vessels. These include primary angiitis of the central nervous system (PACNS), a rare disorder specifically targeting the CNS vasculature, and the systemic vasculitides which may affect the CNS among other organs and systems. Both situations are severe and convey a guarded prognosis. PACNS usually presents with headache and cognitive impairment. Focal symptoms are infrequent at disease onset but are common in more advanced stages. The diagnosis of PACNS is difficult because, although magnetic resonance imaging is almost invariably abnormal, findings are non specific. Angiography has limited sensitivity and specificity. Brain and leptomeningeal biopsy may provide a definitive diagnosis when disclosing blood vessel inflammation and are also useful to exclude other conditions presenting with similar findings. However, since lesions are segmental, a normal biopsy does not completely exclude PACNS. Secondary CNS involvement by systemic vasculitis occurs in less than one fifth of patients but may be devastating. A prompt recognition and aggressive treatment is crucial to avoid permanent damage and dysfunction. Glucocorticoids and cyclophosphamide are recommended for patients with PACNS and for patients with secondary CNS involvement by small-medium-sized systemic vasculitis. CNS involvement in large-vessel vasculitis is usually managed with high-dose glucocorticoids (giant-cell arteritis) or glucocorticoids and immunosuppressive agents (Takayasu’s disease). However, in large vessel vasculitis, where CNS symptoms are usually due to involvement of extracranial arteries (Takayasu’s disease) or proximal portions of intracranial arteries (giant-cell arteritis), revascularization procedures may also have an important role. PMID:22379458

  7. Cutaneous Manifestations of Medium- and Large-Vessel Vasculitis.

    Science.gov (United States)

    Chasset, Francois; Francès, Camille

    2017-12-01

    Dermatologic manifestations are observed in almost all systemic vasculitides, even in large-and medium-vessel vasculitides, although such vessels are not found in the skin. Cutaneous manifestations may be related to a direct skin localization of the systemic vasculitis or a non-specific process associated with the vasculitis. According to the 2012 International Chapel Hill consensus, the two major variants of large-vessel vasculitides are Takayasu arteritis and giant-cell arteritis. In Europe and North America, acute inflammatory nodules or erythema nodosum-like lesions are the most commonly observed skin lesions with Takayasu arteritis. Medium-sized arteriole vasculitis of the dermis or subcutis but also septal or lobular panniculitis may be found during pathological examination. In Japan, widespread pyoderma gangrenosum-like lesions are more frequent. Cutaneous manifestations of giant-cell arteritis are rare; they are ischemic, linked to arterial occlusions, or non-ischemic, with various mechanisms. The two major medium-vessel vasculitides are Kawasaki disease and polyarteritis nodosa. Kawasaki disease is characterized by a mucocutaneous lymph node syndrome without skin vasculitis. Two subsets of polyarteritis nodosa with different skin manifestations are described, without transition from one to the other. In the systemic subset, the most frequent skin lesions are in the order of frequency purpura, livedo, and nodules. Cutaneous polyarteritis nodosa mainly features nodules, livedo racemosa, and ulcerations. Genetic screening and measurement of plasma levels of adenosine deaminase 2 should be considered for patients with uncommon systemic polyarteritis nodosa or early-onset cutaneous polyarteritis nodosa.

  8. Neonatal cerebral oxygenation is not linked to foetal vasculitis and predicts intraventricular haemorrhage in preterm infants

    DEFF Research Database (Denmark)

    Sørensen, Line Carøe; Maroun, Lisa Leth; Borch, K.

    2008-01-01

    AIM: The aim of the study was to compare the cerebral tissue oxygenation index (c-TOI) measured by near infrared spectroscopy (NIRS) in infants with and without foetal vasculitis. METHODS: Twenty-four infants with placental signs of a foetal inflammatory response (FIR), foetal vasculitis, were.......002). CONCLUSION: Cerebral oxygenation was not affected in the first day of life in preterm infants born with foetal vasculitis, while cerebral oxygenation in infants that later developed intraventricular haemorrhage was impaired Udgivelsesdato: 2008/11...

  9. Neonatal cerebral oxygenation is not linked to foetal vasculitis and predicts intraventricular haemorrhage in preterm infants

    DEFF Research Database (Denmark)

    Sorensen, Line C; Maroun, Lisa L; Borch, Klaus

    2008-01-01

    Aim: The aim of the study was to compare the cerebral tissue oxygenation index (c-TOI) measured by near infrared spectroscopy (NIRS) in infants with and without foetal vasculitis. Methods: Twenty-four infants with placental signs of a foetal inflammatory response (FIR), foetal vasculitis, were.......002). Conclusion: Cerebral oxygenation was not affected in the first day of life in preterm infants born with foetal vasculitis, while cerebral oxygenation in infants that later developed intraventricular haemorrhage was impaired....

  10. Primary testicular necrotizing vasculitis clinically presented as neoplasm of the testicle: a case report

    Directory of Open Access Journals (Sweden)

    Španjol Josip

    2011-06-01

    Full Text Available Abstract We present a case of necrotizing vasculitis with the testicle as the isolated affected organ. A 25-year-old man, pretreated for epididymo-orchitis, presented with a presumed testicular neoplasm. Radical orchiectomy was performed and diagnosis of necrotizing vasculitis was established. In the absence of any other sign of systemic disease, the diagnosis of isolated necrotizing vasculitis of the testis was confirmed. Two years after the operation, the patient showed no symptoms of systemic disease.

  11. Clinical features and outcomes of ANCA-associated renal vasculitis

    Directory of Open Access Journals (Sweden)

    Sidy Mohamed Seck

    2012-01-01

    Full Text Available To determine the patterns and outcomes of the pauci-immune vasculitis in the nephrology department at hospital La Conception in Marseille, we conducted a retrospective study including all patients with diagnosis of pauci-immune renal vasculitis between January 1, 2000 and December 31, 2007. Among 33 cases, 25 were diagnosed as Wegener granulomatosis (WG, seven as microscopic polyangitis (MPA and one as Churg-Strauss syndrome (SCS. The median age of the patients was 57.7 years and the sex-ratio (M/F was 1.6. The visceral mani-festations included kidneys (100% of patients, lungs (75%, ENT (52% of WG, and nervous system (57% of MPA. The mean serum creatinine at admission was 3.3 mg/dL. Renal biopsies revealed a pauci-immune crescentic gromerulonephritis in 96% of the cases. Two patients with WG received plasmapheresis and seven patients required emergency hemodialysis. Induction therapy comprised cyclophosphamide IV and corticosteroids, while maintenance therapy included azathioprine for the majority of patients. Eighty four percent of the patients experienced complete remission after induction therapy. During maintenance therapy relapses were more frequent among patients with MPA (28% compared to WG cases (12%. After 35 months of follow-up, eight patients ended on chronic hemodialysis, and five patients died. ANCA associated vasculitis are frequent in our patients. Long-term outcomes are relatively good despite a mortality rate of 15% and 25% of the patients entering dialysis after three years of follow-up.

  12. [Treatment of cryoglobulinemic vasculitis associated with hepatitis C virus infection].

    Science.gov (United States)

    Retamozo, Soledad; Brito-Zerón, Pilar; Ramos-Casals, Manuel

    2015-05-08

    Cryoglobulinemia is a heterogeneous systemic autoimmune disease with a wide variety of causes, symptoms and outcomes, and different etiopathogenic pathways involved in the vasculitic organ damage. The discovery of the hepatitis C virus (HCV) in 1989 changed radically the focus of research of the so-called "essential" cryoglobulinemia. Cryoglobulins can be detected in 25-30% of patients with HCV, overwhelmingly representing mixed cryoglobulins. However, only 10-15% of patients present with cryoglobulinemic vasculitis, with a broad spectrum of symptoms including mild or life-threatening manifestations. Consequently, not all patients can be uniformly treated. The key therapeutic points in HCV+ patients with cryoglobulinemic vasculitis cover different aspects. The first is to treat the underlying cause of cryoglobulinemia whenever possible, hence the use of antiviral therapies must always be considered in these patients. An individualized diagnostic approach to assess the number of organs involved and the severity of organ involvement is also essential in the therapeutic planning. This complex clinical scenario leads to an equally complex therapeutic scenario. There are three main treatment strategies for HCV-associated cryoglobulinemic vasculitis: conventional immunosuppression, antiviral treatment and biological therapies. The most recent studies are suggesting a change from the classical therapeutic approach (monotherapeutic regimens) to combination/sequential regimens, including treatments targeting the virus and those directed against the induced autoimmune disease, with the aim of blocking the various etiopathogenic pathways involved. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

  13. Cutaneous Small Vessel Vasculitis Accompanied by Pustulosis Palmaris et Plantaris

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    Motoko Kosaka

    2012-03-01

    Full Text Available We present the case of a 64-year-old woman who has suffered from pustulosis palmaris et plantaris for 10 years. At the first examination, many erythematous lesions with purpura, blood crusts, and blisters were present in the lower legs and dorsum of the feet. Painful swelling in the sternal region and dorsal pain were also noted. Elevation of the CRP and myogenic enzyme levels, and liver and renal dysfunctions were noted on blood testing. Histopathologically, leukocytoclastic vasculitis was noted in small blood vessels in the whole dermal layers, and deposition of IgM and C3 in the vascular wall was detected by the direct immunofluorescence techniques. Based on these findings, cutaneous small vessel vasculitis was diagnosed. Because the patient complained of a toothache during the clinical course, an X-ray examination was performed. On pantomography, a radicular cyst and apical periodontitis were noted. The tooth symptoms changed with exacerbation and remission of the skin symptoms. These findings indicate that odontogenic infection is very likely to be a cause of cutaneous small vessel vasculitis in a manner similar to pustulosis palmaris et plantaris.

  14. [Hepatitis B associated polyarteriitis nodosa with cerebral vasculitis].

    Science.gov (United States)

    Kohlhaas, K; Brechmann, T; Vorgerd, M

    2007-08-01

    A 53-year-old male was admitted with an acute brainstem syndrome. He developed a severe fluctuating psychosis. Because of the worsening neurological symptoms he was admitted to our neurological clinic five months after onset of the disease. On admission he showed signs of a productive psychosis in addition to akinetic-rigid parkinsonism and cerebellar symptoms. Laboratory tests revealed a HBeAg-negative hepatitis B. The initial neuroradiolgical studies showed multiple supratentorial and periventricular ischemic and hemorrhagic lesions. MR-angiography and conventional cerebral angiography demonstrated multiple irregularities of the intracranial vessels and vascular occlusions, findings which were compatible with cerebral vasculitis. The laboratory and neuroradiological studies indicated a hepatitis B-associated polyarteriitis nodosa and cerebral vasculitis. He was given oral immunsuppressive therapy (prednisolone 60 mg daily) and virostatic drug (lamivudine 100 mg daily). When the steroid dosis was reduced to 40 mg prednisolon a severe relapse of the encephalopathy occurred which was treated with the atypical antipsychotic drug risperidon, 3 mg daily, and intravenous methylprednisolone plus plasmaphereses. Later he was given prednisolone (60 mg daily) and lamivudine (100 mg daily) again which has so far stabilized the clinical course. The main treatment of the rare hepatitis B-associated polyarteriitis nodosa with cerebral vasculitis consists of oral steroids in combination with antiviral drugs. Depending on the course of the disease an escalating steroid pulse administration and plasmaphereses should be considered.

  15. A rheumatology perspective on cutaneous vasculitis: assessment and investigation for the non-rheumatologist.

    Science.gov (United States)

    Rawlings, Charlotte R; Fremlin, Georgina A; Nash, Julian; Harding, Keith

    2016-02-01

    Vasculitis, by definition, is inflammation of the vasculature. This inflammation can result in either vessel wall destruction causing aneurysm or rupture, or stenosis causing ischaemia or necrosis. This autoimmune response does not always have a clear cause. Vasculitis is a heterogeneous group of disorders that has been categorised not only by primary and secondary causes, but also by the size of the affected vessel. The secondary causes that can trigger vasculitis include infection (particularly hepatitis B and C and haemorrhagic fever);cancer, autoimmune diseases such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and Sjogren's; drugs or allergic reactions. As vasculitis can affect any part of the vasculature, it can result in a wide range of signs and symptoms. However, one of the most common presentations of vasculitis is a rash, due to small vessel vasculitis, which is most common. A vasculitic rash has certain characteristics that are easily identifiable and differentiate it from other rashes. This is a review from a rheumatologist's perspective of how to identify vasculitis skin changes. If cutaneous vasculitis is suspected, this article identifies other areas of skin that can be affected that need identification, in addition to what to screen for in the history and other differential diagnoses to consider. Subsequently, the article addresses the key investigations to request and a brief overview of the treatment principles for primary vasculitis. © 2015 Medicalhelplines.com Inc and John Wiley & Sons Ltd.

  16. Severe leukocytoclastic vasculitis secondary to the use of a naproxen and requiring amputation: a case report

    Directory of Open Access Journals (Sweden)

    Martin Jeanine

    2010-07-01

    Full Text Available Abstract Introduction Leukocytoclastic vasculitis (also known as hypersensitivity vasculitis and cutaneous necrotizing vasculitis can present with various manifestations, which often delays the diagnosis and treatment. In order to show the importance of the early recognition of leukocytoclastic vasculitis, we present a case which occurred secondary to the use of a common pharmaceutical, naproxen. We were unable to find a case of leukocytoclastic vasculitis secondary to naproxen in the literature. Case presentation We present the case of a 33-year-old African American woman with below the knee and bilateral digital gangrene from hypersensitivity vasculitis secondary to the non-steroidal anti-inflammatory medication naproxen. Conclusion This is an original case report focusing on the rheumatologic management of leukocytoclastic vasculitis. However, other specialties, such as internal medicine, dermatology, infectious disease, general surgery and pathology, can gain valuable information by reviewing this case report. Reporting a case of leukocytoclastic vasculitis secondary to treatment with naproxen will advance our understanding of this disease etiology by adding yet another non-steroidal anti-inflammatory drug to the list of potential causes of leukocytoclastic vasculitis.

  17. Detection and location of surfaces in a 3D environment through a single transducer and ultrasonic spherical caps

    Directory of Open Access Journals (Sweden)

    Fabio Tomás Moreno-Ortiz

    2017-09-01

    Full Text Available In this paper, an ultrasonic arc map method for flat mapping is extended to three-dimensional space replacing the circumference arcs by spherical caps. An enclosed environment is scanned by employing a single ultrasonic device. The range, position, and orientation of the transducer are used to digitize the uncertainty caps and place them in a three-dimensional map. Through the spatial voting method, the generated voxels are elected in order to distinguish those which mark the true position of an obstacle and discard those that are produced by cross talk, noise, fake ranges, and angular resolution. The results show that it is possible to obtain sufficient information to build a three-dimensional map for navigation by employing inexpensive sensors and a low power data processing.

  18. The Location of the Cochlear Amplifier: Spatial Representation of a Single Tone on the Guinea Pig Basilar Membrane

    Science.gov (United States)

    Russell, I. J.; Nilsen, K. E.

    1997-03-01

    Acoustic stimulation vibrates the cochlear basilar membrane, initiating a wave of displacement that travels toward the apex and reaches a peak over a restricted region according to the stimulus frequency. In this characteristic frequency region, a tone at the characteristic frequency maximally excites the sensory hair cells of the organ of Corti, which transduce it into electrical signals to produce maximum activity in the auditory nerve. Saturating, nonlinear, feedback from the motile outer hair cells is thought to provide electromechanical amplification of the travelling wave. However, neither the location nor the extent of the source of amplification, in relation to the characteristic frequency, are known. We have used a laser--diode interferometer to measure in vivo the distribution along the basilar membrane of nonlinear, saturating vibrations to 15 kHz tones. We estimate that the site of amplification for the 15 kHz region is restricted to a 1.25 mm length of basilar membrane centered on the 15 kHz place.

  19. Single- and cross-commodity discounting among cocaine addicts: the commodity and its temporal location determine discounting rate.

    Science.gov (United States)

    Bickel, Warren K; Landes, Reid D; Christensen, Darren R; Jackson, Lisa; Jones, Bryan A; Kurth-Nelson, Zeb; Redish, A David

    2011-09-01

    Intertemporal choice has provided important insights into understanding addiction, predicted drug-dependence status, and outcomes of treatment interventions. However, such analyses have largely been based on the choice of a single commodity available either immediately or later (e.g., money now vs. money later). In real life, important choices for those with addiction depend on making decisions across commodities, such as between drug and non-drug reinforcers. To date, no published study has systematically evaluated intertemporal choice using all combinations of a drug and a non-drug commodity. In this study, we examine the interaction between intertemporal choice and commodity type in the decision-making process of cocaine-dependent individuals. This study of 47 treatment-seeking cocaine addicts analyzes intertemporal choices of two commodities (equated amounts of cocaine and money), specifically between cocaine now vs. cocaine later (C-C), money now vs. money later (M-M), cocaine now vs. money later (C-M), and money now vs. cocaine later (M-C). Cocaine addicts discounted significantly more in the C-C condition than in M-M (P = 0.032), consistent with previous reports. Importantly, the two cross-commodity discounting conditions produced different results. Discounting in C-M was intermediate to the C-C and M-M rates, while the greatest degree of discounting occurred in M-C. These data indicate that the menu of commodities offered alter discounting rates in intertemporal choice and that the greatest rate is obtained when the drug is the later available commodity. Implications for understanding intertemporal choices and addiction are addressed.

  20. A case of propylthiouracil-induced antineutrophilic cytoplasmic antibody-positive vasculitis successfully treated with radioactive iodine

    Directory of Open Access Journals (Sweden)

    C. Bes

    2013-07-01

    Full Text Available Antineutrophilic cytoplasmic antibody (ANCA associated vasculitis is one of the rare complications of propylthiouracil treatment. Having a variable clinical spectrum, it may be presented with both skin limited vasculitis and life-threatening systemic vasculitis. In this study, we present a case that developed ANCA-positive vasculitis with skin and kidney involvement (hematuria and proteinuria six months after propylthiouracil treatment was initiated for toxic nodular goiter. Proteinuria recovered dramatically subsequent to radioactive iodine treatment following ceasing the drug.

  1. Necrotizing leptomeningeal vasculitis associated with a compressive meningioma in a cat: a rare paraneoplastic syndrome

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    Les J. Gabor

    2012-02-01

    Full Text Available A 17 year old cat with a compressive meningioma was found to have an intradural, severe necrotizing vasculitis, spatially un associated with the neoplasm. Paraneoplastic vasculitis has been reported in two cases in the human literature associated with meningiomas. This is the first report of such an association in a domestic species.

  2. CRYOGLOBULINEMIC VASCULITIS ASSOCIATED WITH HBV INFECTION: CLINICAL OBSERVATIONS AND LITERATURE REVIEW

    Directory of Open Access Journals (Sweden)

    N. V. Dunaeva

    2014-01-01

    Full Text Available Description of two clinical cases of chronic HBV hepatitis at cirrhotic stage associated with type III cryoglobulinemia manifested with symptoms of systemic vasculitis is presented in current article. There were no signs of HCV infection in both patients. In first case cutaneous vasulitis appeared after 19 years since serological finding of HBsAg and vasculitis progressed despite steroid therapy. Initiation of antiviral therapy (entecavir 0.5 g/day induced transient remission. After interruption of antiviral therapy vasculitis reappeared with several vasculitic ulcers on lower legs. Mild improvement of vasculitis was noted after repeated plasmapheresis, steroid and cytostatic treatment with addition of lamivudin. Despite therapy reactivation of HBV infection was detected. Lamivudin was changed to entecavir and rituximab was given in two 500 mg infusions. Combined antiviral and anti-CD20 treatment induced remission of cutaneous vasculitis and healing of leg ulcers. In other case vasculitis manifested after 21 years since detection of HBsAg with cutaneous purpura, arthritis and microhematuria. Entecavir 0.5 g/day induced rapid virological response and complete remission of symptoms related to vasculitis. Similar literature cases were reviewed and available treatment options in refractory cryoglobulinemic vasculitis were discussed.

  3. Pulmonary vasculitis may obscure large cell lung carcinoma. A case report

    NARCIS (Netherlands)

    Kreeftenberg, HG; Ligtenberg, JJM; van der Werf, TS; Timens, W; Tervaert, JWC

    2001-01-01

    Several vasculitic syndromes are recognized as paraneoplastic symdromes of an underlying malignant disease. Most frequently small vessel vasculitis of the skin has been reported. We describe the case of a 62-year-old man with a pulmonary mass due to pulmonary vasculitis. After resection of the

  4. A sphingosine 1-phosphate receptor agonist ameliorates animal model of vasculitis.

    Science.gov (United States)

    Miyabe, Chie; Miyabe, Yoshishige; Komiya, Takaki; Shioya, Hiroki; Miura, Noriko N; Takahashi, Kei; Ohno, Naohito; Tsuboi, Ryoji; Luster, Andrew D; Kawai, Shinichi; Miyasaka, Nobuyuki; Nanki, Toshihiro

    2017-04-01

    Sphingosine 1-phosphate (S1P) is a bioactive lipid that binds to cell surface receptors (S1P 1-5 ). In this study, we examined the effect of S1P 1 agonist, ONO-W061, on murine Candida albicans water-soluble fraction (CAWS)-induced vasculitis. Mice were administered ONO-W061, and the number of peripheral blood cells was counted. Vasculitis was induced by an intraperitoneal injection of CAWS. Expression of S1P receptors and CXCL1 was analyzed by quantitative RT-PCR. ONO-W061 was orally administered, and vasculitis was evaluated histologically. Number of neutrophils, macrophages and T cells in the vasculitis tissue was counted using flow cytometry. Production of chemokines from S1P-stimulated human umbilical vein endothelial cells (HUVECs) was measured by ELISA. Number of peripheral blood lymphocytes was decreased by ONO-W061. Expression of CXCL1 and S1P 1 was enhanced in CAWS-induced vasculitis tissue. Vasculitis score, CXCL1 and number of neutrophils in the vasculitis tissue were lower in ONO-W061-treated mice. Treatment of HUVECs with S1P upregulated the production of CXCL1 and IL-8 in vitro, and this was inhibited by ONO-W061. ONO-W061 significantly improved CAWS-induced vasculitis. This effect may be partly exerted through the inhibited production of chemokines by endothelial cells, which in turn could induce neutrophil recruitment into inflamed vessels.

  5. Anti-neutrophil cytoplasmic antibody-associated vasculitis with renal involvement: Analysis of 89 cases.

    Science.gov (United States)

    Caravaca-Fontán, Fernando; Yerovi, Estefanía; Delgado-Yagu E, María; Galeano, Cristina; Pampa-Saico, Saúl; Tenorio, Maria Teresa; Liaño, Fernando

    2017-01-06

    The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis with renal involvement are associated with high morbi-mortality. In this study we analyse if the prognosis of these diseases have improved in recent years, and which factors influence the outcomes. Retrospective single-centre observational study, which included all patients diagnosed with microscopic polyangiitis and granulomatosis with polyangiitis with renal involvement in the last 25 years. Demographic, clinical and biochemical parameters of prognostic interest were recorded. The differences between four chronological periods were analysed, along with the determinants of a poor outcome (death or end-stage renal disease). Eighty-nine patients were included (mean age 64±15 years). Sixty-four patients (72%) had microscopic polyangiitis and 25 (28%) granulomatosis with polyangiitis. During the study period, 37 (42%) patients died. Through Cox regression analysis, the best determinants of mortality were the initial glomerular filtration rate (HR 0.911; P=.003), Charlson comorbidity index (HR 1.513; P<.0001) and tobacco smoking (HR 1.816; P=.003). 35% developed end-stage renal disease, and the best determinants (by competing-risk regression) were: initial glomerular filtration rate (sub-hazard ratio [SHR]: 0.791; P<.0001), proteinuria (SHR: 1.313; P<.0001), and smoking status (SHR: 1.848; P=.023). No differences were found in patients' mortality or renal survival between the different study periods. Prognosis of anti-neutrophil cytoplasm antibodies vasculitis with renal involvement treated with conventional immunosuppressive therapy remains unsatisfactory, and continues to have increased long-term complications and mortality. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

  6. Incidence of malignancy in patients treated for antineutrophil cytoplasm antibody-associated vasculitis : follow-up data from European Vasculitis Study Group clinical trials

    NARCIS (Netherlands)

    Heijl, C.; Harper, L.; Flossmann, O.; Stucker, I.; Scott, D. G. I.; Watts, R. A.; Hoglund, P.; Westman, K.; Mahr, A.

    Objectives Because standard immunosuppressive treatment for antineutrophil cytoplasm antibody-associated vasculitis (AAV) (granulomatosis with polyangiitis (Wegener's) (GPA) and microscopic polyangiitis (MPA)) has been associated with a significant risk of developing cancer, the cancer incidence of

  7. Determinants of Vessel Targeting in Vasculitis

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    Gary S. Hoffman

    2004-01-01

    Full Text Available Studies of autoimmune diseases have not yet elucidated why certain organs or vessels become the objects of injury while others are spared. This paper will explore the hypothesis that important differences exist in regions of the aorta that determine vulnerability to diseases, such as atherosclerosis, aortitis, giant cell arteritis and Takayasu's disease. The reader is invited to reassess; (1 whether the aorta is indeed a single homogeneous structure, and (2 whether the initial stage of aortitis (and indeed other diseases considered “autoimmune” may be primarily due to acquired alterations of substrate, that influence unique immune profiles, which by themselves may not be pathogenic. Disease susceptibility and patterns are influenced by many factors that are inborn and acquired. Examples include genetic background, gender, ethnicity, aging, prior and concomitant illnesses, habits, diet, toxin and environmental exposures. Studies of vascular diseases must assess how such variables may affect regional differences in endothelial cells, subendothelial matrix, vascular smooth muscle and the response of each to a variety of stimuli.

  8. An improved fault detection classification and location scheme based on wavelet transform and artificial neural network for six phase transmission line using single end data only.

    Science.gov (United States)

    Koley, Ebha; Verma, Khushaboo; Ghosh, Subhojit

    2015-01-01

    Restrictions on right of way and increasing power demand has boosted development of six phase transmission. It offers a viable alternative for transmitting more power, without major modification in existing structure of three phase double circuit transmission system. Inspite of the advantages, low acceptance of six phase system is attributed to the unavailability of a proper protection scheme. The complexity arising from large number of possible faults in six phase lines makes the protection quite challenging. The proposed work presents a hybrid wavelet transform and modular artificial neural network based fault detector, classifier and locator for six phase lines using single end data only. The standard deviation of the approximate coefficients of voltage and current signals obtained using discrete wavelet transform are applied as input to the modular artificial neural network for fault classification and location. The proposed scheme has been tested for all 120 types of shunt faults with variation in location, fault resistance, fault inception angles. The variation in power system parameters viz. short circuit capacity of the source and its X/R ratio, voltage, frequency and CT saturation has also been investigated. The result confirms the effectiveness and reliability of the proposed protection scheme which makes it ideal for real time implementation.

  9. Aggressive cutaneous vasculitis in a patient with chronic lymphatic leukemia following granulocyte colony stimulating factor injection: a case report

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    El Husseiny Noha M

    2011-03-01

    Full Text Available Abstract Introduction Vasculitis has been reported in a few cases of chronic lymphatic leukemia and with granulocytic colony-stimulating factor therapy. Those with granulocytic colony-stimulating factor occurred after prolonged therapy and there was a rise in total leukocyte count unlike that in our patient who received just a single injection for the first time. Case presentation We report the case of a 64-year-old Egyptian man with chronic lymphatic leukemia who developed progressive cutaneous vasculitic lesions following injection of a single dose of a granulocytic colony stimulating factor before a third cycle of chemotherapy to improve neutropenia. This is an unusual case and the pathogenesis is not fully understood. Our patient was not on any medical treatment except for bisoprolol for ischemic heart disease. Although aggressive management with steroids, anticoagulation and plasmapheresis had been carried out, the condition was aggressive and the patient's consciousness deteriorated. A magnetic resonance imaging scan of his brain revealed multiple ischemic foci that could be attributed to vasculitis of the brain. Conclusion The aim of this case report is to highlight the importance of monitoring patients on granulocytic colony-stimulating factor therapy, especially in the context of other conditions (such as a hematological malignancy that may lead to an adverse outcome.

  10. In vivo three-dimensional imaging analysis of femoral and tibial tunnel locations in single and double bundle anterior cruciate ligament reconstructions.

    Science.gov (United States)

    Yang, Jae-Hyuk; Chang, Minho; Kwak, Dai-Soon; Jang, Ki-Mo; Wang, Joon Ho

    2014-03-01

    Anatomic footprint restoration of anterior cruciate ligament (ACL) is recommended during reconstruction surgery. The purpose of this study was to compare and analyze the femoral and tibial tunnel positions of transtibial single bundle (SB) and transportal double bundle (DB) ACL reconstruction using three-dimensional computed tomography (3D-CT). In this study, 26 patients who underwent transtibial SB ACL reconstruction and 27 patients with transportal DB ACL reconstruction using hamstring autograft. 3D-CTs were taken within 1 week after the operation. The obtained digital images were then imported into the commercial package Geomagic Studio v10.0. The femoral tunnel positions were evaluated using the quadrant method. The mean, standard deviation, standard error, minimum, maximum, and 95% confidence interval values were determined for each measurement. The femoral tunnel for the SB technique was located 35.07% ± 5.33% in depth and 16.62% ± 4.99% in height. The anteromedial (AM) and posterolateral (PL) tunnel of DB technique was located 30.48% ± 5.02% in depth, 17.12% ± 5.84% in height and 34.76% ± 5.87% in depth, 45.55% ± 6.88% in height, respectively. The tibial tunnel with the SB technique was located 45.43% ± 4.81% from the anterior margin and 47.62% ± 2.51% from the medial tibial articular margin. The AM and PL tunnel of the DB technique was located 33.76% ± 7.83% from the anterior margin, 45.56% ± 2.71% from the medial tibial articular margin and 53.19% ± 3.74% from the anterior margin, 46.00% ± 2.48% from the medial tibial articular margin, respectively. The tibial tunnel position with the transtibial SB technique was located between the AM and PL tunnel positions formed with the transportal DB technique. Using the 3D-CT measuring method, the location of the tibia tunnel was between the AM and PL footprints, but the center of the femoral tunnel was at more shallow position from the AM bundle footprint when ACL reconstruction was performed by the

  11. Platelet counts as a biomarker in ANCA-associated vasculitis.

    Science.gov (United States)

    Willeke, P; Kümpers, P; Schlüter, B; Limani, A; Becker, H; Schotte, H

    2015-01-01

    To determine whether platelet (PLT) counts might serve as a biomarker to distinguish between active anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and remission and also between active disease and systemic infection. PLTs were analysed before treatment in patients with AAV in the active state and in remission. PLTs were also analysed in AAV patients with acute infections. The results were correlated with clinical manifestations, the Birmingham Vasculitis Activity Score version 3 [BVAS(v.3)], and other laboratory findings [i.e. C-reactive protein (CRP), leucocytes, differential count, procalcitonin (PCT)]. Diagnostic accuracy was calculated with a receiver operating characteristic (ROC) curve. PLT counts were significantly increased in 98 patients with AAV during the active disease state [median: 405 PLTs/nL; interquartile range (IQR) 288-504] compared to patients in remission (246 PLT/nL; IQR 214-289) (p counts in active disease with the BVAS(v.3) (r = 0.582, p counts exhibited significantly lower values (226 PLT/nL; IQR 163-273) compared to patients with active disease (p active disease from systemic infection (AUC 0.868) compared to leucocytes (AUC 0.590), CRP (AUC 0.522), or procalcitonin (AUC 0.515) (p counts were found to correlate with disease activity in AAV and thus may be used to represent immunological activity. In addition, PLT counts serve as a marker that can distinguish acute infection from active disease.

  12. Posterior Spinal Artery Aneurysm Presenting with Leukocytoclastic Vasculitis.

    Science.gov (United States)

    Hill, Travis C; Tanweer, Omar; Thomas, Cheddhi; Engler, John; Shapiro, Maksim; Becske, Tibor; Huang, Paul P

    2016-03-01

    Rupture of isolated posterior spinal artery (PSA) aneurysms is a rare cause of subarachnoid hemorrhage (SAH) that presents unique diagnostic challenges owing to a nuanced clinical presentation. Here, we report on the diagnosis and management of the first known case of an isolated PSA aneurysm in the context of leukocytoclastic vasculitis. A 53-year-old male presented to an outside institution with acute bilateral lower extremity paralysis 9 days after admission for recurrent cellulitis. Early magnetic resonance imaging was read as negative and repeat imaging 15 days after presentation revealed SAH and a compressive spinal subdural hematoma. Angiography identified a PSA aneurysm at T9, as well as other areas suspicious for inflammatory or post-hemorrhagic reactive changes. The patient underwent a multilevel laminectomy for clot evacuation and aneurysm resection to prevent future hemorrhage and to establish a diagnosis. The postoperative course was complicated by medical issues and led to the diagnosis of leukocytoclastic vasculitis that may have predisposed the patient to aneurysm development. Literature review reveals greater mortality for cervical lesions than thoracolumbar lesions and that the presence of meningitic symptoms portents better functional outcome than symptoms of cord compression. The outcome obtained in this case is consistent with outcomes reported in the literature.

  13. Rituximab in the treatment of refractory lupus nephritis with vasculitis

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    Huseyin Kadikoy

    2012-01-01

    Full Text Available Dysfunction of the B lymphocyte, an important component of adaptive immunity, is thought to be important in the pathogenesis of lupus nephritis (LN. There are several novel strategies emerging including B-cell depletion by the monoclonal antibodies to B-cell markers, rituximab. We describe an unusual clinical response of a 22-year-old Hispanic woman with class IV LN with vasculitis while on dialysis to cyclophosphamide (CY and adjunct rituximab. The patient had a history of class III/V LN and was treated with nine months of CY and maintenance therapy with mycophenolate mofetil (MMF for three years. While on MMF, the patient deve-loped class IV LN with vasculitis leading to end-stage renal disease (ESRD. While the patient was on peritoneal dialysis, the patient was treated with two doses of rituximab and six doses of intravenous CY. The patient responded to this regimen and recovered kidney function within four months. The kidney function remained stable nine months after discontinuing peritoneal dialysis.

  14. Cocaine-Levamisole-Induced Vasculitis/Vasculopathy Syndrome.

    Science.gov (United States)

    Marquez, Javier; Aguirre, Lina; Muñoz, Carolina; Echeverri, Andres; Restrepo, Mauricio; Pinto, Luis F

    2017-06-01

    To understand the clinical spectrum of cocaine-levamisole-induced vasculitis. Worldwide recreational drug consumption is high among the adult population from various social strata. The use of cocaine with levamisole, a frequently added antiparasitic diluent, favors the manifestations of vasculitic lesions, especially in the skin. New insights into immunological mechanisms involved in the pathogenesis of the disease. There are still many unknown aspects in the pathogenesis of this disease, such as the immune system interaction with p-ANCAs and the release of inflammatory NETs (neutrophil extracellular traps), which are the origin of auto-antigens and tissue damage, manifesting as vasculitic purpura on the skin. The clinical presentation constitutes a challenge for the clinician to be able to distinguish it from small-vessel vasculitides. This paper intends to improve the understanding of this condition, exhibiting the broad clinical spectrum of local and systemic manifestations of cocaine-levamisole-induced vasculitis, to facilitate a timely diagnosis, in order to take corrective measures and avoid sequelae, along with tissue damage and the consequent deformities and permanent scars.

  15. MRI in human immunodeficiency virus-associated cerebral vasculitis

    International Nuclear Information System (INIS)

    Berkefeld, J.; Lanfermann, H.

    2000-01-01

    Cerebral ischaemia caused by inflammatory vasculopathies has been described as complication of human immunodeficiency virus (HIV) infection. Imaging studies have shown ischaemic lesions and changes of the vascular lumen, but did not allow demonstration of abnormalities within the vessel wall itself. Two HIV-infected men presented with symptoms of a transient ischaemic attack. Initial MRI of the first showed no infarct; in the second two small lacunar lesions were detected. In both cases, multiplanar 3-mm slice contrast-enhanced T1-weighted images showed aneurysmal dilatation, with thickening and contrast enhancement of the wall of the internal carotid and middle cerebral (MCA) arteries. These findings were interpreted as indicating cerebral vasculitis. In the first patient the vasculopathy progressed to carotid artery occlusion, and he developed an infarct in the MCA territory, but then remained neurologically stable. In the second patient varicella zoster virus (VZV) infection was the probable cause of vasculitis. The clinical deficits and vasculitic MRI changes regressed with antiviral and immunosuppressive therapy. (orig.)

  16. Monitoring Vasculitis with 18F-FDG PET

    International Nuclear Information System (INIS)

    Jan BUCERIUS, Jan

    2016-01-01

    Whereas in the past the term “vasculitis” was most frequently used in context with systemic vasculitides, such as the large vessel vasculitides (LVV) Takayasa arteritis and giant cell arteritis, characterized by inflammation of blood vessel walls, it nowadays comprises also inflammatory changes of the vessel wall as a substantial part of the atherosclerotic disease process. Implementing non-invasive imaging techniques, such as computed tomography angiography (CTA), magnetic resonance angiography (MRA) as well as positron emission tomography (PET) in the diagnostic algorithm of atherosclerosis and LVV, depicts a promising step towards an earlier detection with a, consecutively, improved therapeutic approach and potentially prognostic benefit in patients suffering from vasculitis. Mainly molecular imaging with 18F-fluorodeoxyglucose (FDG) PET seems to be promising in offering an early and sensitive identification of inflammatory changes in both, atherosclerosis and LVV. This review will therefore provide an overview on the diagnostic performance and clinical relevance of FDG-PET in monitoring vasculitis in atherosclerosis and LVV, with a focus on LVV.

  17. Eosinophils in vasculitis: characteristics and roles in pathogenesis

    Science.gov (United States)

    Khoury, Paneez; Grayson, Peter C.; Klion, Amy D.

    2016-01-01

    Eosinophils are multifunctional granular leukocytes that are implicated in the pathogenesis of a wide variety of disorders, including asthma, helminth infection, and rare hypereosinophilic syndromes. Although peripheral and tissue eosinophilia can be a feature of many types of small-vessel and medium-vessel vasculitis, the role of eosinophils has been best studied in eosinophilic granulomatosis with polyangiitis (EGPA), where eosinophils are a characteristic finding in all three clinical stages of the disorder. Whereas numerous studies have demonstrated an association between the presence of eosinophils and markers of eosinophil activation in the blood and tissues of patients with EGPA, the precise role of eosinophils in disease pathogenesis has been difficult to ascertain owing to the complexity of the disease process. In this regard, results of clinical trials using novel agents that specifically target eosinophils are providing the first direct evidence of a central role of eosinophils in EGPA. This Review focuses on the aspects of eosinophil biology most relevant to the pathogenesis of vasculitis and provides an update of current knowledge regarding the role of eosinophils in EGPA and other vasculitides. PMID:25003763

  18. Pauci-Immune Crescentic Glomerulonephritis: An ANCA-Associated Vasculitis

    Directory of Open Access Journals (Sweden)

    Rafeel Syed

    2015-01-01

    Full Text Available Rapidly progressive glomerulonephritis (RPGN is a syndrome signified by a precipitous loss of renal function, with features of glomerulonephritis including dysmorphic erythrocyturia and glomerular proteinuria. RPGN is associated with extensive crescent formation, and, thus, the clinical term RPGN is often used interchangeably with the pathologic term crescentic glomerulonephritis (CGN. From an immunopathologic standpoint, primary RPGN is divided into pauci-immune GN (PICG, anti-GBM GN, and immune complex GN. PICG, the most common etiology of primary RPGN, refers to a necrotizing glomerulonephritis with few or no immune deposits by immunofluorescence (IF or electron microscopy (EM. In most patients, pauci-immune CGN is a component of a systemic small vessel vasculitis such as granulomatosis with polyangiitis (GPA. Approximately 90% of patients with PICG have circulating ANCA antibodies, leading to the nomenclature ANCA-associated vasculitis (AAV. Recent research has identified several other antibodies associated with PICG, which is now understood to be a complex spectrum of disease with considerable overlap in terms of clinical phenotype and outcomes. In addition, several genetic and environmental factors have recently been implicated in the pathogenesis of this disorder. With new prognostic classifications, enhanced understanding of immunopathologic mechanisms, and novel treatment paradigms, clinical and experimental interest in PICG remains high.

  19. MR-angiography in vasculitis and benign angiopathy of the central nervous system

    International Nuclear Information System (INIS)

    Schlueter, A.; Hirsch, W.; Jassoy, A.; Behrmann, C.; Spielmann, R.P.; Kornhuber, M.; Keysser, G.

    2001-01-01

    To evaluate TOF 3D magnetic resonance angiography (MRA) of the intracranial arteries in patients with vasculitis or vasculitis-like benign angiopathy of the central nervous system (CNS). Method: The results of MRA in 20 patients with clinically and radiographically proven vasculitis (17/20) or vasculitis-like benign angiopathy (3/20) of the CNS were retrospectively analysed. Patients with hyperintense lesions of more than 3 mm on T 2 -weighted MRI images were included in this trial. An inflammatory, embolic, neurodegenerative or metastatic origin of these lesions was excluded by extensive clinical studies. For the MR-examination a TOF 3D FISP sequence was used on a 1.5 T imager. Results: MRA showed characteristic changes for vasculitis or angiopathy in 15 of 20 patients (75%). Conclusions: In patients suspected of having a vasculitis or vasculitis-like angiopathy, MRA is recommended as a non-invasive modality. If the results of MRI and extensive clinical studies are carefully correlated, MRA may substitute conventional angiography in cases with typical vascular changes. (orig.) [de

  20. Red Blood Cell Distribution Width and Neutrophil-to-Lymphocyte Ratio in Patients with Cutaneous Vasculitis.

    Science.gov (United States)

    Emiroglu, Nazan; Cengiz, Fatma Pelin; Bahalı, Anıl Gulsel; Ozkaya, Dilek Biyik; Su, Ozlem; Onsun, Nahide

    2017-03-01

    Vasculitis represents a specific pattern of inflammation of the blood vessel wall that can occur in any organ system of the body. The neutrophil to lymphocyte ratio (NLR) and red blood cell distribution width (RDW) are currently used as markers of inflammation in several diseases. This study analyzed C-reactive protein level (CRP), erythrocyte sedimentation rate (ESR), white blood cell (WBC), NLR, and RDW in patients who had cutaneous vasculitis, or cutaneous vasculitis with systemic involvement, and in healthy controls. A total of 85 individuals were included in our study: 45 with vasculitis and 40 healthy controls. Patients who had complete blood count (CBC) analysis, CRP, and ESR at the time of skin biopsy were included in the study. NLR was calculated from these parameters. NLR, CRP, ESR, and WBC were significantly higher in patients with vasculitis than in healthy controls (p≤0.05), but RDW did not significantly differ between the two groups. This study suggests that blood NLR may be used for predicting vasculitis, especially cutaneous vasculitis with systemic involvement. © 2017 by the Association of Clinical Scientists, Inc.

  1. Leukocytoclastic vasculitis in children: clinical characteristics, subtypes, causes and direct immunofluorescence findings of 56 biopsy-confirmed cases.

    Science.gov (United States)

    Johnson, E F; Wetter, D A; Lehman, J S; Hand, J L; Davis, D M R; Tollefson, M M

    2017-03-01

    Leukocytoclastic vasculitis (LCV) in children is a complex group of conditions. This study presents the demographics, clinical features, direct immunofluorescence (DIF) results and suspected aetiologies of 56 biopsy-confirmed cases of leukocytoclastic vasculitis in children. Retrospective review of 56 children seen at Mayo Clinic in Rochester, Minnesota, from 1993 to 2013 with clinical features and cutaneous biopsy consistent with LCV. Twenty-seven (48%) cases were found to be due to IgA vasculitis (Henoch-Schonlein purpura). The remaining cases were found to be due to cutaneous small-vessel vasculitis (n = 19, 34%), urticarial vasculitis (n = 5, 9%), ANCA-associated vasculitis (n = 4, 7%) and acute haemorrhagic oedema of infancy (n = 1, 2%). IgA vasculitis was found to be associated with abdominal pain (P = 0.008), whereas the non-IgA vasculitis group was associated with headache (P = 0.052). Children with IgA vasculitis had palpable purpura (P = <0.001), petechia (P = 0.057), vesicles (P = 0.009) and involvement of the buttock (P = 0.004) more frequently than the non-IgA vasculitis group. On DIF, perivascular IgA was positive in IgA vasculitis compared to non-IgA vasculitis cases (P = <0.001), the other conjugates were similar between the two groups. The most common subtype of biopsy-confirmed LCV in children is IgA vasculitis. Clinical features, exam characteristics and DIF results can be helpful in determining the subtype of cutaneous vasculitis in children. © 2016 European Academy of Dermatology and Venereology.

  2. ANCA-associated vasculitis in scleroderma: a case series of fourteen patients

    Directory of Open Access Journals (Sweden)

    Kimberly P. Liang

    2011-01-01

    Full Text Available Antimyeloperoxidase (MPO, perinuclear antineutrophil cytoplasmic antibodies (pANCA, and/or clinically evident vasculitis in patients with scleroderma have been reported only rarely. The clinical significance and prognosis of ANCA-associated vasculitis in systemic sclerosis is uncertain. To report a case and identify the clinical characteristics of scleroderma patients with ANCA-associated vasculitis. Patients with both vasculitis and scleroderma occurring between 1976 to 2006 were identified using an electronic diagnostic index. These diagnoses were confirmed by retrospective review of complete medical records. Clinical features and outcomes recorded included age at vasculitis diagnosis, connective tissue disease (CTD features, type of scleroderma (limited or diffuse; ANCA serology, vasculitic organ system manifestations; and death. Fourteen cases of scleroderma patients with ANCA-associated and/or small vessel vasculitis were identified. The majority (71% were female, with mean age at vasculitis diagnosis 53 years. Seven patients (50% had overlap CTD features, and the majority (79% had limited variant of scleroderma. All of the 10 patients tested were MPO and pANCA positive. Seven patients (50% had glomerulonephritis, 11 (79% pulmonary involvement including 3 with pulmonary-renal syndrome, 6 skin purpura, and 5 mononeuritis multiplex and/or peripheral neuropathy. Six patients (43% died during followup to 2008. The presence of pANCA-associated small vessel vasculitis is a rarely reported complication of scleroderma. It occurs most commonly in women with limited scleroderma and most commonly includes pulmonary and/or renal involvement, including severe organ-threatening manifestations and death. Further studies are needed to clarify the role and clinical impact of ANCA in scleroderma patients with and without vasculitis.

  3. Recurrence and graft loss after renal transplantation in adults with IgA vasculitis.

    Science.gov (United States)

    Kawabe, Mayuko; Yamamoto, Izumi; Komatsuzaki, Yo; Yamakawa, Takafumi; Katsumata, Haruki; Katsuma, Ai; Mafune, Aki; Nakada, Yasuyuki; Kobayashi, Akimitsu; Tanno, Yudo; Ohkido, Ichiro; Tsuboi, Nobuo; Yokoyama, Keitaro; Horita, Shigeru; Okumi, Masayoshi; Ishida, Hideki; Yamamoto, Hiroyasu; Yokoo, Takashi; Tanabe, Kazunari

    2017-08-01

    IgA vasculitis, a rare condition resulting in end-stage renal disease, is a small-vessel vasculitis that affects the kidney in 49-83 % of adults. The reported recurrence rate of IgA vasculitis in renal transplant recipients is 11.5-60 %, leading to graft loss in 0-50 % of these patients. However, limited data are available on recurrence and graft loss after renal transplantation. We evaluated renal transplant recipients seen from 1987 to 2015 at the Jikei University School of Medicine and the Department of Urology, Tokyo Women's Medical University. Using a 1:2 match, 21 patients with IgA vasculitis and 42 controls were selected. The mean post-transplant follow-up was 121 ± 69 months for IgA vasculitis and 147 ± 66 months for the controls. The 15-year patient survival was 100 % in IgA vasculitis and 97.6 % in the controls (p = 0.22). The 5-, 10-, and 15-year graft survival rates were 95.2, 90.5, and 81 % in IgA vasculitis and 100, 90.5, and 88.1 % in the controls, respectively (p = 0.63). The recurrence rate was 28.6 % (6 of 21 cases) and half of them (3 of 6 cases) showed histological activity (ISKDC III). We treated them with methylprednisolone pulse therapy and/or tonsillectomy. None of the recurrence cases lost the allograft. The long-term patient and graft survival of IgA vasculitis in renal transplantation were comparable with the previous reports. The recurrence rate was 28.6 %, but none of the recurrent cases showed allograft loss in this study. We speculate that methylprednisolone pulse therapy and/or tonsillectomy prevent the progression of recurrent IgA vasculitis.

  4. Causal Attributions about Disease-Onset and Relapse in Patients with Systemic Vasculitis

    Science.gov (United States)

    Grayson, Peter C.; Amudala, Naomi A.; McAlear, Carol A.; Leduc, Renée L.; Shereff, Denise; Richesson, Rachel; Fraenkel, Liana; Merkel, Peter A.

    2014-01-01

    Objectives Patients vary in their beliefs related to the cause of serious illness. The impact of these beliefs among patients with systemic vasculitis is not known. This study aimed to describe causal attributions about disease-onset and relapse in systemic vasculitis and to examine whether causal beliefs a) differ by type of vasculitis; and b) are associated with negative health outcomes. Methods Patients with vasculitis were recruited to complete an online questionnaire. Categories of causal beliefs were assessed with the Revised Illness Perception Questionnaire (IPQ-R). Differences in beliefs about disease-onset versus relapse were compared across different forms of vasculitis. Causal beliefs were assessed in association with several health outcomes including fatigue, functional impairments, and personal understanding of the condition. Results 692 patients representing 9 forms of vasculitis completed the questionnaire. The majority (90%) of patients had beliefs about the cause of their illness. Causal attributions were highly variable, but altered immunity and stress were the most commonly agreed upon causal beliefs. Frequencies of causal beliefs were strikingly similar across different forms of vasculitis, with few notable exceptions primarily in Behçet’s disease. Beliefs differed about causes of disease-onset versus relapse. Specific beliefs about disease-onset and relapse were weakly associated with fatigue, functional impairments, and understanding of the condition. Conclusion Patient beliefs related to the cause of systemic vasculitis are highly variable. Patterns of causal beliefs are associated with important negative health outcomes. Clinicians who care for patients with vasculitis should be mindful of these associations and consider asking about patients’ causal beliefs. PMID:24634202

  5. A project by the SIDeMaST Immunopathology Group on cutaneous vasculitis.

    Science.gov (United States)

    Papini, M; Quaglino, P; La Placa, M; Marzano, A V

    2015-04-01

    Vasculitides are a challenge to the clinician, in terms of both diagnosis and therapy. Multiple classification systems have been implemented and the numerous classification schemes reflect the complexity of establishing a simple classification that could be functional for daily care. Although vasculitis classification has become increasingly elaborated, some areas remain ill defined. Some forms of vasculitis are still difficult to assign to a specific disease entity. Generally accepted operational criteria are available for many vasculitides, but for some entities there are no effective criteria. Moreover, diagnostic criteria for vasculitis with sufficient strength and/or confidence that can be universally accepted are not yet available. The need for diagnostic criteria validated and agreed upon is particularly relevant in the context of cutaneous vasculitis. The project of the SIDeMaST Italian Group of Immunopathology on cutaneous vasculitis is a national prospective observational study designed to develop and validate diagnostic criteria and to improve and validate classification criteria for cutaneous small vessel vasculitis also known as leukocytoclastic vasculitis (CLV). Primary objective of the study will also be that of developing the CUtaneous VAsculitis Severity Index (CUVASI). Secondary objectives of the project will be: 1) definition of the etiological agents that are most frequently associated with CLV; 2) search for possible correlations between causative agent and peculiar clinical and/or histopathological aspects; 3) evaluation of immunofluorescence pattern observed in this specific group of primitive cutaneous vasculitis in order to characterize the diagnostic sensitivity and specificity of this technique; 4) identification of a set of clinical investigations and laboratory tests to be performed for a correct CLV assessment. Actually 15 Italian dermatological clinics are contributing to the project and anticipated recruiting >100 patients with CLV

  6. Focal cerebral vasculitis associated with circulating immune complexes and brain irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Groothuis, D.R.; Mikhael, M.A.

    1986-06-01

    In this report we describe a patient with a benign glioma treated with surgery and radiation. After a period of stability he developed subacute bacterial endocarditis, and deteriorated neurologically. Computed tomographic scans did not show recurrent tumor. An angiogram showed vasculitis restricted to the previously irradiated area. Secondary to subacute bacterial endocarditis was the presence of high levels of circulating immune complexes. His neurological status was unchanged after antibiotics, but improved after treatment with dexamethasone. We interpret the clinical course as an immune-complex-mediated vasculitis superimposed on a subclinical radiation vasculitis. This case supports the hypothesis that immune mechanisms may be involved in delayed radiation injury to the nervous system.

  7. Omalizumab for Urticarial Vasculitis: Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Misbah Nasheela Ghazanfar

    2015-01-01

    Full Text Available Urticarial vasculitis is characterised by inflamed itching or burning red patches or wheals that resemble urticaria but persist for greater than 24 hours. It is often idiopathic but is sometimes associated with collagen-vascular disease, particularly systemic lupus erythematosus. Treatment options include oral antihistamines, oral corticosteroids, dapsone, colchicine or hydroxychloroquine. We describe a male patient with urticarial vasculitis who was treated with omalizumab (anti-IgE with convincing results and provide a review of previous reports of patients with urticarial vasculitis treated with omalizumab.

  8. Caracterización clínica de las vasculitis cutáneas

    OpenAIRE

    Loricera García, Javier

    2015-01-01

    RESUMEN: El término de vasculitis cutánea (VC) comprende un conjunto de síndromes caracterizados por la inflamación de los vasos de la piel. Clínicamente se va a traducir fundamentalmente en una púrpura palpable e histológicamente en una vasculitis leucocitoclástica. La VC puede tratarse de un proceso primario o bien ser secundaria a una causa subyacente como vasculitis necrotizantes sistémicas, otras alteraciones del tejido conectivo, infecciones sistémicas o neoplasias. Una adecuada clasifi...

  9. Focal cerebral vasculitis associated with circulating immune complexes and brain irradiation

    International Nuclear Information System (INIS)

    Groothuis, D.R.; Mikhael, M.A.

    1986-01-01

    In this report we describe a patient with a benign glioma treated with surgery and radiation. After a period of stability he developed subacute bacterial endocarditis, and deteriorated neurologically. Computed tomographic scans did not show recurrent tumor. An angiogram showed vasculitis restricted to the previously irradiated area. Secondary to subacute bacterial endocarditis was the presence of high levels of circulating immune complexes. His neurological status was unchanged after antibiotics, but improved after treatment with dexamethasone. We interpret the clinical course as an immune-complex-mediated vasculitis superimposed on a subclinical radiation vasculitis. This case supports the hypothesis that immune mechanisms may be involved in delayed radiation injury to the nervous system

  10. [IgA vasculitis (Henoch-Schönlein purpura)].

    Science.gov (United States)

    Dylewska, Magdalena; Wieliczko, Monika

    IgA vasculitis (Schönlein-Henoch purpura) is a systemic inflammation of the small vessels associated with the deposition of IgA antibodies in the vascular wall. Typical clinical symptoms are: skin lesions (purpura), joint pain, abdominal discomfort and renal disorder (most common haematuria/proteinuria). The disease affects usually the pediatric population; in those patients its course is benign and usually not associated with permanent complications. However, adults often present severe symptoms of organ damage, and the risk of complications, especially chronic renal failure is high. The diagnosis is based on clinical signs and skin biopsy. In mild cases the treatment is symptomatic. In cases with severe clinical symptoms corticosteroids are the basis of the therapy.

  11. Biomarkers of IgA vasculitis nephritis in children

    Science.gov (United States)

    Pillebout, Evangeline; Jamin, Agnès; Ayari, Hamza; Housset, Pierre; Pierre, Melissa; Sauvaget, Virginia; Viglietti, Denis; Deschenes, Georges

    2017-01-01

    Henoch–Schönlein purpura is a systemic vasculitis characterized by IgA deposits, which target the skin, joints, and kidneys, among other organs. In children, prognosis is often good but little is known about biomarkers of pediatric nephritis. We hypothesized that biological markers, including cytokines, immunoglobulins, IgA-immune complexes, IgA glycosylation and neutrophil gelatinase-associated lipocalin (NGAL), may discriminate IgA vasculitis (IgAV) pediatric patients with renal involvement from those without renal involvement. Fifty children at the time of IgAV rash between 2010 and 2015 were prospectively enrolled and compared to 21 controls. All patients were assessed for clinical and biological parameters at the time of diagnosis, including the levels of cytokines, immunoglobulins, immune complexes, IgA glycosylation and NGAL in serum and urine. Among IgAV patients, 33 patients exhibited nephritis (IgAV-N) and 17 children were without nephritis (IgAV-woN). The serum level of galactose-deficient (Gd)-IgA1 (pmarkers, urinary IgA and IgM had the highest AUC (0.86 and 0.87 respectively, p<0.0001). This prospective cohort study furthers our understanding of the pathophysiology of IgAV. We identified biomarkers that are able to distinguish patients initially with or without nephritis. To conclude, serum Gd-IgA1 and urinary IgA, IgG, IgM, IL-6, IL-8, IL-10, and IgA-IgG and IgA-sCD89 complexes could identify IgAV pediatric patients with renal involvement at the time of diagnosis. PMID:29190714

  12. Vasculitis in patients with inflammatory bowel diseases: A study of 32 patients and systematic review of the literature.

    Science.gov (United States)

    Sy, Alice; Khalidi, Nader; Dehghan, Natasha; Barra, Lillian; Carette, Simon; Cuthbertson, David; Hoffman, Gary S; Koening, Curry L; Langford, Carol A; McAlear, Carol; Moreland, Larry; Monach, Paul A; Seo, Philip; Specks, Ulrich; Sreih, Antoine; Ytterberg, Steven R; Van Assche, Gert; Merkel, Peter A; Pagnoux, Christian

    2016-02-01

    Published small case series suggest that inflammatory bowel disease [IBD; Crohn's disease (CD) or ulcerative colitis (UC)] and vasculitis co-occur more frequently than would be expected by chance. To describe this association by an analysis of a large cohort of carefully studied patients and through a systematic literature review. Patients with both IBD and vasculitis enrolled in the Vasculitis Clinical Research Consortium (VCRC) Longitudinal Studies, followed in Canadian Vasculitis research network (CanVasc) centers and/or in the University of Toronto's IBD clinic were included in this case series. A systematic literature review of patients with IBD and vasculitis involved a PubMed search through February 2014. The main characteristics of patients with Takayasu arteritis (TAK) and IBD were compared to those in patients with TAK without IBD followed in the VCRC. The study identified 32 patients with IBD and vasculitis: 13 with large-vessel vasculitis [LVV; 12 with TAK, 1 with giant cell arteritis (GCA); 8 with CD, 5 with UC]; 8 with ANCA-associated vasculitis [AAV; 6 granulomatosis with polyangiitis (GPA), 2 with eosinophilic granulomatosis with polyangiitis (EGPA)]; 5 with isolated cutaneous vasculitis; and 6 with other vasculitides. Patients with LVV and AAV were mostly female (18/21). The diagnosis of IBD preceded that of vasculitis in 12/13 patients with LVV and 8/8 patients with AAV. The review of the literature identified 306 patients with IBD and vasculitis: 144 with LVV (133 TAK; 87 with IBD preceding LVV), 19 with AAV [14 GPA, 1 EGPA, 4 microscopic polyangiitis (MPA)], 66 with isolated cutaneous vasculitis, and 77 with other vasculitides. Patients with IBD and TAK were younger and had more frequent headaches, constitutional symptoms, or gastrointestinal symptoms compared to those patients in the VCRC who had TAK without IBD. These findings highlight the risk of vasculitis, especially TAK, in patients with IBD (both CD and UC). Copyright © 2015 Elsevier

  13. Antineutrophil cytoplasmic antibodes : A new class of autoantibodies in vasculitis and glomerulonephritis

    NARCIS (Netherlands)

    Cohen Tervaert, Jan Willem

    1990-01-01

    Vasculitis is een ontsteking van bloedvaten waardoor organen, die door deze bloedvaten van bloed worden voorzien, geleidelijk afsterven. Als meerdere bloedvaten ontstoken zijn en verschillende organen daardoor worden aangetast, spreekt men van systeemvasculitis. Indien een systeemvasculitis niet

  14. Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

    DEFF Research Database (Denmark)

    Morishita, Kimberly A; Moorthy, Lakshmi N; Lubieniecka, Joanna M

    2017-01-01

    OBJECTIVE: To characterize the early disease course in childhood-onset antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and the 12-month outcomes in children with AAV. METHODS: Eligible subjects were children entered into the Pediatric Vasculitis Initiative study who were...... (Pediatric Vasculitis Activity Score [PVAS] of 0) at 12 months with a corticosteroid dosage of ..., presence of damage at 12 months (measured by a modified Pediatric Vasculitis Damage Index [PVDI]; score 0 = no damage, score 1 = one damage item present), and relapse rates at 12 months. RESULTS: In total, 105 children with AAV were included in the study. The median age at diagnosis was 13.8 years...

  15. Cerebral angiography as a guide for therapy in isolated central nervous system vasculitis

    International Nuclear Information System (INIS)

    Stein, R.L.; Martino, C.R.; Weinert, D.M.; Hueftle, M.; Kammer, G.M.

    1987-01-01

    The authors present a case of isolated central nervous system vasculitis documented by cerebral arteriography in which remission, using a treatment regimen of prednisone and cyclophosphamide, was guided by serial arteriography during a 15-month period

  16. Plasma Exchange for Renal Vasculitis and Idiopathic Rapidly Progressive Glomerulonephritis: A Meta-analysis

    DEFF Research Database (Denmark)

    Walsh, Michael; Catapano, Fausta; Szpirt, Wladimir

    2010-01-01

    BACKGROUND:: Plasma exchange may be effective adjunctive treatment for renal vasculitis. We performed a systematic review and meta-analysis of randomized controlled trials of plasma exchange for renal vasculitis. STUDY DESIGN:: Systematic review and meta-analysis of articles identified from...... electronic databases, bibliographies, and studies identified by experts. Data were abstracted in parallel by 2 reviewers. SETTING & POPULATION:: Adults with idiopathic renal vasculitis or rapidly progressive glomerulonephritis. SELECTION CRITERIA FOR STUDIES:: Randomized controlled trials that compared...... standard care with standard care plus adjuvant plasma exchange in adult patients with either renal vasculitis or idiopathic rapidly progressive glomerulonephritis. INTERVENTION:: Adjuvant plasma exchange. OUTCOME:: Composite of end-stage renal disease or death. RESULTS:: We identified 9 trials including...

  17. [Uptodate in the management and treatment of ANCA-associated vasculitis].

    Science.gov (United States)

    Belaconi, Ionela Nicoleta; Toma, Claudia Lucia; Bogdan, Miron Alexandru

    2014-01-01

    The antineutrophil cytoplasm antibody (ANCA)-associated vasculitis are heterogeneous, multisystem, autoimmune diseases characterized by necrotizing small and medium vessel vasculitis and the association with ANCA. The diagnosis and management of these patients may be challenging due to the variability of clinical features, the possibility of life-threatening events (acute renal failure or pulmonary hemorrhage) and the relative rarity of these syndromes. ANCA-associated vasculitis include granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. The treatment requires significant immunosuppression and there are frequent treatment related side effects. Although the standard protocol with cytotoxic agents and glucocorticoids has dramatically improved patient outcome, its toxic profile remains a major problem. Recent progress in evidence base and consensus in understanding the pathogenic mechanism and the quantification of disease activity further improved patient's life. Special attention was paid in refining immunosuppressive treatment to minimize his toxicity. This review will focus on evidence based treatment of ANCA-associated vasculitis.

  18. A single-use site selection technique, using GIS, for aquaculture planning: choosing locations for mangrove oyster raft culture in Margarita Island, Venezuela.

    Science.gov (United States)

    Buitrago, Joaquín; Rada, Martín; Hernández, Hernando; Buitrago, Esperanza

    2005-05-01

    Oyster culture has a potential to generate income for coastal communities and to lessen pressure on natural overexploited populations. A project to transfer mangrove oyster Crassostrea rhizophorae raft culture technology to selected coastal communities in Margarita Island, Venezuela is being developed, and an optimum location selection technique was devised. To pick the variables or factors that determine site suitability, a bibliographic database was made, aspects of interest chosen, and the most comprehensive ones singled out, eliminating redundant ones. Twenty variables were grouped in criteria based on the way they influence the project. Variables were classified as intrinsic environmental, environmental extrinsic, logistic, and socioeconomic criteria. Thirty-five experts were asked to evaluate the factors and to score each according to their suitability weight. Logistic criterion received the highest values, followed by environmental extrinsic issues. A Geographic Information System using a base map compiled from 1:25,000 scale maps was developed. A thematic map for each factor was completed, dividing graphically the 3896-km2 study area into polygons of equal weight for each factor. The Multi-Criteria Evaluation (MCE) was used to combine the variables. Resultant vectors in thematic maps were added to obtain smaller polygons with the same value sum. Finally, MCE was used to generate a final output: the optimum sites for oyster aquaculture resulting from the added values of over 3000 polygons in the maps, for the 20 criteria. Higher scores were reached in 13 areas covering 4.1 km2, those places having the optimum conditions for oyster raft aquaculture in the region. Additional locations meeting 75% to 70% of the demanded criteria for a final suitable selection cover 137 sites encompassing 37.5 km2.

  19. Best single-slice location to measure visceral adipose tissue on paediatric CT scans and the relationship between anthropometric measurements, gender and VAT volume in children.

    Science.gov (United States)

    O'Connor, Michelle; Ryan, John; Foley, Shane

    2015-10-01

    Visceral adipose tissue (VAT) is a significant risk factor for obesity-related metabolic diseases. This study investigates (1) the best single CT slice location for predicting total abdominal VAT volume in paediatrics and (2) the relationship between waist circumference (WC), sagittal diameter (SD), gender and VAT volume. A random sample of 130 paediatric abdomen CT scans, stratified according to age and gender, was collected. Three readers measured VAT area at each intervertebral level between T12 and S1 using ImageJ analysis (National Institute of Health, Bethesda, MD) software by thresholding -190 to -30 HU and manually segmenting VAT. Single-slice VAT measurements were correlated with total VAT volume to identify the most representative slice. WC and SD were measured at L3-L4 and L4-L5 slices, respectively. Regression analysis was used to evaluate WC, SD and gender as VAT volume predictors. Interviewer and intraviewer reliability were excellent (intraclass correlation coefficient = 0.99). Although VAT measured at multiple slices correlated strongly with abdominal VAT, only one slice in females at L2-L3 and two slices in males at L1-L2 and L5-S1 were strongly correlated across all age groups. Linear regression analysis showed that WC was strongly correlated with VAT volume (beta = 0.970, p VAT measurements are highly reproducible. Measurements performed at L2-L3 in females and L1-L2 or L5-S1 in males were most representative of VAT. WC is indicative of VAT. VAT should be measured at L2-L3 in female children and at either L1-L2 or L5-S1 in males. WC is a strong indicator of VAT in children.

  20. Crohn’s Disease – The Pathogenesis of a Granulomatous Vasculitis: A Hypothesis

    OpenAIRE

    Andrew J Wakefield

    1995-01-01

    Dissatisfied with traditional approaches to studying the pathogenesis of Crohn’s disease, the author and colleagues proposed and developed the hypothesis that Crohn’s disease is a granulomatous vasculitis mediated by a persistent viral infection of the mesenteric microvascular endothelium. Employing a range of techniques, the mesenteric vascular anatomy of intestine affected by Crohn’s disease was studied and the presence of a widespread multifocal vasculitis was demonstrated. Based upon cert...

  1. Acute kidney injury with granulomatous interstitial nephritis and vasculitis revealing sarcoidosis

    OpenAIRE

    Amel Harzallah; Hayet Kaaroud; Karima Boubaker; Samia Barbouch; Rim Goucha; Fethi Ben Hamida; Taieb Ben Abdallah

    2017-01-01

    Sarcoidosis is an inflammatory disease that affects mostly the lungs and lymph glands. Renal involvement is rare and especially vasculitis. We report a case who presented an acute kidney failure and had sarcoidosis with vasculitis and nodular splenic involvement. A 35-year-old woman presenting a Lofgren syndrome was hospitalized for acute renal failure with cervical lymphadenopathy without other clinical findings. Laboratory data disclosed elevated angiotensin converting enzyme serum level. A...

  2. Vasculitis: análisis de 12.683 protocolos de autopsia. Estudio de 34 casos

    Directory of Open Access Journals (Sweden)

    César Augusto Gutiérrez

    1996-04-01

    Full Text Available We estudied 12.683 post-morten reports from Hospital San Juan de Dios permorfed between 1954 and 1990. We wanted to know mortality rates from connective tissue primary and secondary vasculitis, 16 ofwhich were systemic lupus erythematosus, one case of rheumatoid arthritis and one case of scleroderma. Skin, kidney and myocardial muscle were, The most affected organs only seventeen patients died directly from vasculitis. More studies have to be performed.

  3. Vasculitis secundaria a infección por Fasciola hepática Secondary vasculitis to infection by Fasciola hepatica

    Directory of Open Access Journals (Sweden)

    Germán Málaga

    2012-09-01

    Full Text Available Se presenta el caso de un paciente varón de 38 años, procedente de una zona endémica para fascioliasis hepática en el Perú. Fue hospitalizado por presentar un cuadro de cuatro semanas de evolución; con fiebre, mialgias intensas, lesiones eritematosas y dolorosas en las regiones de extensión de las extremidades. La electromiografía y la velocidad de conducción nerviosa mostraron una miopatía inflamatoria global. La biopsia de piel evidenció una vasculitis de tipo poliarteritis nodosa. En el proceso de evaluación previa a la terapia inmunosupresora, se hallaron huevos de Fasciola hepática en el examen coproparasitológico. El diagnóstico de fascioliasis se confirmó con fas2-ELISA: 0,46 (VN There is a case of a 38 year-old male patient coming from an area where hepatic fascioliasis is endemic in Peru. He was hospitalized because he showed 4 weeks of symptoms like fever, intense myalgias, erythematous and painful injuries on limb extensions. The electromyography and nerve conduction velocity showed a global inflammatory myopathy. A skin biopsy showed polyarteritis nodosa-type vasculitis. During the evaluation process prior to the immunosuppressive therapy, hepatic Fasciola eggs were found in the parasitological examination of stools. The fascioliasis diagnosis was confirmed by fas2-ELISA: 0.46 (VN <0.20. Clinical symptoms started to subside after treatment with ticlabendazol. Contact with the patient was maintained for a year and there was no evidence of disease recurrence, and he was asymptomatic

  4. Role of FDG-PET and PET/CT in the diagnosis and management of vasculitis

    Energy Technology Data Exchange (ETDEWEB)

    Zerizer, Imene; Tan, Kathryn; Khan, Sameer; Barwick, Tara [Department of Nuclear Medicine, Imperial College Healthcare, Hammersmith Hospital, Du Cane Road, London (United Kingdom); Marzola, Maria Cristina [Department of Nuclear Medicine, PET/CT Centre, Radiology and Medical Physics, ' Santa Maria della Misericordia' Hospital, Rovigo (Italy); Rubello, Domenico [Department of Nuclear Medicine, PET/CT Centre, Radiology and Medical Physics, ' Santa Maria della Misericordia' Hospital, Rovigo (Italy)], E-mail: domenico.rubello@libero.it; Al-Nahhas, Adil [Department of Nuclear Medicine, Imperial College Healthcare, Hammersmith Hospital, Du Cane Road, London (United Kingdom)

    2010-03-15

    Purpose: to investigate the role of FDG-PET and PET/CT in the evaluation of vasculitis. Materials and methods: a systematic revision of the papers published in PubMed/Medline until December 2009 was done. Results: FDG-PET and PET/CT have been proven to be valuable in the diagnosis of large-vessel vasculitis, especially giant cells arteritis with sensitivity values ranging 77% to 92%, and specificity values ranging 89% to 100%. In particular, FDG-PET/CT has demonstrated the potential to non-invasively diagnose the onset of the vasculitis earlier than traditional anatomical imaging techniques, thus enabling prompt treatment. False positive results mainly occur in the differential diagnosis between vasculitis and atherosclerotic vessels in elderly patients. Another area where FDG-PET/CT is gaining wider acceptance is in monitoring response to therapy; it can reliably detect the earliest changes of disease improvement post-therapy, and persistent activity is an indicator of non-responders to therapy. A few data have been reported about medium/small vessel vasculitis. Discussion: FDG-PET and PET/CT have proven utility: (a) in the initial diagnosis of patients suspected of having vasculitis particularly in those who present with non-specific symptoms; (b) in the identification of areas of increased FDG uptake in which a biopsy should be done for obtaining a diagnosis; (c) in evaluating the extent of the disease; (d) in assessing response to treatment.

  5. Advances in the use of biologic agents for the treatment of systemic vasculitis

    Science.gov (United States)

    Chung, Sharon A.; Seo, Philip

    2010-01-01

    Purpose of review Due to the well-known toxicities of cyclophosphamide, substantial interest exists in finding other therapies to treat primary systemic vasculitis. Biologic agents have been proposed as an alternative to cyclophosphamide for these disorders because of their recent success in treating other rheumatic diseases. This article reviews the current state-of-the-art with regards to the use of biologic agents as a treatment for systemic vasculitis. Recent findings The greatest amount of experience with these agents for the treatment of systemic vasculitis is with anti-tumor necrosis factor agents, pooled intravenous immunoglobulin, and anti-B cell therapies such as rituximab. Intravenous immunoglobulin is already a standard therapy for Kawasaki's disease, but should also be considered for the treatment of ANCA-associated vasculitis when standard therapies are either ineffective or contraindicated. Early experience with tumor necrosis factor inhibitors indicates that they may be effective for the treatment of Takayasu's arteritis, but their role in the treatment of other forms of vasculitis remains controversial. Early experience with rituximab for the treatment of several forms of vasculitis has been quite promising, but must be confirmed by ongoing randomized clinical trials. Summary Biologic agents represent the next evolution in treatment for the primary systemic vasculitides. Greater understanding of these diseases has allowed use to move further away from non-specific, highly toxic therapies towards a more directed approach. As our experience with these agents increases, they will likely form the keystone of treatment in the near future. PMID:19077713

  6. Role of FDG-PET and PET/CT in the diagnosis and management of vasculitis

    International Nuclear Information System (INIS)

    Zerizer, Imene; Tan, Kathryn; Khan, Sameer; Barwick, Tara; Marzola, Maria Cristina; Rubello, Domenico; Al-Nahhas, Adil

    2010-01-01

    Purpose: to investigate the role of FDG-PET and PET/CT in the evaluation of vasculitis. Materials and methods: a systematic revision of the papers published in PubMed/Medline until December 2009 was done. Results: FDG-PET and PET/CT have been proven to be valuable in the diagnosis of large-vessel vasculitis, especially giant cells arteritis with sensitivity values ranging 77% to 92%, and specificity values ranging 89% to 100%. In particular, FDG-PET/CT has demonstrated the potential to non-invasively diagnose the onset of the vasculitis earlier than traditional anatomical imaging techniques, thus enabling prompt treatment. False positive results mainly occur in the differential diagnosis between vasculitis and atherosclerotic vessels in elderly patients. Another area where FDG-PET/CT is gaining wider acceptance is in monitoring response to therapy; it can reliably detect the earliest changes of disease improvement post-therapy, and persistent activity is an indicator of non-responders to therapy. A few data have been reported about medium/small vessel vasculitis. Discussion: FDG-PET and PET/CT have proven utility: (a) in the initial diagnosis of patients suspected of having vasculitis particularly in those who present with non-specific symptoms; (b) in the identification of areas of increased FDG uptake in which a biopsy should be done for obtaining a diagnosis; (c) in evaluating the extent of the disease; (d) in assessing response to treatment.

  7. Comparison of disease activity measures for anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis

    Science.gov (United States)

    Merkel, PA; Cuthbertson, DD; Hellmich, B; Hoffman, GS; Jayne, DRW; Kallenberg, CGM; Krischer, JP; Luqmani, R; Mahr, AD; Matteson, EL; Specks, U; Stone, JH

    2011-01-01

    Aim Currently, several different instruments are used to measure disease activity and extent in clinical trials of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, leading to division among investigative groups and difficulty comparing study results. An exercise comparing six different vasculitis instruments was performed. Methods A total of 10 experienced vasculitis investigators from 5 countries scored 20 cases in the literature of Wegener granulomatosis or microscopic polyangiitis using 6 disease assessment tools: the Birmingham Vasculitis Activity Score (BVAS), The BVAS for Wegener granulomatosis (BVAS/WG), BVAS 2003, a Physician Global Assessment (PGA), the Disease Extent Index (DEI) and the Five Factor Score (FFS). Five cases were rescored by all raters. Results Reliability of the measures was extremely high (intraclass correlations for the six measures all=0.98). Within each instrument, there were no significant differences or outliers among the scores from the 10 investigators. Test/retest reliability was high for each measure: range=0.77 to 0.95. The scores of the five acute activity measures correlated extremely well with one another. Conclusions Currently available tools for measuring disease extent and activity in ANCA-associated vasculitis are highly correlated and reliable. These results provide investigators with confidence to compare different clinical trial data and helps form common ground as international research groups develop new, improved and universally accepted vasculitis disease assessment instruments. PMID:18664546

  8. Membranoproliferative glomerulonephritis presenting as arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis: a case report.

    Science.gov (United States)

    Park, Chuiyoung; Choi, Seung Won; Kim, Misung; Park, Jongha; Lee, Jong Soo; Chung, Hyun Chul

    2014-10-22

    Hypocomplementemic urticarial vasculitis syndrome is a rare disorder characterized by chronic urticarial vasculitis, arthralgia, arthritis, and hypocomplementemia. Previously, only six patients with concomitant hypocomplementemic urticarial vasculitis syndrome, Jaccoud's arthropathy, and valvular heart disease have been reported. A 30-year-old Korean man presented with hypocomplementemic urticarial vasculitis syndrome. In addition to urticarial cutaneous lesions, he experienced polyarthralgia and arthritis that resulted in progressive deformity of the joints of both hands, cardiac valvulopathy with mitral, tricuspid, and aortic regurgitation, and intermittent neck swelling with laryngeal edema. He also developed nephritis with azotemia. His renal biopsy results revealed membranoproliferative glomerulonephritis, type I. He showed a partial response to a combination therapy of steroid, cyclophosphamide, and mycophenolate mofetil. We describe, to the best of our knowledge, the first case of glomerulonephritis presenting a arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis syndrome. A combination of corticosteroids, cyclophosphamide, and mycophenolate mofetil appear to be a safe and effective treatment for nephropathy, however are less effective for cutaneous vasculitis, cardiac valvulopathy, and arthropathy.

  9. Cardiac involvement in primary systemic vasculitis and potential drug therapies to reduce cardiovascular risk.

    Science.gov (United States)

    Misra, Durga Prasanna; Shenoy, Sajjan N

    2017-01-01

    Cardiac involvement is common in primary systemic vasculitides and may be due to direct effect of the disease on the heart or due to therapy. We shall review involvement of the heart in the various forms of primary systemic vasculitis. Among anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), eosinophilic granulomatosis with polyangiitis most commonly involves the heart. Involvement of the heart confers poorer prognosis in AAV, which is also complicated by increased risk of cardiovascular events. Kawasaki's disease (KD) is the most common form of medium-vessel vasculitis to affect the heart, with coronary artery aneurysms being the most common manifestation. These predispose patients with KD to develop premature ischemic heart disease. Takayasu's arteritis is the most common large-vessel vasculitis to involve the heart and can result in aortic incompetence, myocarditis, or coronary heart disease. Involvement of the heart in Behcet's disease is usually in the form of intracardiac mass lesions, thrombosis, or endomyocardial fibrosis. Drugs used in the treatment of systemic vasculitis influence the risk of developing cardiovascular events. Corticosteroid therapy has been shown to increase the risk of myocardial infarction, whereas methotrexate, azathioprine, mycophenolate mofetil, rituximab, and anti-tumor necrosis alpha agents favorably modulate the risk of cardiovascular events, predominantly by dampening systemic inflammation. Awareness of cardiac involvement in vasculitis and accelerated cardiovascular risk in these patients should help clinicians to maximize the modulation of modifiable risk factors for heart disease in these individuals.

  10. Circulating ADAM17 Level Reflects Disease Activity in Proteinase-3 ANCA-Associated Vasculitis

    Science.gov (United States)

    Bertram, Anna; Lovric, Svjetlana; Engel, Alissa; Beese, Michaela; Wyss, Kristin; Hertel, Barbara; Park, Joon-Keun; Becker, Jan U.; Kegel, Johanna; Haller, Hermann; Haubitz, Marion

    2015-01-01

    ANCA-associated vasculitides are characterized by inflammatory destruction of small vessels accompanied by enhanced cleavage of membrane-bound proteins. One of the main proteases responsible for ectodomain shedding is disintegrin and metalloproteinase domain-containing protein 17 (ADAM17). Given its potential role in aggravating vascular dysfunction, we examined the role of ADAM17 in active proteinase-3 (PR3)-positive ANCA-associated vasculitis (AAV). ADAM17 concentration was significantly increased in plasma samples from patients with active PR3-AAV compared with samples from patients in remission or from other controls with renal nonvascular diseases. Comparably, plasma levels of the ADAM17 substrate syndecan-1 were significantly enhanced in active AAV. We also observed that plasma-derived ADAM17 retained its specific proteolytic activity and was partly located on extracellular microparticles. Transcript levels of ADAM17 were increased in blood samples of patients with active AAV, but those of ADAM10 or tissue inhibitor of metalloproteinases 3, which inhibits ADAMs, were not. We also performed a microRNA (miR) screen and identified miR-634 as significantly upregulated in blood samples from patients with active AAV. In vitro, miR-634 mimics induced a proinflammatory phenotype in monocyte-derived macrophages, with enhanced expression and release of ADAM17 and IL-6. These data suggest that ADAM17 has a prominent role in AAV and might account for the vascular complications associated with this disease. PMID:25788529

  11. Clasificación de las vasculitis cutáneas : vasculitis cutáneas por fármacos

    OpenAIRE

    Ortiz Sanjuán, Francisco Miguel

    2016-01-01

    RESUMEN: La sistematización de los diferentes tipos de vasculitis continúa siendo a día de hoy un tema complejo. La adecuada caracterización de los diferentes tipos de vasculitis cutánea (VC) podría ayudar a profundizar en el conocimiento de su etiología, clínica, clasificación, tratamiento y pronóstico. Por otra parte, los fármacos se ven frecuentemente implicados como posible agente etiológico de una VC, si bien su mecanismo etiopatogénico no está claramente definido. Este trabajo b...

  12. Cardiovascular magnetic resonance for evaluation of heart involvement in ANCA-associated vasculitis. A luxury or a valuable diagnostic tool?

    Science.gov (United States)

    Mavrogeni, Sophie; Markousis-Mavrogenis, George; Kolovou, Genovefa

    2014-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-related vasculitis is a systemic small-vessel vasculitis, including 3 clinical syndromes: granulomatosis with polyangiitis, known as Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and the Churg-Strauss syndrome (CSS). ANCA-related vasculitis usually presents with severe kidney or pulmonary disease, has a mortality of 28% at 5 years, and also contributes to increased morbidity in vasculitis patients. Cardiac involvement in this entity may have different forms, including coronary vessels, pericarditis, myocarditis, endocarditis, myocardial infarction and subendocardial vasculitis that can contribute to reduced life expectancy. Cardiovascular magnetic resonance using oedema and fibrosis imaging can early reveal, noninvasively and without radiation, heart involvement during vasculitis, undetected by other imaging techniques and guide further risk stratification and treatment of these patients.

  13. Measurement of Liver Stiffness Using Shear Wave Elastography in a Rat Model: Factors Impacting Stiffness Measurement with Multiple- and Single-Tracking-Location Techniques.

    Science.gov (United States)

    Langdon, Jonathan H; Elegbe, Etana; Gonzalez, Raul S; Osapoetra, Laurentius; Ford, Tristan; McAleavey, Stephen A

    2017-11-01

    The clinical use of elastography for monitoring fibrosis progression is challenged by the subtle changes in liver stiffness associated with early-stage fibrosis and the comparatively large variance in stiffness estimates provided by elastography. Single-tracking-location (STL) shear wave elasticity imaging (SWEI) is an ultrasound elastography technique previously found to provide improved estimate precision compared with multiple-tracking-location (MTL) SWEI. Because of the improved precision, it is reasonable to expect that STL-SWEI would provide improved ability to differentiate liver fibrosis stage compared with MTL-SWEI. However, this expectation has not been previously challenged rigorously. In this work, the performance of STL- and MTL-SWEI in the setting of a rat model of liver fibrosis is characterized, and the advantages of STL-SWEI in staging fibrosis are explored. The purpose of this study was to determine what advantages, if any, arise from using STL-SWEI instead of MTL-SWEI in the characterization of fibrotic liver. Thus, the ability of STL-SWEI to differentiate livers at various METAVIR fibrosis scores, for ex vivo postmortem measurements, is explored. In addition, we examined the effect of the common confounding factor of fluid versus solid boundary conditions in SWEI experiments. Sprague-Dawley rats were treated with carbon tetrachloride over several weeks to produce liver disease of varying severity. STL and MTL stiffness measurements were performed ex vivo and compared with the METAVIR scores from histological analysis and the duration of treatment. A strong association was observed between liver stiffness and weeks of treatment with the liver toxin carbon tetrachloride. Direct comparison of STL- and MTL-SWEI measurements revealed no significant difference in ability to differentiate fibrosis stages based on SWEI mean values. However, image interquartile range was greatly improved in the case of STL-SWEI, compared with MTL-SWEI, at small beam

  14. Analysis of human rotaviruses from a single location over an 18-year time span suggests that protein coadaption influences gene constellations.

    Science.gov (United States)

    Zhang, Shu; McDonald, Paul W; Thompson, Travis A; Dennis, Allison F; Akopov, Asmik; Kirkness, Ewen F; Patton, John T; McDonald, Sarah M

    2014-09-01

    Rotaviruses (RVs) are 11-segmented, double-stranded RNA viruses that cause severe gastroenteritis in children. In addition to an error-prone genome replication mechanism, RVs can increase their genetic diversity by reassorting genes during host coinfection. Such exchanges allow RVs to acquire advantageous genes and adapt in the face of selective pressures. However, reassortment may also impose fitness costs if it unlinks genes/proteins that have accumulated compensatory, coadaptive mutations and that operate best when kept together. To better understand human RV evolutionary dynamics, we analyzed the genome sequences of 135 strains (genotype G1/G3/G4-P[8]-I1-C1-R1-A1-N1-T1-E1-H1) that were collected at a single location in Washington, DC, during the years 1974 to 1991. Intragenotypic phylogenetic trees were constructed for each viral gene using the nucleotide sequences, thereby defining novel allele level gene constellations (GCs) and illuminating putative reassortment events. The results showed that RVs with distinct GCs cocirculated during the vast majority of the collection years and that some of these GCs persisted in the community unchanged by reassortment. To investigate the influence of protein coadaptation on GC maintenance, we performed a mutual information-based analysis of the concatenated amino acid sequences and identified an extensive covariance network. Unexpectedly, amino acid covariation was highest between VP4 and VP2, which are structural components of the RV virion that are not thought to directly interact. These results suggest that GCs may be influenced by the selective constraints placed on functionally coadapted, albeit noninteracting, viral proteins. This work raises important questions about mutation-reassortment interplay and its impact on human RV evolution. Rotaviruses are devastating human pathogens that cause severe diarrhea and kill >450,000 children each year. The virus can evolve by accumulating mutations and by acquiring new genes

  15. Neutrophil Extracellular Traps in ANCA-Associated Vasculitis.

    Science.gov (United States)

    Söderberg, Daniel; Segelmark, Mårten

    2016-01-01

    A group of pauci-immune vasculitides, characterized by neutrophil-rich necrotizing inflammation of small vessels and the presence of antineutrophil cytoplasmic antibodies (ANCAs), is referred to as ANCA-associated vasculitis (AAV). ANCAs against proteinase 3 (PR3) (PR3-ANCA) or myeloperoxidase (MPO) (MPO-ANCA) are found in over 90% of patients with active disease, and these ANCAs are implicated in the pathogenesis of AAV. Dying neutrophils surrounding the walls of small vessels are a histological hallmark of AAV. Traditionally, it has been assumed that these neutrophils die by necrosis, but neutrophil extracellular traps (NETs) have recently been visualized at the sites of vasculitic lesions. AAV patients also possess elevated levels of NETs in the circulation. ANCAs are capable of inducing NETosis in neutrophils, and their potential to do so has been shown to be affinity dependent and to correlate with disease activity. Neutrophils from AAV patients are also more prone to release NETs spontaneously than neutrophils from healthy blood donors. NETs contain proinflammatory proteins and are thought to contribute to vessel inflammation directly by damaging endothelial cells and by activating the complement system and indirectly by acting as a link between the innate and adaptive immune system through the generation of PR3- and MPO-ANCA. Injection of NET-loaded myeloid dendritic cells into mice results in circulating PR3- and MPO-ANCA and the development of AAV-like disease. NETs have also been shown to be essential in a rodent model of drug-induced vasculitis. NETs induced by propylthiouracil could not be degraded by DNaseI, implying that disordered NETs might be important for the generation of ANCAs. NET degradation was also highlighted in another study showing that AAV patients have reduced DNaseI activity resulting in less NET degradation. With this in mind, it might be that prolonged exposure to proteins in the NETs due to the overproduction of NETs and/or reduced

  16. Meta-Analysis of Associations Between Interleukin-10 Polymorphisms and Susceptibility to Vasculitis.

    Science.gov (United States)

    Jung, Jae Hyun; Song, Gwan Gyu; Lee, Young Ho

    2015-01-01

    This study determined whether interleukin-10 (IL-10) polymorphisms are associated with susceptibility to vasculitis. A meta-analysis was conducted of the associations between the IL-10 -1082 G/A, -819 C/T, and -592 C/A polymorphisms and the haplotype of the IL-10-1082 G/A, -819 C/T, -592 C/A polymorphisms and vasculitis. A total of 21 comparative studies involving 4121 patients and 5504 controls were considered in the meta-analysis. Meta-analysis revealed no association between the IL-10-1082 G allele and vasculitis in all study subjects (OR = 0.927, 95% CI = 0.780-1.102, p = 0.389). However, disease-specific meta-analysis showed an association between Wegener's granulomatosis (WG) and the IL-10-1082 G allele (OR = 0.729, 95% CI = 0.547-0.971, p = 0.031). Meta-analysis revealed an association between vasculitis and the IL-10-819 C allele (OR = 0.804, 95% CI = 0.706-0.916, p = 0.001) in all study subjects and Behcet's disease (BD) (OR = 0.724, 95% CI = 0.679-0.781, p vasculitis in all study subjects (OR = 0.805, 95% CI = 0.619-0.938, p = 0.005) and BD (OR = 0.718, 95% CI = 0.661-0.781, p vasculitis in Europeans (OR = 1.239, 95% CI = 1.105-1.513, p = 0.035). This meta-analysis showed that IL-10 polymorphisms are associated with vasculitis susceptibility, especially in WG and BD.

  17. New Features of Disease after Diagnosis in Six Forms of Systemic Vasculitis

    Science.gov (United States)

    Grayson, Peter C.; Cuthbertson, David; Carette, Simon; Hoffman, Gary S.; Khalidi, Nader A.; Koening, Curry L.; Langford, Carol A.; Maksimowicz-McKinnon, Kathleen; Monach, Paul A.; Seo, Philip; Specks, Ulrich; Ytterberg, Steven R.; Merkel, Peter A.

    2015-01-01

    Objective To quantify the occurrence of features of vasculitis that initially present after diagnosis in 6 types of primary vasculitis. Methods Standardized collection of data on 95 disease manifestations in 6 vasculitides, including granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA), polyarteritis nodosa (PAN), giant cell arteritis (GCA), and Takayasu's arteritis (TAK), was performed within a set of multicenter longitudinal, observational cohorts. For each form of vasculitis, the frequency of disease-specific manifestations at diagnosis was compared to the cumulative frequency of each manifestation. The percentage of patients who initially developed “severe” manifestations after diagnosis, defined as organ- or life-threatening in the small and medium vessel vasculitides (GPA, MPA, EGPA, PAN) and as ischemic/vascular in the large vessel vasculitides (GCA, TAK), was reported. Results Out of 838 patients with vasculitis, 490 (59%) experienced ≥ 1 new disease manifestation after diagnosis. On average, patients with vasculitis experienced 1.3 new manifestations after diagnosis (GPA - 1.9, MPA - 1.2, EGPA - 1.5, PAN - 1.2, GCA - 0.7, TAK - 1.0). New severe manifestations occurred after diagnosis in 224 (27%) out of 838 patients (GPA - 26%, MPA - 19%, EGPA - 21%, PAN - 23%, GCA - 24%, and TAK - 44%). Timing of onset of new manifestations was not significantly associated with disease duration. Conclusion A majority of patients with vasculitis develop new disease features after diagnosis, including a substantial number of new, severe manifestations. Ongoing assessment of patients with established vasculitis should remain broad in scope. PMID:23908447

  18. Evaluation of the FIDIS vasculitis multiplex immunoassay for diagnosis and follow-up of ANCA-associated vasculitis and Goodpasture's disease

    NARCIS (Netherlands)

    Damoiseaux, J.; Vaessen, M.; Knapen, Y.; Csernok, E.; Stegeman, C. A.; Van Paassen, P.; Tervaert, J. W. Cohen; Gershwin, ME; Shoenfeld, Y

    2007-01-01

    We have evaluated a new-multiplex immunoassay (FIDIS Vasculitis) for simultaneous detection and quantification of anti-MPO, -PR3, and -glomerular basement membrane (GBM) antibodies in diagnosis and follow-up of ANCA-associated vasculitides (AAV) and Goodpasture's disease. ANCA were determined in

  19. Clinical features and etiology of retinal vasculitis in Northern Thailand

    Directory of Open Access Journals (Sweden)

    Supanut Apinyawasisuk

    2013-01-01

    Full Text Available Purpose: To report on the clinical features and etiology of patients with retinal vasculitis (RV. Materials and Methods: We reviewed medical records of 47 patients (75 affected eyes diagnosed with RV. Clinical presentations, ocular complications, associated systemic diseases, and treatment regimens were registered. Results: Etiology of RV included infectious causes in 10/47, (21% while an association with systemic and/or ocular non-infectious disorders was noted in 22/47 (47%. Eales′ disease and Behcet′s disease represented the most common clinical entities in non-infectious group while tuberculosis-associated RV was diagnosed in 6/10 (60% among those with infectious disorders. RV was bilateral in 28/47 (60% patients. Retinal veins were most commonly affected (72%, 34/47. Involvement of arteries was present in 12/47 (25% and was associated with viral infections and Behcet′s disease. Ocular complications developed in 60/75 (80% eyes. The most common complications were elevated intraocular pressure and/or glaucoma (33/75, 44%. Retinal detachment, vitreous hemorrhage, and cystoid macular edema developed in similar percentages (15%. Conclusions: RV in Thailand manifested mostly in male patients, was typically bilateral and involved mostly veins. Involvement of arteries was observed in patients with viral infections and Behcet′s disease. Tuberculosis was the most common infectious cause.

  20. [18F]FDG-PET in large vessel vasculitis

    International Nuclear Information System (INIS)

    Hauser, A.S.D.; Walter, M.A.

    2007-01-01

    [ 18 F]FDG-PET is a non-invasive metabolic imaging modality based on the regional distribution of fluorine-18-fluorodeoxyglucose that is highly effective in assessing the activity and the extent of giant cell arteritis and Takayasu's arteritis. It has shown to identify more affected vascular regions than morphologic imaging with Magnetic Resonance Imaging in both diseases. A visual grading of vascular [ 18 F]FDG-uptake helps to discriminate arteritis from atherosclerosis und therefore provides high specificity. High sensitivity is reached by scanning during the active inflammatory phase. [ 18 F]FDG-PET has the potential to develop into a valuable tool in the diagnostic work-up of giant cell arteritis and Takayasu's arteritis, respectively, and might become a first-line investigation technique. Therefore consensus regarding the most favorable imaging procedure as well as further clinical evidence is needed. The purpose of this review is to summarize current information on the present clinical data and to assist nuclear medicine practitioners in recommending, performing and interpreting the results of [ 18 F]FDG-PET in patients with suspected large vessel vasculitis. (orig.)

  1. Leukocytoclastic vasculitis: A window to systemic Churg Strauss syndrome

    Directory of Open Access Journals (Sweden)

    Sudhir V Medhekar

    2012-01-01

    Full Text Available A twenty year old male presented with purpuric lesions with chronic painful ulcers over the lower extremities and a recurrent pruritic rash on the trunk for 10 years. He was diagnosed as idiopathic leukocytoclastic vasculitis (LCV after investigations failed to reveal a systemic association. He was treated with immunosuppressants at each visit with partial remission. In 2004, he was diagnosed with bronchial asthma and allergic rhinitis. In his recent admission, he showed necrotic ulcers on legs and extensive shiny, truncal micropapules. Examination revealed maxillary sinus tenderness and loss of sensation on the medial aspect of the left lower limb. Biopsy of ulcer and the micropapules showed the presence of extravascular eosinophils, while hematological investigations showed peripheral eosinophilia of 18%, raised serum Immunoglobulin E (IgE, Anti nuclear antibody (ANA positivity and negative antineutrophil cytoplasmic antibody (ANCA. Radiography confirmed maxillary sinusitis, nerve conduction studies revealed mononeuritis of the anterior tibial nerve and pulmonary function tests (PFT were normal. Clinical examination and investigations pointed towards the diagnosis of Churg-Strauss syndrome (CSS. This report highlights the development of full-blown CSS over a period of 12 years in a patient initially diagnosed as idiopathic LCV, emphasizing the need for regular follow-up of resistant and recurrent cases of LCV.

  2. Primary vasculitis of the cns restricted to the brainstem

    Directory of Open Access Journals (Sweden)

    Aljoša Polenčič

    2005-07-01

    Full Text Available Background: Primary or isolated vasculitis of the central nervous system (PVCNS is a rare disease with an atypical clinical picture. It involves both small and mediumsized leptomeningeal, cortical and subcortical vessels. Etiology and pathogenesis are unknown. The first clinical sign is usually a headache and later in the disease virtually every neurological symptom and sign has been reported. The golden standard for diagnosis is a biopsy. High doses of corticosteroids and cyclophosphamide have been recommended for treatment.Patient and methods: A case of a 61 years old woman with diabetes and hypertension is reported. She sudenly developed signs of focal ischemic brain stem lesion and had lesions visualised on MR in brain stem and both hemisferes. After temporary remission she developed similar signs five months later. She had died before the diagnostic procedures were performed. The diagnosis of PVCNS isolated to the brain stem was made post mortem.Conclusions: The diagnostic and therapeutic challenges of PVCNS are big even for an experienced clinician. Diagnosis depends on a combination of clinical, imaging and histological features. Often the diagnosis is confirmed post mortem.

  3. Acute hamstring injury in football players: Association between anatomical location and extent of injury-A large single-center MRI report.

    Science.gov (United States)

    Crema, Michel D; Guermazi, Ali; Tol, Johannes L; Niu, Jingbo; Hamilton, Bruce; Roemer, Frank W

    2016-04-01

    To describe in detail the anatomic distribution of acute hamstring injuries in football players, and to assess the relationship between location and extent of edema and tears, all based on findings from MRI. Retrospective observational study. We included 275 consecutive male football players who had sustained acute hamstring injuries and had positive findings on MRI. For each subject, lesions were recorded at specific locations of the hamstring muscles, which were divided into proximal or distal: free tendon, myotendinous junction, muscle belly, and myofascial junction locations. For each lesion, we assessed the largest cross-sectional area of edema and/or tears. We calculated the prevalence of injuries by location. The relationships between locations and extent of edema and tears were assessed using a one-sample t-test, with significance set at pinjuries were most common in the myotendinous junction and in proximal locations. The proximal myotendinous junction was associated with a greater extent of edema in the LHBF and semitendinosus (ST) muscles (p<0.05). Proximal locations in the LHBF had larger edema than distal locations (p<0.05). Distal locations in the ST muscle had larger tears than proximal locations (p<0.05). The proximal myotendinous junction (LHBF and ST muscles) and proximal locations (LHBF muscle) are more commonly affected and are associated with a greater extent of edema in acute hamstring muscle injury. Distal locations (ST muscle), however, seem to be more commonly associated with larger tears. Copyright © 2015 Sports Medicine Australia. Published by Elsevier Ltd. All rights reserved.

  4. Associations between interleukin-1 polymorphisms and susceptibility to vasculitis: a meta-analysis.

    Science.gov (United States)

    Song, G G; Kim, J-H; Lee, Y H

    2016-05-01

    The objective of this study was to determine whether interleukin-1 (IL-1) polymorphisms are associated with susceptibility to vasculitis. A meta-analysis was conducted to investigate possible associations between IL-1A, IL-1B, and IL-1 receptor antagonist (IL1RN) polymorphisms and vasculitis. A total of 17 studies involving 1384 vasculitis cases [Behçet's disease (BD), IgA vasculitis, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), Kawasaki disease (KD), giant cell arteritis, and Takayasu's arteritis] and 2710 controls were included in the meta-analysis. This analysis showed an association between BD and the TT + TC genotypes of the IL-1A-889 C/T polymorphism in the entire study population [odds ratio (OR) = 0.623, 95 % CI = 0.395-0.981, p = 0.045), and a trend toward an association in a Turkish population (OR = 0.578, 95 % CI = 0.331-1.010, p = 0.054). A meta-analysis of the IL1RN polymorphism revealed no association with vasculitis in all study subjects (OR for IL1RN*2 = 0.904, 95 % CI = 0.626-1.304, p = 0.588). However, stratification by ethnicity revealed a significant association between the IL1RN*2 allele and vasculitis including AAV, BD, KD in Asians (OR = 2.393, 95 % CI = 1.429-4.006, p = 0.001), but not in Caucasian and Turkish populations (OR = 0.776, 95 % CI = 0.487-1.238, p = 0.288; OR = 0.914, 95 % CI = 0.667-1.252, p = 0.576, respectively). No association was found between vasculitis and the IL-1B-511 C/T polymorphism, or the IL-1B+3953 C/T polymorphism. This meta-analysis suggests that the IL-1A-889 C/T polymorphism is associated with susceptibility to BD, and that the IL1RN*2 allele is associated with susceptibility to vasculitis including AAV, BD, and KD in Asians.

  5. A Case Of Primary Central Nervous System Vasculitis Who Presented With Status Epilepticus

    Directory of Open Access Journals (Sweden)

    Sırma Geyik

    2014-12-01

    Full Text Available Primary central nervous system vasculitis (PCNV is limited with central nervous system and rare vasculitis that mostly seen in middle-aged men. PCNV vasculitis is usually presented that headache, dementia, stroke and multifocal common neurological symptoms. PCNV especially involves small medium-sized leptomeningeal and cortical arteries. 43 years old male patient who have been progressive forgetfulness and headache for 3 years. He applied with recurrent that before starting right focal and than sprawling whole body which generalized tonic-clonic seizures to us. During management that he was transfered to the intensive care unit due to status epilepticus (SE. Later than we found right hemiparesis, motor aphasia and right babinski positivity in neurologic examination. Diffusion restriction was revealed in left MCA territory in diffusion magnetic resonance imaging(MRI. EEG showed two types abnormality that a slow background ritm and epileptiform activity. Biochemistry of blood, complete blood count, blood sedimentation rate, CRP and markers of vasculitis were found in the normal range. Cerebral anjiography revealed that irregularities in the distal vascular areas and fusiform aneurysm at the top of basilar artery. He was consulted with rheumatology and diagnosed central nervous system vasculitis with the existing findings. Biopsy couldn't be taken from the brain to verify the diagnosis. Finally, we applied treatment that pulse steroid and cyclophosphamide to patient. This case has been presented due to emphasize that PCNV rarely may play a role in the etiology of recurrent stroke and status epilepticus.

  6. Leukocytoclastic vasculitis complicating cisplatin + radiation treatment for laryngeal cancer: a case report.

    Science.gov (United States)

    Quintanilha, Júlia Coelho França; Visacri, Marília Berlofa; Amaral, Laís Sampaio; Lima, Carmen Silvia Passos; Cintra, Maria Letícia; Moriel, Patricia

    2017-12-06

    Leukocytoclastic vasculitis is typically mediated by deposition of immune complexes and is related to many causes, including medication. To the best of our knowledge, leukocytoclastic vasculitis related to cisplatin has not yet been described in the scientific literature. We report a rare case of leukocytoclastic vasculitis after the first cycle of high-dose cisplatin chemotherapy in a patient with larynx carcinoma. A 48-year-old Caucasian man with larynx carcinoma received a high-dose of cisplatin monochemotherapy (100 mg/m 2 every 21 days), along with 70 Gy of radiotherapy divided into 35 sessions, as a therapeutic schedule. Twelve days after the first chemotherapy administration and after 8 sessions of radiotherapy (total of 16 Gy), the patient presented with acute onset of palpable purpura in the lower limbs. The patient was hospitalized for 10 days, and during this period, he underwent several examinations to rule out infectious, autoimmune, and neoplastic disorders. A skin biopsy showed leukocytoclastic vasculitis with a positive pattern for IgM and C3, as detected through direct immunofluorescence. Twenty-five days after cisplatin administration, the chemotherapy regimen was changed to carboplatin AUC 5, and the episodes of purpura ceased, reinforcing the hypothesis of an adverse reaction to cisplatin. Cisplatin can induce leukocytoclastic vasculitis and clinicians should be aware of this potential effect for better case management and diagnosis.

  7. An Interferon-Induced Digital Vasculitis-Like Syndrome: A Case Report.

    Science.gov (United States)

    Hamidi, Oksana; Reiser, Jochen; Hasler, Scott

    2016-01-01

    This report describes a patient with chronic hepatitis C undergoing therapy with interferon (IFN) alpha who developed bilateral ischemia of his fingers. We present a 43-year-old man with a failed renal transplant and chronic hepatitis C. He was treated with 6 months of IFN therapy with good reduction of his viral load. He presented with 2 days of pain and swelling in the second digits of both hands. Workup for extrahepatic manifestations of hepatitis C was initiated including assessment for vasculitis because of cryoglobulin- and noncryoglobulin-related causes. Extensive assessment with invasive and noninvasive vascular testing was performed. His workup for vasculitis did not reveal any specific reasons for the ischemic changes. Angiography of his fingers showed mild stenotic changes but no evidence of systemic vasculitis. IFN therapy was stopped and over several weeks his symptoms resolved. The ischemic changes were attributed to IFN therapy. The patient in this report is unique because although IFN has been historically reported to cause a variety of vascular syndromes, the reported experience in hepatitis C patients is small. In addition, the likelihood of encountering vasculitis and vasculitis-like syndromes in patients with hepatitis C is significant, and the increasing use of IFN in this population makes drug-induced vascular changes an essential consideration in this subset of patients.

  8. Use of positron emission tomography (PET) for the diagnosis of large-vessel vasculitis.

    Science.gov (United States)

    Loricera, J; Blanco, R; Hernández, J L; Martínez-Rodríguez, I; Carril, J M; Lavado, C; Jiménez, M; González-Vela, C; González-Gay, M Á

    2015-01-01

    The term vasculitis encompasses a heterogeneous group of diseases that share the presence of inflammatory infiltrates in the vascular wall. The diagnosis of large-vessel vasculitis is often a challenge because the presenting clinical features are nonspecific in many cases and they are often shared by different types of autoimmune and inflammatory diseases including other systemic vasculitides. Moreover, the pathogenesis of large-vessel vasculitis is not fully understood. Nevertheless, the advent of new imaging techniques has constituted a major breakthrough to establish an early diagnosis and a promising tool to monitor the follow-up of patients with largevessel vasculitis. This is the case of the molecular imaging with the combination of positron emission tomography with computed tomography (PET/CT) using different radiotracers, especially the (18)F-fluordeoxyglucose ((18)F-FDG). In this review we have focused on the contribution of (18)F-FDG PET in the diagnosis of large-vessel vasculitis. Copyright © 2015 Elsevier España, S.L.U. and SEMNIM. All rights reserved.

  9. Histopathological Evaluation of Horse Serum-induced Immune Complex Vasculitis in Swine: Implication to Coronary Artery Lesions in Kawasaki Disease

    Directory of Open Access Journals (Sweden)

    Saji Philip

    2014-08-01

    Conclusion: HS can induce IC vasculitis in swine. The rashes and 2D echocardiographic and histopathological studies of the acute to healing phases showed close similarities with KD, and it is concluded that swine may serve as a unique experimental model for IC vasculitis and for various therapeutic trials.

  10. Progress Towards a Core Set of Outcome Measures in Small-vessel Vasculitis. Report from OMERACT 9

    Science.gov (United States)

    MERKEL, PETER A.; HERLYN, KAREN; MAHR, ALFRED D.; NEOGI, TUHINA; SEO, PHILIP; WALSH, MICHAEL; BOERS, MAARTEN; LUQMANI, RAASHID

    2011-01-01

    The past decade has seen a substantial increase in the number and quality of clinical trials of new therapies for vasculitis, including randomized, controlled, multicenter trials that have successfully incorporated measures of disease activity and toxicity. However, because current treatment regimens for severe disease effectively induce initial remission and reduce mortality, future trials will focus on any of several goals including: (a) treatment of mild—moderate disease; (b) prevention of chronic damage; (c) reduction in treatment toxicity; or (d) more subtle differences in remission induction or maintenance. Thus, new trials will require outcome measure instruments that are more precise and are better able to detect effective treatments for different disease states and measure chronic manifestations of disease. The OMERACT Vasculitis Working Group comprises international clinical investigators with expertise in vasculitis who, since 2002, have worked collaboratively to advance the refinement of outcome measures in vasculitis, create new measures to address domains of illness not covered by current research approaches, and harmonize outcome assessment in vasculitis. The focus of the OMERACT group to date has been on outcome measures in small-vessel vasculitis with an overall goal of creating a core set of outcome measures for vasculitis, each of which fulfills the OMERACT filter of truth, discrimination, feasibility, and identifying additional domains requiring further research. This process has been informed by several ongoing projects providing data on outcomes of disease activity, disease-related damage, multidimensional health-related quality of life, and patient-reported ratings of the burden of vasculitis. PMID:19820226

  11. Novel CFI mutation in a patient with leukocytoclastic vasculitis may redefine the clinical spectrum of Complement Factor I deficiency

    DEFF Research Database (Denmark)

    Bay, Jakob Thaning; Katzenstein, Terese Lea; Kofoed, Kristian

    2015-01-01

    presentation of Factor I deficiency varies and includes severe recurrent bacterial infections, glomerulonephritis and autoimmune diseases. The patient, a 28-years old woman with consanguineous parents, presented with recurrent leukocytoclastic vasculitis in the lower extremities with no associated systemic...... mutations vary among patients sole association with leukocytoclastic vasculitis redefines the clinical spectrum of complete Factor I deficiency....

  12. Infliximab effects compared to conventional therapy in the management of retinal vasculitis in Behçet disease.

    Science.gov (United States)

    Tabbara, Khalid F; Al-Hemidan, Amal I

    2008-12-01

    To assess the outcome of retinal vasculitis in patients with Behçet disease treated with infliximab compared to treatment with conventional therapy. Nonrandomized, retrospective comparative clinical study. Patients with Behçet disease with all four major criteria were included in this study. Patients had recurrent episodes of uveitis and retinal vasculitis. Thirty-three patients (Group 1) were treated with oral prednisone, cyclosporine, and azathioprine or methotrexate for a minimum period of three months. Ten patients (Group 2) who failed to respond to conventional therapy were given infliximab at a dose of 5 mg/kg in a single intravenous infusion on day 1 and every two weeks for a total of six doses. Patients were given the same treatment during each subsequent relapse. The main outcome measures were the number of relapses, visual outcome, and ocular complications. The mean follow-up period was 36 months in Group 1 and 30 months in Group 2. The mean number of relapses was significantly reduced and the duration of remission was longer in the infliximab therapy group compared to conventional therapy group (P < .0001). The visual acuity at 24 months follow-up was significantly better in patients treated with infliximab (Group 2) when compared to conventional therapy (Group 1) (P = .0059). Patients with Behçet disease had significant decrease in inflammation, improvement of visual acuity, and reduced ocular complications following infliximab when compared to conventional therapy. The number of relapses was less in the infliximab treatment group than the conventional therapy group.

  13. Plasma Exchange for Renal Vasculitis and Idiopathic Rapidly Progressive Glomerulonephritis: A Meta-analysis

    DEFF Research Database (Denmark)

    Walsh, Michael; Catapano, Fausta; Szpirt, Wladimir

    2010-01-01

    .9). LIMITATIONS:: Although the primary result was statistically significant, there is insufficient statistical information to reliably determine whether plasma exchange decreases the composite of end-stage renal disease or death. CONCLUSIONS:: Plasma exchange may decrease the composite end point of end......BACKGROUND:: Plasma exchange may be effective adjunctive treatment for renal vasculitis. We performed a systematic review and meta-analysis of randomized controlled trials of plasma exchange for renal vasculitis. STUDY DESIGN:: Systematic review and meta-analysis of articles identified from...... electronic databases, bibliographies, and studies identified by experts. Data were abstracted in parallel by 2 reviewers. SETTING & POPULATION:: Adults with idiopathic renal vasculitis or rapidly progressive glomerulonephritis. SELECTION CRITERIA FOR STUDIES:: Randomized controlled trials that compared...

  14. Levamisole-Contaminated Cocaine: An Emergent Cause of Vasculitis and Skin Necrosis

    Directory of Open Access Journals (Sweden)

    Osama Souied

    2014-01-01

    Full Text Available The prevalence of cocaine adulterated with levamisole-induced vasculitis is increasing and physicians should be aware of this unique entity. There have been many reports of cutaneous vasculitis syndrome caused by cocaine which is contaminated with levamisole. Levamisole was used as an antihelminth drug and later was rescinded from use in humans due to adverse effects. Through this paper, we will report a 39-year-old crack cocaine user who presented with purpuric rash and skin necrosis of his ear lobes. Levamisole-induced vasculitis syndrome was suspected. A urine toxicology screen was positive for cocaine, opiates, and marijuana. Blood work revealed positive titres of ANA and p-ANCA, as well as anti-cardiolipin antibody. Biopsy taken from the left ear showed focal acute inflammation, chronic inflammation with thrombus formation, and extravasated blood cells. Treatment was primarily supportive with wound care.

  15. Radiation retinopathy caused by low dose irradiation and antithyroid drug-induced systemic vasculitis

    International Nuclear Information System (INIS)

    Sonoda, Koh-hei; Ishibashi, Tatsuro

    2005-01-01

    We report on a patient with Graves' disease with radiation retinopathy caused by low-dose irradiation and antithyroid drug-induced antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis. A 38-year-old woman with Graves' disease presented with bilateral blurred vision, micro-aneurysms, telangiectasia, and macular edema. The patient was examined by ophthalmoscopy and fluorescein angiography, and radiation retinopathy was diagnosed. The patient had been treated with low-dose irradiation for her Graves' ophthalmopathy a few years earlier. She also had ANCA-positive vasculitis induced by the antithyroid drug (propylthiouracil, PTU) that had been prescribed for her at that time. Because of multiple avascular areas on both retinas, she was treated by intensive retinal photocoagulation to control progressive retinopathy. The radiation doses used to treat Graves' disease ophthalmopathy are low. Nevertheless, there is still a risk of radiation retinopathy developing in patients with PTU-induced ANCA-positive vasculitis. (author)

  16. Cocaine-induced vasculitis with cutaneous manifestation: A recurrent episode after 2 years

    Directory of Open Access Journals (Sweden)

    Thein Swe

    2016-01-01

    Full Text Available Cocaine is a popular recreational drug in the United States, and up to 70% of the seized cocaine contains levamisole which is an antihelminthic that can cause cutaneous vasculitis with necrosis and positive antineutrophil cytoplasmic antibodies (ANCAs. Here, we report a unique case of recurrent cocaine-induced vasculitis in a patient who smokes cocaine for more than 20 years. A 38-year-old woman complained of painful erythematous rash in her right arm and right thigh which appeared some hours after smoking cocaine. Physical examination revealed tender, erythematous base, retiform purpura with necrosis and bullae. Serological test showed high atypical perinuclear ANCA titer of 1:320 and antimyeloperoxidase antibody level of 20.4 U/mL. Cocaine-induced vasculitis should be one of the differential diagnoses in cocaine abusers who present with painful rash and areas of necrosis. Early diagnosis is important since it is an emerging public health concern.

  17. Deferasirox-induced urticarial vasculitis in a patient with myelodysplastic syndrome.

    Science.gov (United States)

    Polat, Asude Kara; Belli, Asli Akin; Karakus, Volkan; Dere, Yelda

    2017-01-01

    Deferasirox is an iron chelator agent used in the treatment of diseases with iron overload, such as thalassemia and myelodysplastic syndrome. Although the majority of adverse reactions of deferasirox involve gastrointestinal symptoms and increase in serum creatinine and transaminases, skin rashes, such as maculopapular and urticarial eruptions, have also been reported. This study reports a case of myelodysplastic syndrome with urticarial vasculitis due to deferasirox therapy. Drug eruption was been confirmed by means of a challenge test, together with histopathological and clinical findings. To the best of our knowledge, we report the first case of deferasirox-induced urticarial vasculitis. Physicians should be aware of the possibility of urticarial vasculitis on deferasirox therapy and the fact that the discontinuation of the drug generally results in improvement.

  18. Acute kidney injury with granulomatous interstitial nephritis and vasculitis revealing sarcoidosis

    Directory of Open Access Journals (Sweden)

    Amel Harzallah

    2017-01-01

    Full Text Available Sarcoidosis is an inflammatory disease that affects mostly the lungs and lymph glands. Renal involvement is rare and especially vasculitis. We report a case who presented an acute kidney failure and had sarcoidosis with vasculitis and nodular splenic involvement. A 35-year-old woman presenting a Lofgren syndrome was hospitalized for acute renal failure with cervical lymphadenopathy without other clinical findings. Laboratory data disclosed elevated angiotensin converting enzyme serum level. Abdominal ultrasound showed a multinodular spleen. Renal histology revealed granulomatous interstitial nephritis with necrotizing vasculitis. Outcome was favorable after the institution of high dose corticosteroids along with cyclophosphamide. Renal involvement is rare in sarcoidosis. However, the diagnostic delay should be avoided to improve the outcome.

  19. Acute kidney injury with granulomatous interstitial nephritis and vasculitis revealing sarcoidosis.

    Science.gov (United States)

    Harzallah, Amel; Kaaroud, Hayet; Boubaker, Karima; Barbouch, Samia; Goucha, Rim; Hamida, Fethi Ben; Abdallah, Taieb Ben

    2017-01-01

    Sarcoidosis is an inflammatory disease that affects mostly the lungs and lymph glands. Renal involvement is rare and especially vasculitis. We report a case who presented an acute kidney failure and had sarcoidosis with vasculitis and nodular splenic involvement. A 35-year-old woman presenting a Lofgren syndrome was hospitalized for acute renal failure with cervical lymphadenopathy without other clinical findings. Laboratory data disclosed elevated angiotensin converting enzyme serum level. Abdominal ultrasound showed a multinodular spleen. Renal histology revealed granulomatous interstitial nephritis with necrotizing vasculitis. Outcome was favorable after the institution of high dose corticosteroids along with cyclophosphamide. Renal involvement is rare in sarcoidosis. However, the diagnostic delay should be avoided to improve the outcome.

  20. Urinary Biomarkers in Relapsing Antineutrophil Cytoplasmic Antibody-associated Vasculitis

    Science.gov (United States)

    Lieberthal, Jason G.; Cuthbertson, David; Carette, Simon; Hoffman, Gary S.; Khalidi, Nader A.; Koening, Curry L.; Langford, Carol A.; Maksimowicz-McKinnon, Kathleen; Seo, Philip; Specks, Ulrich; Ytterberg, Steven R.; Merkel, Peter A.; Monach, Paul A.

    2015-01-01

    Objective Glomerulonephritis (GN) is common in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), but tools for early detection of renal involvement are imperfect. We investigated 4 urinary proteins as markers of active renal AAV: alpha-1 acid glycoprotein (AGP), kidney injury molecule-1 (KIM-1), monocyte chemoattractant protein-1 (MCP-1), and neutrophil gelatinase-associated lipocalin (NGAL). Methods Patients with active renal AAV (n = 20), active nonrenal AAV (n = 16), and AAV in longterm remission (n = 14) were identified within a longitudinal cohort. Urinary biomarker concentrations (by ELISA) were normalized for urine creatinine. Marker levels during active AAV were compared to baseline remission levels (from 1–4 visits) for each patient. Areas under receiver-operating characteristic curves (AUC), sensitivities, specificities, and likelihood ratios (LR) comparing disease states were calculated. Results Baseline biomarker levels varied among patients. All 4 markers increased during renal flares (p < 0.05). MCP-1 discriminated best between active renal disease and remission: a 1.3-fold increase in MCP-1 had 94% sensitivity and 89% specificity for active renal disease (AUC = 0.93, positive LR 8.5, negative LR 0.07). Increased MCP-1 also characterized 50% of apparently nonrenal flares. Change in AGP, KIM-1, or NGAL showed more modest ability to distinguish active renal disease from remission (AUC 0.71–0.75). Hematuria was noted in 83% of active renal episodes, but also 43% of nonrenal flares and 25% of remission samples. Conclusion Either urinary MCP-1 is not specific for GN in AAV, or it identifies early GN not detected by standard assessment and thus has potential to improve care. A followup study with kidney biopsy as the gold standard is needed. PMID:23547217

  1. Clinical features and prognostic factors of cutaneous vasculitis among dermatology patients in Johor Bahru, Malaysia.

    Science.gov (United States)

    Latha, S; Choon, S E; Tey, K E; Chee, Y N

    2017-12-01

    Cutaneous vasculitis is common, yet the risk factors for its chronicity have not been established. To describe the clinical spectrum and identify risk factors for chronicity of cutaneous vasculitis. Retrospective data analysis of 275 patients diagnosed with cutaneous vasculitis from January 2008 to December 2013. The mean age was 33.7 (±17.89) years, with female predominance. The majority of patients were Malays (67.3%). Skin biopsy was performed in 110 (40%) patients. The commonest sign was palpable purpura (30.6%). The aetiology remained elusive in 51.3% of patients. Common identifiable causes include infection (19.7%) and connective tissue disease (10.2%). Extracutaneous features were noted in 46.5% of patients. Erythrocyte sedimentation rate and antinuclear antibody were raised in 124 of 170 and 27 of 175 patients with documented results respectively. Cutaneous vasculitis was the presenting symptom in seven patients with newly diagnosed systemic lupus erythematosus. Anti Streptolysin O Titre was positive in 82 of 156 patients with documented results. Despite antibiotics, 31.7% of them had chronic lesions. Prednisolone alone was used in 20% of patients while 16.4% needed steroid-sparing agents. Most patients who needed systemic therapy (62%) had unidentifiable aetiology. Among the 155 patients who remained under follow up, 36.4% had chronic disease, one patient succumbed due to septicaemia, and the rest fully recovered within three months. The presence of ulcerative lesion was significantly associated with developing chronic vasculitis (p=0.003). The clinical spectrum of cutaneous vasculitis in our population was similar to other studies. Ulcerative lesion predicts a chronic outcome.

  2. Transcriptional profiling of PBMCs unravels B cell mediated immunopathogenic imprints of HCV vasculitis.

    Science.gov (United States)

    Comstock, Emily; Kim, Cheol-Woo; Murphy, Alison; Emmanuel, Benjamin; Zhang, Xi; Sneller, Michael; Poonia, Bhawna; Kottilil, Shyamasundaran

    2017-01-01

    B cell depletion therapy using rituximab has been shown to be effective in achieving remission in patients with HCV-mixed cryoglobulinemic (MC) vasculitis. Previously, we have demonstrated abnormalities in peripheral immune cells involving neutrophils, chemotaxis, and innate immune activation among patients with HCV-MC vasculitis when compared to HCV patients without vasculitis. In this study, we evaluated the effect of B cell depletion therapy on transcriptional profiles of peripheral blood mononuclear cells before and after riruximab therapy, in order to unravel the pathogenic mechanism involved in HCV-MC vasculitis induced by abnormal B cell proliferation. DNA microarray analysis was performed using RNA from PBMCs from seven patients with HCV-MC vasculitis and seven normal volunteers. DNA was hybridized to Affymetrix U133A chips. After normalization, differentially expressed gene list with treatment was generated using partitional clustering. RT-PCR, flow cytometry, and enzyme immunoassay (EIA) was used to validate DNA microarray findings. Differentially expressed genes included B cells and non-B cell genes. Validation of genes using purified cell subsets demonstrated distinct effect of B cell depletion therapy on non-B cells, such as monocytes, T cells, and NK cells. Notably, B lymphocyte stimulator (BLyS) levels were persistently elevated in patients who subsequently relapsed. In conclusion, pathogenesis of HCV-MC vasculitis is mediated by abnormal proliferation of B cells, driven by BLyS, leading to significant effects on non-B cells in mediating symptomatology. Future therapeutics using a combination approach of B cell depletion and proliferation may be desired to achieve long-term remission.

  3. Vasculites dos médios e grandes vasos Medium and large vessel vasculitis

    Directory of Open Access Journals (Sweden)

    Hebert Roberto Clivati Brandt

    2009-02-01

    Full Text Available As vasculites são constituídas por um grande grupo de síndromes caracterizadas por inflamação e necrose da parede dos vasos sangüíneos, resultando em estreitamento ou oclusão do lúmen. A distribuição dos vasos envolvidos varia consideravelmente e serve de base para a classificação das síndromes vasculíticas: grandes vasos (arterite de Takayasu, arterite temporal; pequenos e médios vasos (poliarterite nodosa, síndrome de Churg-Strauss, granulomatose de Wegener, vasculites nas doenças reumáticas; e pequenos vasos (vasculite de hipersensibilidade, púrpura de Henoch-Schonlein, poliangeíte microscópica, crioglobulinemia. Este artigo revisa as vasculites dos médios e grandes vasos e opções terapêuticas atuais; além de oferecer orientação sobre abordagem diagnóstica e tratamento dos pacientes com suspeita de vasculite cutânea.Vasculitis comprises a broad group of syndromes characterized by inflammation and necrosis on the walls of blood vessels, resulting in narrowing or occlusion of the lumen. The distribution of involved blood vessel varies considerably and serves as the basis for one classification of vasculitic syndromes: large vessels (Takayasu arteritis, giant cell arteritis; medium and small muscular arteries (polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, vasculitis in rheumatic diseases; and small vessels ("hypersensitivity" vasculitis, Henoch-Schonlein purpura, microscopic polyangiitis, cryoglobulinemia. This article reviews medium and large vessel vasculitis and current treatment options; it also presents a comprehensive approach to diagnosing and treating patients with suspected cutaneous vasculitis.

  4. Isolated vasculitis involving the female genital tract: clinicopathologic spectrum and phenotyping of inflammatory cells.

    Science.gov (United States)

    Abu-Farsakh, H; Mody, D; Brown, R W; Truong, L D

    1994-06-01

    Isolated vasculitis involving the female genital tract (IVF) is rare. Although both giant cell arteritis (GCA) and polyarteritis nodosa (PAN) types of involvement have been documented, several clinicopathologic features of IVF are not clearly understood. We wish to report two cases of IVF (one GCA and one PAN) and compare them with previously reported cases. Including our two cases, we found a total of 33 reported cases of IVF, which included 18 GCA and 15 PAN. In cases of GCA, all parts of the female genital tract were involved with roughly comparable frequency, whereas in the case of PAN, the cervix is uniformly involved, with the vulva and myometrium being affected once and twice, respectively. In all cases of GCA and most cases of PAN, the vasculitis represented an incidental finding upon microscopic examination of the female genital tract removed for unrelated problems; however, in one case of PAN, vaginal bleeding probably related to vasculitis-induced cervical ulcer was reported. In either category, no clearcut pattern of predisposing factors was identified and, at the time of diagnosis, none of the patients had any diseases known to be associated with vasculitis, such as connective tissue disease, rheumatoid arthritis, or drug hypersensitivity. Although antineutrophilic cytoplasmic antibodies were recently described as sensitive and specific markers for systemic vasculitis, tests for these antibodies were not done on previously reported cases and were negative for both current cases. Long-term follow-up in IVF indicates that even without any specific treatment for vasculitis, systemic involvement did not occur.(ABSTRACT TRUNCATED AT 250 WORDS)

  5. Churg-Strauss syndrome associated with antiphospholipid antibodies in a patient with retinal vasculitis.

    Science.gov (United States)

    Sánchez-Vicente, J L; Gálvez-Carvajal, S; Medina-Tapia, A; Rueda, T; González-García, L; Szewc, M; Muñoz-Morales, A

    2016-11-01

    We present the case of a 69-year-old woman with unilateral retinal vasculitis. Investigations showed asthma, rhinosinusitis, nasal polyposis, peripheral blood eosinophilia, increased sedimentation rate, proteinuria, and antiphospholipid antibodies. Anti-neutrophil cytoplasmic antibodies (ANCA) were negative. Although her anti-neutrophil cytoplasmatic antibody (ANCA) status was negative, taking into account the other clinical and laboratory features, retinal vasculitis was thought to be an ocular manifestation of Churg-Strauss syndrome. Treatment was started with high-dose corticosteroids and anticoagulant therapy. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  6. [Diffuse large B-cell lymphoma complicated with drug-induced vasculitis during administration of pegfilgrastim].

    Science.gov (United States)

    Ito, Yuta; Noda, Kentaro; Aiba, Keisuke; Yano, Shingo; Fujii, Tsunehiro

    A 59-year-old female with diffuse large B-cell lymphoma was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP) regimen. In addition, we administered pegfilgrastim for treating chemotherapy-induced febrile neutropenia. She complained of fever and neck and chest pain a few days after pegfilgrastim administration during the third and fourth courses of R-CHOP. Radiological imaging revealed an inflammation of large vessels, which led to the diagnosis of drug-associated vasculitis. We confirmed that vasculitis observed in this case was caused by pegfilgrastim administration because similar symptoms appeared with both injections of pegfilgrastim.

  7. Urticaria Vasculitis: Estudio retrospectivo de 20 casos y protocolo diagn??stico-terap??utico

    OpenAIRE

    Soriano Hern??ndez, Isabel; Orgaz Molina, Jacinto; Arias Santiago, Salvador; El-Ahmed, Husein; Ortego-Centeno, Norberto; Callejas, Jos?? L.; Fern??ndez Pugnaire, M. Antonia; Naranjo Sintes, Ram??n Jos??

    2011-01-01

    La urticaria vasculitis es una enfermedad cr??nica que se caracteriza por episodios urticariales o de angioedema que puede asociarse a niveles bajos de complemento. Hemos realizado un estudio descriptivo retrospectivo que incluye a 20 pacientes con urticaria vasculitis diagnosticados en los ??ltimos 5 a??os donde analizamos los s??ntomas m??s frecuentes y los tratamientos que se han empleado. A continuaci??n se ha dise??ado un protocolo diagn??stico-terap??utico de Urticaria Vascu...

  8. Infective endocarditis due to Bartonella bacilliformis associated with systemic vasculitis: a case report

    Directory of Open Access Journals (Sweden)

    Joshua Peñafiel-Sam

    Full Text Available Abstract Infective endocarditis due to Bartonella bacilliformis is rare. A 64-year-old woman, without previous heart disease, presented with 6 weeks of fever, myalgias, and arthralgias. A systolic murmur was heard on the tricuspid area upon examination, and an echocardiogram showed endocardial lesions in the right atrium. Bartonella bacilliformis was isolated in blood cultures, defining the diagnosis of infective endocarditis using Duke’s criteria. Subsequently, the patient developed clinical and laboratory features compatible with antineutrophil cytoplasmic antibody-associated vasculitis. This case presents an uncommon complication of B. bacilliformis infection associated with the development of systemic vasculitis.

  9. Library Locations

    Data.gov (United States)

    Allegheny County / City of Pittsburgh / Western PA Regional Data Center — Carnegie Library of Pittsburgh locations including address, coordinates, phone number, square footage, and standard operating hours. The map below does not display...

  10. Treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis with high-dose intravenous immunoglobulin.

    Science.gov (United States)

    Richter, C; Schnabel, A; Csernok, E; De Groot, K; Reinhold-Keller, E; Gross, W L

    1995-07-01

    In this uncontrolled study 15 patients with ANCA-associated systemic vasculitis, who were poor responders to conventional therapy, were treated with single or multiple courses of intravenous immunoglobulin (IVIG), 30 g/day over 5 days. Clinical and serological evaluation was performed before and 4 weeks after IVIG. Six of the 15 patients experienced clinically significant benefit from IVIG. Improvement was confined to single organ manifestations (skin, ENT findings), no improvement was seen with conjunctivitis and scleritis, pericarditis or nephritis. No patient experienced complete remission after IVIG. Repeated courses of IVIG at 4-week intervals were no more effective than single courses. In six anti-proteinase 3 (PR3)-positive patients pretreatment sera were incubated with F(ab')2 fragments of the IVIG preparation in vitro to measure the inhibitory effect of IVIG on anti-PR3 activity. An inhibition of anti-PR3 activity by 25-70% was observed; this did not correlate with clinical effects. Approximately 40% of patients benefited from IVIG treatment, though complete remission of disease activity did not occur. Neither clinical characteristics nor the inhibitory effect of the IVIG preparation on serum anti-PR3 activity in vitro predicted clinical response to this treatment modality.

  11. Gene Locater

    DEFF Research Database (Denmark)

    Anwar, Muhammad Zohaib; Sehar, Anoosha; Rehman, Inayat-Ur

    2012-01-01

    software's for calculating recombination frequency is mostly limited to the range and flexibility of this type of analysis. GENE LOCATER is a fully customizable program for calculating recombination frequency, written in JAVA. Through an easy-to-use interface, GENE LOCATOR allows users a high degree...... of flexibility in calculating genetic linkage and displaying linkage group. Among other features, this software enables user to identify linkage groups with output visualized graphically. The program calculates interference and coefficient of coincidence with elevated accuracy in sample datasets. AVAILABILITY......: The database is available for free at http://www.moperandib.com....

  12. Magnetic resonance sialography of the parotid glands in chronic hepatitis C virus patients with and without vasculitis.

    Science.gov (United States)

    Shahin, Amira A; Hussein, Hanan; Gaber, Wafaa; Elbaz, Tamer; Salah El Din, Lamia A

    2017-03-01

    Hepatitis C virus (HCV) is sialotropic. The pathogenesis of sicca manifestations in patients with chronic HCV infection is not fully understood. We aimed to detect changes in magnetic resonance sialography (MRS) of HCV patients with and without vasculitis. We studied 32 HCV patients (19 female, mean age 48.8 ± 10.3 years) and 20 age- and gender-matched healthy controls. Half of the patients had vasculitis. Demographic, clinical and serological data were prospectively evaluated. In patients with vasculitis, the disease activity was assessed by the Birmingham Vasculitis Activity Score (BVAS). MRS was performed on all patients and controls. Abnormal MRS was found in 25% of patients, (6/16 and 2/16 in patients with and without vasculitis, respectively). Among patients with vasculitis, those with abnormal MRS had longer disease duration, higher leukocytic and lymphocytic counts and more frequent cryoglobulinemia (P vasculitis, longer disease duration and cryoglobulinemia were associated with abnormal findings on MRS. To confirm our results, we propose larger-scale, multicentre studies with longer evaluation periods. © 2014 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  13. Vasculitis and infections : Contribution to the issue of autoimmunity reviews devoted to "autoimmunity and infection"

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.; Tadema, Henko

    2008-01-01

    Infections are associated with secondary forms of vasculitis. However, there is increasing evidence that microbial agents play a role also in primary systemic vasculitides. For a long time it has been noted that Hepatitis B virus (HBV) is involved in polyarteritis nodosa (PAN) although the incidence

  14. Necrotizing Lymphocytic Vasculitis Limited to the Peripheral Nerves: Report of Six Cases and Review

    Directory of Open Access Journals (Sweden)

    José Félix Restrepo

    2009-01-01

    Full Text Available Background. The systemic vasculitides are syndromes characterized by inflammation and injury (necrosis or thrombosis of blood vessels, resulting in clinical manifestations according to the affected vascular bed, but not classically in stocking-glove neuropathy. Objective. To describe a form of primary vasculitis affecting strictly peripheral nerves manifesting as stocking-glove neuropathy. Methods. Case series of 110 patients seen in three centers in Bogotá who presented with symptoms and signs of polyneuropathy and/or were identified with vasculitis affecting only the peripheral nerves, and who underwent sural nerve biopsy. Results. Six patients had a vasculitis affecting only the peripheral nerves diagnosed on sural nerve biopsy which demonstrated a mixed infiltrate of monocytes/macrophages and lymphocytes especially in the small epineurial blood vessels. Over time, all had worsening of symptoms, with grip weakness and motor deficits in the hand and feet. Serologies and acute phase reactants were normal in all patients. Treatment response to immunosuppression was satisfactory in 5 patients; 1 patient had progressive neurologic damage. Conclusions. There is a distinct form of primary vasculitis of the peripheral nervous system characterized by distal sensory polyneuropathy with stocking-glove distribution with good prognosis, few and minor relapses and good response to treatment even after delayed diagnosis.

  15. Patients with systemic vasculitis have increased levels of autoantibodies against oxidized LDL

    NARCIS (Netherlands)

    Swets, BP; Brouwer, DAJ; Tervaert, JWC

    Oxidation of low density lipoprotein (LDL) is considered to play an important role in the development of atherosclerosis and increased levels of autoantibodies against oxidized LDL have been found in patients with various manifestations of atherosclerosis. Patients with vasculitis are prone to the

  16. Ultrasound and Biochemical Indices of Endothelial Dysfunction at Hemorrhagic Vasculitis in Children

    Directory of Open Access Journals (Sweden)

    Yu.V. Odynets

    2016-03-01

    Full Text Available 60 children with hemorrhagic vasculitis were examined in order to detect ultrasound and biochemical markers of endothelial dysfunction, depending on the degree of acti­vity and the form. Serum monocyte chemoattractant protein 1 (MCP-1 concentration was determined using enzyme-linked immunoassay, the content of nitric oxide by its stable metabolites — NO2, NO3 and S-nitrosothiol, von Willebrand factor — using aggregometry, intima-media complex thickness of the common carotid artery and endothelium-dependent dilation of the brachial artery were evaluated. Levels of von Willebrand factor and MCP-1 significantly increased, depending on the degree of hemorrhagic vasculitis activity. Statistical data show a significant decrease in the concentration of nitric oxide metabolites in the blood serum of children with mixed form and nephritic syndrome. Intima-media complex thickness of the common carotid artery was increased in children with severe hemorrhagic vasculitis, and in children with a mixed form with renal syndrome. We have detected a pathological reaction of endothelium-dependent dilation of the brachial artery regardless of the degree of activity and the form of hemorrhagic vasculitis, this fact evidences direct involvement of the endothelium in the pathological process.

  17. HBV-Associated Cryoglobulinemic Vasculitis: Remission after Antiviral Therapy with Entecavir

    Directory of Open Access Journals (Sweden)

    Mauro Viganò

    2014-06-01

    Full Text Available Background/Aims: Cryoglobulinemic vasculitis remains an uncommon complication of hepatitis B virus infection. Methods: We report the case of a 40-years old female Chinese patient with chronic hepatitis B developing cryoglobulinemic vasculitis with multiple organ involvement (liver, kidney, and skin coupled with weakness, arthralgias, haemolytic anaemia, and autoimmune thyroiditis. She received entecavir mono-therapy at dose adjusted for estimated glomerular filtration rate. Results: Within five months of entecavir treatment, hepatitis B viraemia decreased below the limit of detection with normal serum amino-transferase levels, HBeAg clearance occurred, vasculitis regressed with disappearance of purpura and ascites; in addition, renal function normalized and nephritic syndrome remitted. After a five-year follow-up, the patient is asymptomatic with intact kidney function, proteinuria in the normal range, and normal liver biochemistry, despite the antiviral treatment was withdrawn and the patient remained HBsAg positive. Conclusions: This is the second case of hepatitis B virus-related cryoglobulinemic vasculitis successfully treated with entecavir suggesting that effective antiviral therapy may counteract both the hepatic and extra-hepatic manifestations of infection by hepatitis B virus.

  18. Candida albicans-associated necrotizing vasculitis producing life-threatening gastrointestinal hemorrhage.

    LENUS (Irish Health Repository)

    Sargent, Jeremy

    2012-02-01

    Patients undergoing treatment of acute lymphoblastic leukemia are at risk for fungal infections including disseminated candidiasis. We describe a case of systemic Candida albicans infection associated with life-threatening gastrointestinal hemorrhage due to unusual necrotizing vasculitis involving the gastrointestinal tract. We explore the association between Candida and such vasculopathy.

  19. Plasma exchange in antineutrophil cytoplasmic antibody-associated vasculitis--a 25-year perspective

    DEFF Research Database (Denmark)

    Szpirt, Wladimir M

    2015-01-01

    Demonstration of a pathogenic role for antineutrophil cytoplasmic antibodies (ANCA) underlies the scientific rationale for plasma exchange (PLEX) in the treatment of ANCA-associated vasculitis (AAV). Most clinical evidence of efficacy concerns the use of PLEX for the recovery of renal function...

  20. Epitope specificity determines pathogenicity and detectability in ANCA-associated vasculitis

    Science.gov (United States)

    ABSTRACT BACKGROUND Anti-neutrophil cytoplasmic autoantibodies (ANCA) specific for myeloperoxidase (MPO) or proteinase 3 (PR3) are detectable in >90% of patients with ANCA-associated vasculitis (AAV). ANCA titers do not correlate well with disease activity. In vivo and in vi...

  1. Imaging Findings of Central Nervous System Vasculitis Associated with Goodpasture's Syndrome: a Case Report

    International Nuclear Information System (INIS)

    Kim, Jee Young; Ahn, Kook Jin; Jung, Jung Im; Jung, So Lyung; Kim, Bum Soo; Hahn, Seong Tae

    2007-01-01

    We report a rare case of CNS vasculitis associated with Goodpasture's syndrome in a 34-year-old man, who presented with a seizure and sudden onset of right sided weakness. He also had recurrent hemoptysis of one month's duration. Goodpasture's syndrome is histologically diagnosed by intense linear deposits of IgG along the glomerular basement membrane in both renal and lung tissues. oodpasture's syndrome is a rare disease, characterized by rapidly progressive glomerulonephritis, diffuse pulmonary hemorrhage and circulating antiglomerular basement membrane antibody (anti-GBM antibody). Central nervous system (CNS) manifestations in Goodpasture's syndrome are extremely rare, with only a few cases having been reported in the literature (8 10). Therefore, we present our imaging findings of CNS vasculitis associated with Goodpasture's syndrome, together with a review of the relevant literature. In summary, CNS vasculitis associated with Goodpasture's syndrome is extremely rare. Awareness of the imaging findings, as well as the clinical significance of CNS vasculitis associated with Goodpasture's syndrome, can be helpful in making the correct diagnosis and subsequent management of this rare condition

  2. Microvesicle transfer of kinin B1-receptors is a novel inflammatory mechanism in vasculitis.

    Science.gov (United States)

    Kahn, Robin; Mossberg, Maria; Ståhl, Anne-Lie; Johansson, Karl; Lopatko Lindman, Ingrid; Heijl, Caroline; Segelmark, Mårten; Mörgelin, Matthias; Leeb-Lundberg, L M Fredrik; Karpman, Diana

    2017-01-01

    During vasculitis, activation of the kinin system induces inflammation, whereby the kinin B1-receptor is expressed and activated after ligand binding. Additionally, activated blood cells release microvesicles into the circulation. Here we determined whether leukocyte-derived microvesicles bear B1-kinin receptors during vasculitis, and if microvesicles transfer functional B1-receptors to recipient cells, thus promoting inflammation. By flow cytometry, plasma from patients with vasculitis were found to contain high levels of leukocyte-derived microvesicles bearing B1-receptors. Importantly, renal biopsies from two patients with vasculitis showed leukocyte-derived microvesicles bearing B1-receptors docking on glomerular endothelial cells providing in vivo relevance. Microvesicles derived from B1-receptor-transfected human embryonic kidney cells transferred B1-receptors to wild-type human embryonic kidney cells, lacking the receptor, and to glomerular endothelial cells. The transferred B1-receptors induced calcium influx after B1-receptor agonist stimulation: a response abrogated by a specific B1-receptor antagonist. Microvesicles derived from neutrophils also transferred B1-receptors to wild-type human embryonic kidney cells and induced calcium influx after stimulation. Thus, we found a novel mechanism by which microvesicles transfer functional receptors and promote kinin-associated inflammation. Copyright © 2016 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.

  3. Settings and artefacts relevant for Doppler ultrasound in large vessel vasculitis

    DEFF Research Database (Denmark)

    Terslev, L; Diamantopoulos, A P; Døhn, U Møller

    2017-01-01

    Ultrasound is used increasingly for diagnosing large vessel vasculitis (LVV). The application of Doppler in LVV is very different from in arthritic conditions. This paper aims to explain the most important Doppler parameters, including spectral Doppler, and how the settings differ from those used...

  4. [Acute pancreatitis as the presenting feature of an IgA vasculitis: An unusual presentation].

    Science.gov (United States)

    Fertitta, L; Noel, N; Ackermann, F; Lerolle, N; Benoist, S; Rocher, L; Lambotte, O

    2017-10-01

    IgA vasculitis is a systemic small vessel leukocytoclastic vasculitis characterized by skin purpura, arthritis, abdominal pain and nephritis. Most of the abdominal complications are due to edema and hemorrhage in the small bowel wall, but rarely to acute secondary pancreatitis. Here, we report a 53-year-old woman who presented with acute pancreatitis and, secondarily, developed skin purpura and arthritis at the seventh day of the clinical onset. Biological tests and computed tomographic scan allowed to rule out another cause of pancreatitis and IgA vasculitis was diagnosed as its etiology. The outcome was favorable without any relapse on glucocorticoids. Despite its rarity, pancreatitis is a potential life-threatening complication of IgA vasculitis in which the role of glucocorticoids and immunosuppressive drugs remains uncertain. A prompt elimination of other usual pancreatitis etiologies is mandatory to improve the management of the patients. Copyright © 2017 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  5. The incidence of vasculitis is increased in female stroke-prone ...

    African Journals Online (AJOL)

    Background: Vascular changes in hypertension share common characteristics with inflammatory wall injury. Since it is known that chronic inflammatory diseases are frequently more prevalent in females, this study tested the hypothesis that vasculitis would be more evident in female stroke-prone spontaneously hypertensive ...

  6. The Th1 and Th2 paradigm in ANCA-associated vasculitis

    NARCIS (Netherlands)

    Sanders, J S F; Stegeman, C A; Kallenberg, C G M

    2003-01-01

    In the pathogenesis of anti-neutrophil cytoplasm antibodies (ANCA)-associated vasculitis, T cell contribution is indicated by T cell-dependent ANCA production combined with the presence of T cells in inflammatory infiltrates. However, the exact pathogenic role of T cells in ANCA-associated

  7. Urinary matrix metalloproteinases reflect renal damage in anti-neutrophil cytoplasm autoantibody-associated vasculitis

    NARCIS (Netherlands)

    Sanders, J.S.F.; Huitema, M.G.; Hanemaaijer, R.; Goor, H. van; Kallenberg, C.G.M.; Stegeman, C.A.

    2007-01-01

    Renal expression of MMP-2, -9, and tissue inhibitor of MMP-1 (TIMP-1) correlates with histological disease activity in anti-neutrophil cytoplasm autoantibody (ANCA)-associated vasculitis (AAV). We studied whether urinary and plasma levels of MMP-2, -9, and TIMP-1 reflect renal expression of these

  8. Varicella Zoster Virus and Large Vessel Vasculitis, the Absence of an Association

    Directory of Open Access Journals (Sweden)

    Gary W. Procop

    2017-06-01

    Full Text Available Objective: It is controversial whether microorganisms play a role in the pathogenesis of large and medium vessel vasculitides (eg, giant cell arteritis [GCA], Takayasu arteritis [TAK] and focal idiopathic aortitis [FIA]. Recent studies have reported the presence of Varicella Zoster Virus (VZV within formalin-fixed, paraffin-embedded temporal arteries and aortas of about three-quarters or more of patients with these conditions, and in a minority of controls. In a prospective study, we sought to confirm these findings using DNA extracted from vessels that were harvested under surgically aseptic conditions and snap frozen. Methods and Results: DNA samples extracted from 11 surgically sterile temporal arteries and 31 surgically sterile thoracic aortas were used in an attempt to identify the vessel-associated VZV genome. Two different validated PCR methods were used. Thirty-one thoracic aorta aneurysm specimens included biopsies from 8 patients with GCA, 2 from patients with TAK, 6 from patients with FIA, and 15 from patients without vasculitis, who had non-inflammatory aneurysms. Eleven temporal artery biopsies were collected from 5 patients with GCA and 6 controls. The presence of VZV was not identified in either the specimens from patients with large vessel vasculitis or from the controls. Conclusions: Using surgically sterile snap-frozen specimens, we were unable to confirm recent reports of the presence of VZV in either aortas or temporal arteries from patients with large vessel vasculitis or controls. Keywords: Aorta and temporal artery biopsies, Varicella Zoster Virus, Large Vessel Vasculitis

  9. [The contribution of genes polymorphism of thrombophilia in clinical variability of hemorrhagic vasculitis].

    Science.gov (United States)

    Zhdanova, L V; Patrushev, L I; Dolgikh, V V; Bimbaev, A B-Zh; Khoĭkova, O Ch

    2014-01-01

    The article is devoted to the study of clinical and laboratory characteristics of the current of hemorrhagic vasculitis in children in the Republic of Buryatia. The study included 27 patients aged 7.6 +/- 4.02 years, who conducted clinical and laboratory tests, immunological study of antiphospholipids of antibodies, genetic testing for thrombophilia markers of candidate genes. The results showed that hemorrhagic vasculitis often affects children of Buryat nationality. In 96% of cases there are mixed clinical forms of the disease. 63% of children of hemorrhagic vasculitis preceded by various factors, a higher percentage of infectious diseases. The first clinical symptom in 63% of patients is a typical purpura hemorrhagic rash. Results of clinical laboratory blood tests revealed no significant deviations. Circulation of lupus anticoagulant was detected in 37% of subjects. The alphaCL IgM detected in 3 children, alphabeta2-GP-I IgA--in 4, alphabeta2-GP-I IgM--in 1 patient. Carriers of thrombophilia polymorphisms were in 95% of children. Noted that homozygous variants of genes polymorphisms of methylenetetrahydrofolatered reductase and plasminogen activator inhibitor-1 correlate with the presence of urinary symptoms and recurrence of the rash. The study shows the risk of recurrent flow of hemorrhagic vasculitis and nefritis of Henoch-Schonlein in children with thrombophilia gene polymorphism.

  10. Clinical Outcomes of Remission Induction Therapy for Severe Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

    NARCIS (Netherlands)

    Miloslavsky, E. M.; Specks, U.; Merkel, P. A.; Seo, P.; Spiera, R.; Langford, C. A.; Hoffman, G. S.; Kallenberg, C. G. M.; St Clair, E. W.; Tchao, N. K.; Viviano, L.; Ding, L.; Sejismundo, L. P.; Mieras, K.; Ikle, D.; Jepson, B.; Mueller, M.; Brunetta, P.; Allen, N. B.; Fervenza, F. C.; Geetha, D.; Keogh, K.; Kissin, E. Y.; Monach, P. A.; Peikert, T.; Stegeman, C.; Ytterberg, S. R.; Stone, J. H.

    Objective. To evaluate the reasons that complete remission is not achieved or maintained with original treatment in some patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) treated with rituximab (RTX) or with cyclophosphamide/azathioprine (CYC/AZA). Methods. The

  11. Rituximab for the Treatment of Relapses in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

    NARCIS (Netherlands)

    Miloslavsky, E. M.; Specks, U.; Merkel, P. A.; Seo, P.; Spiera, R.; Langford, C. A.; Hoffman, G. S.; Kallenberg, C. G. M.; Clair, E. W. St.; Tchao, N. K.; Viviano, L.; Ding, L.; Ikle, D.; Villarreal, M.; Jepson, B.; Brunetta, P.; Allen, N. B.; Fervenza, F. C.; Geetha, D.; Keogh, K.; Kissin, E. Y.; Monach, P. A.; Peikert, T.; Stegeman, C.; Ytterberg, S. R.; Stone, J. H.

    2014-01-01

    Objective. Disease relapses are frequent in anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). This study was undertaken to evaluate outcomes in patients with AAV who are re-treated with rituximab (RTX) and prednisone for severe disease relapses. Methods. The Rituximab in AAV trial

  12. Kidney Damage in Hemorrhagic Vasculitis Occurring in Childhood and Adulthood

    Directory of Open Access Journals (Sweden)

    O.V. Syniachenko

    2016-10-01

    Full Text Available Introduction. Nephropathy is diagnosed in 30–60 % of patients with hemorrhagic vasculitis (HV (Schönlein Henoch puprupa and occurred in each fourth of them in the onset of the disease and with the same incidence at first recurrence of the patho­logical process. In recent years, the relative and absolute number of patients with this form of glomerular disease significantly increased. According to the results of the kidney biopsy in children, Henoch glomerulonephritis (HGN is the most common variant of the secondary immunoglobulin (Ig A nephritis. The nature of the clinical course and morphological manifestations of the HGN in patients with HV, which began in childhood and adulthood, remains unexplored. This was the purpose and objectives of this study. Materials and methods. The study included 174 patients with HV (83 % of men and 47 % of women. In 92 cases, vasculitis debuted in children (on average in 12 years, and in 82 — in the adults (on average in 25 years. I, II and III degree of activity of pathological process are set at a ratio of 1 : 2 : 2. Seropositivity for high levels of IgA occurred in 40 % of cases, by the presence of rheumatoid factor — in 27 %. At the time of the survey, cutaneous syndrome was diagnosed in 68 % of patients in the form of urticarial, hemorrhagic, papule-nodular, papule-necro­tic, pustular-ulcerative, necrotic-ulcerative, nodose-ulcerative and polymorphic forms, and articular syndrome — in 48 %. In 24 cases, kidney biopsy was performed. Results. Renal disease was revealed in 71 % of patients with HV, while on the background of nephropathy the integral index of the severity of extrarenal patho­logy was significantly higher. According to the characteristics of the articular syndrome, patients with nephropathy and without it differed little among themselves. The severity of muscle syndrome has the impact on the development of the HV. In turn, renal pathology significantly influenced the development

  13. Location and logistics

    OpenAIRE

    Alumura, Sibel A.; Karab, Bahar Y.; Melo, M. Teresa

    2013-01-01

    Facility location decisions play a critical role in designing logistics networks. This article provides some guidelines on how location decisions and logistics functions can be integrated into a single mathematical model to optimize the configuration of a logistics network. This will be illustrated by two generic models, one supporting the design of a forward logistics network and the other addressing the specific requirements of a reverse logistics network. Several special cases and extensio...

  14. [Necrotizing vasculitis of the panarteritis nodosa type in a long-course primary Sjögren's syndrome].

    Science.gov (United States)

    Lahoz Rallo, C; Arribas López, J R; Arnalich Fernández, F; Monereo Alonso, A; Llanos Chavarri, M C; Camacho Siles, J

    1990-10-01

    Vasculitis is a complication happening in a third of patients with primary Sjögren's Syndrome. It can be of 2 types: neutrophilic or mononuclear and sometimes mixed. Very occasionally, a necrotizing vasculitis polyarteritis nodosa type during the evolution of Sjögren's Syndrome has appeared. A case of a female patient with a Sjögren's Syndrome of large evolution, who suddenly showed a poly-systemic affliction (nervous system, kidneys, muscles and digestive system) secondary to a necrotizing vasculitis type polyarteritis nodosa with a good response to immunosuppressors, is presented.

  15. Análisis Histórico de la Vasculitis. Clasificación y propuestas para un entendimiento.

    OpenAIRE

    Antonio Iglesias Gamarra

    2002-01-01

    Apartir de 1978, siendo residente de Reumatología iniciamos el estudio de las vasculitis en el Hospital San Juan de Dios de Bogotá. Se escribió el primer texto de vasculitis necrotizante que ganó el premio Salvat en 1980 y se publicó en 1982: Este libro tenía como objeto informar el tema a la comunidad médica.

    Durante mi estadía en Barranquilla, se realizó una descripción de 9 pacientes con los doctores Eduardo Egea, Gloria Garavito y Silvio Severini sobre vasculiti...

  16. A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report.

    Science.gov (United States)

    Bonilla-Abadía, Fabio; Muñoz-Buitrón, Evelyn; Ochoa, Carlos D; Carrascal, Edwin; Cañas, Carlos A

    2012-12-20

    The localized scleroderma (LS) known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our knowledge this is the first case of a morphea forming part of a multiple autoimmune syndrome (MAS) and presenting simultaneously with autoimmune thrombocytopenic purpura and central nervous system vasculitis. We report an uncommon case of a white 53 year old female patient with LS as part of a multiple autoimmune syndrome associated with pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis presenting a favorable response with thrombopoietin receptor agonists, pulses of methylprednisolone and cyclophosphamide. Is likely that LS have an autoimmune origin and in this case becomes part of MAS, which consist on the presence of three or more well-defined autoimmune diseases in a single patient.

  17. A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report

    Directory of Open Access Journals (Sweden)

    Bonilla-Abadía Fabio

    2012-12-01

    Full Text Available Abstract Background The localized scleroderma (LS known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our knowledge this is the first case of a morphea forming part of a multiple autoimmune syndrome (MAS and presenting simultaneously with autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case presentation We report an uncommon case of a white 53 year old female patient with LS as part of a multiple autoimmune syndrome associated with pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis presenting a favorable response with thrombopoietin receptor agonists, pulses of methylprednisolone and cyclophosphamide. Conclusion Is likely that LS have an autoimmune origin and in this case becomes part of MAS, which consist on the presence of three or more well-defined autoimmune diseases in a single patient.

  18. Fundamental Study of a Single Point Lean Direct Injector. Part I: Effect of Air Swirler Angle and Injector Tip Location on Spray Characteristics

    Science.gov (United States)

    Tedder, Sarah A.; Hicks, Yolanda R.; Tacina, Kathleen M.; Anderson, Robert C.

    2015-01-01

    Lean direct injection (LDI) is a combustion concept to reduce oxides of nitrogen (NOx) for next generation aircraft gas turbine engines. These newer engines have cycles that increase fuel efficiency through increased operating pressures, which increase combustor inlet temperatures. NOx formation rates increase with higher temperatures; the LDI strategy avoids high temperature by staying fuel lean and away from stoichiometric burning. Thus, LDI relies on rapid and uniform fuel/air mixing. To understand this mixing process, a series of fundamental experiments are underway in the Combustion and Dynamics Facility at NASA Glenn Research Center. This first set of experiments examines cold flow (non-combusting) mixing using air and water. Using laser diagnostics, the effects of air swirler angle and injector tip location on the spray distribution, recirculation zone, and droplet size distribution are examined. Of the three swirler angles examined, 60 degrees is determined to have the most even spray distribution. The injector tip location primarily shifts the flow without changing the structure, unless the flow includes a recirculation zone. When a recirculation zone is present, minimum axial velocity decreases as the injector tip moves downstream towards the venturi exit; also the droplets become more uniform in size and angular distribution.

  19. Lesson of the month 1: Large vessel vasculitis - a diagnostic challenge and the role of 18-fluorodeoxyglucose positron emission tomography.

    Science.gov (United States)

    Allard, Andrew; Mootoo, Ramesh

    2017-07-01

    Large vessel vasculitis can pose a significant diagnostic challenge. It may be insidious in onset with the only presenting symptoms consisting of constitutional compromise. It may mimic other pathologies and the only serological abnormalities may be abnormal inflammatory markers. Conventional imaging modalities may not be diagnostic. We present a case of large vessel vasculitis that proved a significant diagnostic challenge with diagnosis established on 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) computerised tomography (CT) imaging. This is one of five cases of large vessel vasculitis that were diagnosed in the rheumatology department at our trust over a 12-month period with diagnosis established with the use of 18F-FDG PET CT. We discuss the advantages of 18F-FDG PET CT over more conventional imaging modalities in diagnosing large vessel vasculitis. © Royal College of Physicians 2017. All rights reserved.

  20. Plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS)

    DEFF Research Database (Denmark)

    Walsh, Michael; Merkel, Peter A; Peh, Chen Au

    2013-01-01

    Granulomatosis with polyangiitis (GPA, Wegener's) and microscopic polyangiitis (MPA) are small vessel vasculitides collectively referred to as anti-neutrophil cytoplasm antibody-associated vasculitis (AAV). AAV is associated with high rates of morbidity and mortality due to uncontrolled disease...

  1. Características generales de 29 pacientes con vasculitis de pequeños vasos General characteristics of 29 patients with small vessel vasculitis

    Directory of Open Access Journals (Sweden)

    Nicolás Di Benedetto

    2010-04-01

    Full Text Available El objetivo de esta serie fue describir las características generales y manifestaciones clínicas de pacientes con vasculitis de pequeños vasos que concurrieron al servicio de autoinmunidad de un hospital de la comunidad y compararlas con la literatura. Se revisaron de manera retrospectiva las historias clínicas archivadas bajo el rótulo de vasculitis en un período de 16 años. Se seleccionaron aquellos pacientes que cumplieran criterios diagnósticos de vasculitis de pequeños vasos. Se extrajeron los datos de interés y se constató la supervivencia mediante contacto telefónico. Posteriormente se realizó una búsqueda bibliográfica y se compararon los resultados. Se incluyeron 13 pacientes con granulomatosis de Wegener, 6 con síndrome de Churg Strauss y 10 con poliangeítis microscópica. El 55% (16 fueron menores de 55 años al diagnóstico y hubo predominio del sexo femenino (2.6:1. La demora diagnóstica fue mayor a un año en el 46% de los casos. Los sistemas más frecuentemente afectados fueron respiratorio y otorrinolaringológico. El 79% presentó anticuerpos anti-citoplasma de neutrófilos (ANCA positivos. La mortalidad fue del 24% (7/29. Al comparar las características de los pacientes incluidos en esta serie con lo descripto en la literatura, se hallaron diferencias en cuanto a la forma de presentación, sistemas comprometidos y porcentaje de pacientes ANCA positivos; además se observó que el resultado negativo de este marcador parece asociarse con mayor demora diagnóstica y en consecuencia peor pronóstico, lo que remarca su importancia como herramienta adicional no invasiva al momento del diagnóstico.The objective of this series was to describe the general characteristics and clinical manifestations of patients with small vessel vasculitis who were assisted in the autoimmunity department of a community public hospital and to compare the results with the literature. Clinical records under the label of vasculitis in a

  2. A case of central nervous system vasculitis related to an episode of Guillain-Barrè syndrome

    OpenAIRE

    Sinardi, Daniele; Spada, Antonella; Marino, Antonella; Mondello, Epifanio

    2000-01-01

    The authors report their knowledge about an uncommon case of isolated vasculitis, restricted to the left sylvian artery during an auto-immune Guillain-Barrè syndrome (GBS), sustained by cytomegalovirus (CMV). An acute cardiopulmonary failure requiring a ventilator and vasopressor support manifested, notwithstanding plasma exchanging and immune-modulating therapy. An IgM-enriched formula administration coincided with a rapid amelioration of GBS and vasculitis to a complete recovery the next mo...

  3. Levels of Matrix Metalloproteinase-9 within Cerebrospinal Fluid in a Rabbit Model of Coccidioidal Meningitis and Vasculitis

    OpenAIRE

    Williams, Paul L.; Leib, Stephen L.; Kamberi, Perparim; Leppert, David; Sobel, Raymond A.; Bifrare, Yoeng-Delphine; Clemons, Karl V.; Stevens, David A.

    2017-01-01

    Matrix metalloproteinase (MMP)-9 is produced by the central nervous system and inflammatory cells in a variety of inflammatory conditions in both animals and humans. MMP-9 promotes inflammation, breakdown of the blood-brain barrier, and vasculitis. Because vasculitis is seen frequently in patients with coccidioidal meningitis (CM), this study evaluated the presence of MMP-9 within the cerebrospinal fluid (CSF) of rabbits infected intracisternally with Coccidioides immitis arthroconidia. Infec...

  4. A case of urticarial vasculitis in a female patient with lupus: Mycoplasma pneumoniae infection or lupus reactivation?

    Science.gov (United States)

    Diplomatico, Mario; Gicchino, Maria Francesca; Ametrano, Orsola; Marzuillo, Pierluigi; Olivieri, Alma Nunzia

    2017-05-01

    A 17-year-old female patient affected by systemic lupus erythematosus (SLE) (who had been taking 300 mg/die of hydroxychloroquine for 3 years), Graves' disease (treated with 10 mg/die of tapazole), and celiac disease came to our attention for urticarial vasculitis. She had been taking prednisone (25 mg/die) for 3 days, and her blood tests showed high levels of Mycoplasma pneumoniae IgM and IgG antibodies. The association between urticaria and M. pneumoniae infections can be present in up to 7% of the cases and, to the best of our knowledge, only two reports of urticarial vasculitis and M. pneumoniae in adults are available in the literature. Urticarial vasculitis can also be a rare cutaneous manifestation of SLE (affecting 2% of the patients), and our case is the first in the literature describing the coexistence of M. pneumoniae infection, SLE, and urticarial vasculitis in a pediatric patient, a case that rises an important differential diagnosis issue about the origin of urticarial vasculitis: SLE reactivation or urticarial vasculitis due to M. pneumoniae infection?

  5. Mnemonic strategy training improves memory for object location associations in both healthy elderly and patients with amnestic mild cognitive impairment: a randomized, single-blind study.

    Science.gov (United States)

    Hampstead, Benjamin M; Sathian, Krish; Phillips, Pamela A; Amaraneni, Akshay; Delaune, William R; Stringer, Anthony Y

    2012-05-01

    To evaluate the efficacy of mnemonic strategy training versus a matched-exposure control condition and to examine the relationship between training-related gains, neuropsychological abilities, and medial temporal lobe volumetrics in patients with amnestic mild cognitive impairment (aMCI) and age-matched healthy controls. Twenty-three of 45 screened healthy controls and 29 of 42 screened patients with aMCI were randomized to mnemonic strategy or matched-exposure groups. Groups were run in parallel, with participants blind to the other intervention. All participants completed five sessions within 2 weeks. Memory testing for object-location associations (OLAs) was performed during sessions one and five and at a 1-month follow-up. During Sessions 2-4, participants received either mnemonic strategy training or a matched number of exposures with corrective feedback for a total of 45 OLAs. Structural magnetic resonance imaging was performed in most participants, and medial temporal lobe volumetrics were acquired. Twenty-one healthy controls and 28 patients with aMCI were included in data analysis. Mnemonic strategy training was significantly more beneficial than matched exposure immediately after training, p = .006, partial η2 = .16, and at 1 month, p Mnemonic strategy-related improvement was correlated positively with baseline memory and executive functioning and negatively with inferior lateral ventricle volume in patients with aMCI; no significant relationships were evident in matched-exposure patients. Mnemonic strategies effectively improve memory for specific content for at least 1 month in patients with aMCI.

  6. Ultraviolet erythema as an indicator of nonspecific reactivity during hemorrhagic vasculitis in children

    Energy Technology Data Exchange (ETDEWEB)

    Kharitonova, A.V.

    1973-01-01

    From 1955 to 1967 studies were carried out on 407 children suffering from hemorrhagic vasculitis. For patients in the severe stage of hemorrhagic vasculitis it is characteristic that there are functional shifts in the central nervous system, which are manifested by a changed sensitivity of the skin to ultraviolet irradiation. The appearance of slowed, inert or paradoxal reactions indicates the predominance of processes in the brain that slow down and decrease the reactivity of the organism. In rheumatoid and abdominal-rheumatoid syndromes of the disease, disturbances in indices to skin light-sensitivity are the most manifest. During the recovery period, the indices of ultraviolet erythema, on approaching normal values, still remain low. This behavior requires extensive outpatient observation and treatment of sick children to avoid recidivism. 9 references. (SJR)

  7. Vasculitis assessment with [18F]F.D.G. positron emission tomography

    International Nuclear Information System (INIS)

    Liozon, E.; Monteil, J.

    2008-01-01

    [ 18 F]fluorodeoxyglucose ( 18 F.D.G.) positron emission tomography (PET) is a noninvasive metabolic imaging modality that is well suited to the assessment of activity and extent of large vessel vasculitis, such as giant cell arteritis and Takayasu arteritis. PET could be more effective than magnetic resonance imaging in detecting the earliest stages of vascular wall inflammation. The visual grading of vascular [ 18 F]F.D.G. uptake makes it possible to discriminate arteritis from atherosclerosis, providing therefore high specificity. High sensitivity can be achieved provided scanning is performed during active inflammatory phase, preferably before starting corticosteroid treatment. Large scale prospective studies are needed to determine the exact value of PET imaging in assessing the large vessel vasculitis outcome and response to immunosuppressive treatment

  8. Orbital phlebography for differentiation between multiple sclerosis and venous vasculitis in subacute blindness

    International Nuclear Information System (INIS)

    Hannerz, J.; Ericson, K.; Bergstrand, G.

    1988-01-01

    Thirteen consecutive patients with subacute unilateral loss of vision and periorbital pain but without pathology of the fundus or increased erythrocyte sedimentation rate, were investigated with visual evoked response, electrophoresis of serum and cerebrospinal fluid, and orbital phlebography. Seven of these patients were found to suffer from multiple sclerosis. The remaining 6 were considered to have venous vasculitis. There was a spontaneous recovery from visual impairment in all patients with multiple sclerosis, but not in patients with venous vasculitis. Of the latter patients, only two, who were treated with steroids within the first four days after onset of symptoms, regained vision. It appears that orbital phlebography is the diagnostic procedure of choice for proper management of patients with subacute loss of vision. (orig.)

  9. Perfusion and spectroscopy magnetic resonance imaging in a case of lymphocytic vasculitis mimicking brain tumor

    International Nuclear Information System (INIS)

    Muccio, Carmine Franco; Di Blasi, Arturo; Esposito, Gennaro; Brunese, Luca; D’Arco, Felice; Caranci, Ferdinando

    2013-01-01

    Lymphocytic vasculitis of the central nervous system is an uncommon subtype of primary angiitis of the central nervous system (PACNS) – a rare inflammatory disorder affecting parenchymal and leptomeningeal arteries and veins. Establishing diagnosis on the basis of neuroimaging only is difficult, as it can mimic a brain tumor. Thus, histological diagnosis is essential for appropriate management. We present a case of biopsy-proven lymphocytic vasculitis mimicking a brain tumor on neuroimaging that was subsequently successfully treated with steroid therapy. We also discuss the findings in perfusion MR (PWI) and MR spectroscopy (MRS). Regional hypoperfusion on PWI and elevation of glutamate and glutamine levels on MRS (without associated typical tumor spectra) are common findings in inflammatory disorders, including PACNS, and can be useful in differential diagnosis with tumors

  10. Renal sarcoidosis presenting as acute kidney injury with granulomatous interstitial nephritis and vasculitis.

    Science.gov (United States)

    Agrawal, Varun; Crisi, Giovanna M; D'Agati, Vivette D; Freda, Benjamin J

    2012-02-01

    Among the various renal manifestations of sarcoidosis, granulomatous inflammation confined to the tubulointerstitial compartment is the most commonly reported finding. We present the case of a 66-year-old man with acute kidney injury, hypercalcemia, mild restrictive pulmonary disease, and neurologic signs of parietal lobe dysfunction. Kidney biopsy showed diffuse interstitial inflammation with noncaseating granulomas that exhibited the unusual feature of infiltrating the walls of small arteries with destruction of the elastic lamina, consistent with granulomatous vasculitis. The findings of granulomatous interstitial nephritis on kidney biopsy, hypercalcemia, and possible cerebral and pulmonary involvement in the absence of other infectious, drug-induced, or autoimmune causes of granulomatous disease established the diagnosis of sarcoidosis. Pulse methylprednisolone followed by maintenance prednisone therapy led to improvement in kidney function, hypercalcemia, and neurologic symptoms. Vasculocentric granulomatous interstitial nephritis with granulomatous vasculitis is a rare and under-recognized manifestation of renal sarcoidosis. Copyright © 2012 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

  11. Cutaneous Necrotizing Vasculitis and Leukopenia in a Cocaine User: Is Levamisole the Culprit?

    Directory of Open Access Journals (Sweden)

    Lara El Khoury

    2016-01-01

    Full Text Available Levamisole is an antihelminthic drug banned by the US Food and Drug Administration (FDA in 2000 because of its dangerous side effects. Over the past few years, it has been identified as an adulterant in cocaine and reported to cause cutaneous vasculitis in cocaine users. The health burden of levamisole is serious since it is estimated that over 5 million Americans use cocaine and that 70% of the cocaine used in the USA contains levamisole. In this paper we report the case of a 23-year-old female cocaine user that presented with purpuric rash and skin necrosis, found to have positive c-ANCA and anti-proteinase 3 antibodies. Her skin biopsy showed fibroconnective tissue with signs of necrosis, acute and chronic inflammation, and thrombus formation. She was diagnosed with levamisole-induced vasculitis and successfully treated with withdrawal of cocaine use and local wound care.

  12. Dobutamine stress thallium-201 single-photon emission tomography versus echocardiography for evaluation of the extent and location of coronary artery disease late after myocardial infarction

    International Nuclear Information System (INIS)

    Elhendy, A.; Bax, J.J.; Domburg, R.T. van; Cornel, J.H.; Roelandt, J.R.T.C.; Valkema, R.; Reijs, A.E.M.; Krenning, E.P.

    1999-01-01

    Dobutamine stress echocardiography and thallium-201 myocardial perfusion scintigraphy are clinically useful methods for the evaluation of coronary artery disease (CAD). However, the relative merits of these imaging modalities in the evaluation of the extent of CAD after myocardial infarction have not been well studied. The aim of this study was to compare the accuracy of dobutamine stress echocardiography and simultaneous 201 Tl single-photon emission tomography (SPET) imaging for the diagnosis and localization of CAD late after acute myocardial infarction. Dobutamine (up to 40 μg kg -1 min -1 )-atropine (up to 1 mg) stress echocardiography in conjunction with stress-reinjection 201 Tl SPET was performed for the evaluation of myocardial ischaemia in 90 patients with previous myocardial infarction who underwent coronary angiography. Significant CAD was predicted on bases of myocardial ischemia (new or worsening wall motion abnormalities on echocardiography and reversible perfusion defects on 201 Tl SPET). Significant CAD (≥ 50% luminal diameter stenosis) was detected in 73 (81%) patients. The sensitivity, specificity and accuracy of echocardiography in detecting remote ischaemia for the diagnosis of remote CAD (present in 53 patients) were, respectively, 79% (CI 70%-88%), 85% (CI 77%-93%) and 81% (CI 73%-90%), while the corresponding figures for 201 Tl SPET were 75% (CI 66%-85%), 78% (CI 69%-87%) and 76% (CI 67%-86%) respectively (P = NS vs echocardiography). The sensitivity, specificity and accuracy of echocardiography in detecting peri-infarction ischaemia for the diagnosis of infarct-related artery stenosis (present in 70 patients) were, rspectively, 77% (CI 68%-86%), 85% (CI 78%-92%) and 79% (CI 70%-87%) while the corresponding figures for 201 Tl SPET were 73% (CI 64%-82%), 85% (CI 78%-92%) and 76% (CI 67%-84%) respectively (P = NS vs echocardiography). The agreement between the two methods for the diagnosis of peri-infarction and remote ischaemia was 70

  13. Postoperative spinal infection mimicking systemic vasculitis with titanium-spinal implants

    Directory of Open Access Journals (Sweden)

    Stathopoulos Konstantinos

    2011-09-01

    Full Text Available Abstract Background Secondary systemic vasculitis after posterior spinal fusion surgery is rare. It is usually related to over-reaction of immune-system, to genetic factors, toxicity, infection or metal allergies. Case Description A 14 year-old girl with a history of extended posterior spinal fusion due to idiopathic scoliosis presented to our department with diffuse erythema and nephritis (macroscopic hemuresis and proteinuria 5 months post surgery. The surgical trauma had no signs of inflammation or infection. The blood markers ESR and CRP were increased. Skin tests were positive for nickel allergy, which is a content of titanium alloy. The patient received corticosteroids systematically (hydrocortisone 10 mg for 6 months, leading to total recess of skin and systemic reaction. However, a palpable mass close to the surgical wound raised the suspicion of a late infection. The patient had a second surgery consisting of surgical debridement and one stage revision of posterior spinal instrumentation. Intraoperative cultures were positive to Staphylococcus aureus. Intravenous antibiotics were administered. The patient is now free of symptoms 24 months post revision surgery without any signs of recurrence of either vasculitis or infection. Literature Review Systemic vasculitis after spinal surgery is exceptionally rare. Causative factors are broad and sometimes controversial. In general, it is associated with allergy to metal ions. This is usually addressed with metal on metal total hip bearings. In spinal surgery, titanium implants are considered to be inert and only few reports have presented cases with systemic vasculitides. Therefore, other etiologies of immune over-reaction should always be considered, such as drug toxicity, infection, or genetic predisposition. Purposes and Clinical Relevance Our purpose was to highlight the difficulties during the diagnostic work-up for systemic vasculitis and management in cases of posterior spinal surgery.

  14. EULAR points to consider in the development of classification and diagnostic criteria in systemic vasculitis

    DEFF Research Database (Denmark)

    Basu, Neil; Watts, Richard; Bajema, Ingeborg

    2010-01-01

    The systemic vasculitides are multiorgan diseases where early diagnosis and treatment can significantly improve outcomes. Robust nomenclature reduces diagnostic delay. However, key aspects of current nomenclature are widely perceived to be out of date, these include disease definitions, classific......, classification and diagnostic criteria. Therefore, the aim of the present work was to identify deficiencies and provide contemporary points to consider for the development of future definitions and criteria in systemic vasculitis....

  15. Isolated periostitis as a manifestation of systemic vasculitis in a child: imaging features

    Energy Technology Data Exchange (ETDEWEB)

    Cheon, Jung-Eun; Kim, In-One; Kim, Woo Sun; Yeon, Kyung Mo [Seoul National University College of Medicine and the Institute of Radiation Medicine, Department of Radiology, 101 Daehang-ro Jongro-gu, Seoul (Korea, Republic of)

    2010-12-15

    We report a case of isolated periostitis associated with Takayasu arteritis in a 10-year-old boy presenting with calf pain. Radiographs revealed a localized, thick periosteal reaction with irregular margin in the proximal left fibula. MRI revealed irregular thickening with enhancement of the periosteum in the proximal fibula and heterogeneous enhancement along vascular bundles in the calf. Isolated periostitis is a rare skeletal manifestation of systemic vasculitis that could be misdiagnosed as neoplastic or traumatic periosteal reaction. (orig.)

  16. Comparability of patients with ANCA-associated vasculitis enrolled in clinical trials or in observational cohorts

    Science.gov (United States)

    Pagnoux, Christian; Carette, Simon; Khalidi, Nader A.; Walsh, Michael; Hiemstra, Thomas F.; Cuthbertson, David; Langford, Carol; Hoffman, Gary S.; Koening, Curry L.; Monach, Paul A.; Moreland, Larry; Mouthon, Luc; Seo, Phil; Specks, Ulrich; Ytterberg, Steven; Westman, Kerstin; Hoglund, Peter; Harper, Lorraine; Flossmann, Oliver; Luqmani, Raashid; Savage, Caroline; Rasmussen, Niels; de Groot, Kirstin; Tesar, Vladimir; Jayne, David; Merkel, Pater A.; Guillevin, Loic

    2015-01-01

    Objective To analyse the differences between patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) entered into randomised clinical trials (RCTs) and those followed in large observational cohorts. Methods The main characteristics and outcomes of patients with generalised and/or severe GPA or MPA with a five-factor score ≥1 enrolled in the French Vasculitis Study Group (FVSG) or the US-Canadian-based Vasculitis Clinical Research Consortium cohorts were compared to those enrolled in one of 2 FVSG clinical RCTs (WEG91, WEGENT) or 3 European Vasculitis Society clinical trials (CYCLOPS, CYCAZAREM, IMPROVE). Results 657 patients (65.3% with GPA) in RCTs were compared to 437 in cohorts (90.6% with GPA). RCT patients were older at diagnosis than the cohort patients (56.6±13.9 vs. 46.8±17.3 years), had higher Birmingham vasculitis activity score (19.5±9.1 vs. 16.9±7.4), and more frequent kidney disease (84.0% vs. 54.9%) but fewer ear, nose, and throat symptoms (56.8% vs. 72.2%). At 56 months post-diagnosis, mortality and relapse rates, adjusted for age and renal function, were higher for patients with GPA in RCTs vs. cohorts (10.7% vs. 2.5% [p=0.001] and 22.5% vs. 15.6% [p=0.03], respectively) but similar for patients with MPA (6.2% vs. 6.6% [p=0.92] and 16.6% vs. 10.1% [p=0.39], respectively). Conclusion Patients with GPA or MPA in RCTs and those in observational cohorts show important differences that should be remembered when interpreting results based on these study populations. PMID:26016754

  17. MR brain scanning in patients with vasculitis: Differentiation from multiple sclerosis

    International Nuclear Information System (INIS)

    Miller, D.H.; Ormerod, I.E.C.; Du Boulay, E.P.G.H.; Rudge, P.; McDonald, W.I.; Gibson, A.

    1987-01-01

    We performed MR (magnetic resonance) brain imaging on 24 patients with a systemic vasculitis. MRI proved to be a sensitive method for detecting brain lesions (clinically silent or manifest) in these patients. The most frequent abnormalities were periventricular lesions seen in 12 cases. Such changes are not specific for vascular disease, and are often seen in multiple sclerosis. However, additional changes were commonly seen which suggested the correct diagnosis. (orig.)

  18. Outcome and Treatment of Elderly Patients with ANCA-Associated Vasculitis

    Science.gov (United States)

    Goh, Su Mein; Mohammad, Aladdin J.; Hruskova, Zdenka; Tanna, Anisha; Bruchfeld, Annette; Selga, Daina; Chocova, Zdenka; Westman, Kerstin; Eriksson, Per; Pusey, Charles D.; Tesar, Vladimir; Salama, Alan D.; Segelmark, Mårten

    2015-01-01

    Background and objectives ANCA-associated vasculitis is commonly found in elderly patients, but there are few data concerning outcome and treatment in the highest age groups. Design, setting, participants, & measurements Consecutive patients (N=151) presenting between 1997 and 2009 were retrospectively included from local registries in six centers in Sweden, the United Kingdom, and the Czech Republic if diagnosed with microscopic polyangiitis or granulomatosis with polyangiitis at age ≥75 years during the study period. Patients were followed until 2 years from diagnosis or death. Data on survival and renal function were analyzed with respect to age, sex, ANCA specificity, renal function, C-reactive protein, comorbidities, and Birmingham Vasculitis Activity Score at diagnosis as well as treatment during the first month. Results Median follow-up was 730 days (interquartile range, 244–730). Overall 1-year survival was 71.5% and 2-year survival was 64.6%. Older age, higher creatinine, and lower Birmingham Vasculitis Activity Score were associated with higher mortality in multivariable analysis. Patients who were not treated with standard immunosuppressive therapy had significantly worse survival. Renal survival was 74.8% at 1 year. No new cases of ESRD occurred during the second year. High creatinine at diagnosis was the only significant predictor of renal survival in multivariable analysis. Conclusions ANCA-associated vasculitis is a disease with substantial mortality and morbidity among elderly patients. This study showed a better prognosis for those who received immunosuppressive treatment and those who were diagnosed before having developed advanced renal insufficiency. PMID:26100457

  19. Circulating C3 levels predict renal and global outcome in patients with renal vasculitis.

    Science.gov (United States)

    Villacorta, Javier; Diaz-Crespo, Francisco; Acevedo, Mercedes; Cavero, Teresa; Guerrero, Carmen; Praga, Manuel; Fernandez-Juarez, Gema

    2016-11-01

    Several studies have demonstrated the crucial role of complement activation in the pathogenesis of ANCA-associated vasculitis. We aimed to assess the association between baseline serum C3 (sC3) levels and long-term outcomes in patients with renal vasculitis. This retrospective study included 111 patients with renal vasculitis from three hospitals who underwent a renal biopsy between 1997 and 2014. Serum levels of C3 were measured at the onset and the study population was divided into three tertiles according to sC3 concentrations (tertile 1 128 mg/dl). Patients with lower sC3 (tertile 1) were compared with those having higher levels of sC3 (tertile 2 and tertile 3). Histological, clinical, and laboratory data were recorded for analysis. The primary end point was the composite of end-stage renal disease (ESRD) and death from any cause. Lower sC3 levels were associated with a higher need for dialysis and lower response rate to treatment (p = 0.04 and p = 0.007, respectively). Renal and global survival at 1 and 5 years was 53 and 46 % in patients with lower sC3 (tertile 1) compared with 72 and 65 % in patients with higher sC3 (upper two tertiles) (p = 0.04). In a multivariate Cox-regression model, when adjusted by renal function and histopatholologic categories, lower sC3 remained as an independent predictor of ESRD and death (HR, 1.9; 95 % CI, 1.1 to 3.4; p = 0.02). Baseline serum C3 levels have an independent prognostic value in predicting long-term renal and global survival in patients with renal vasculitis.

  20. Deferasirox-induced urticarial vasculitis in a patient with myelodysplastic syndrome

    OpenAIRE

    Polat, Asude Kara; Belli, Asli Akin; Karakus, Volkan; Dere, Yelda

    2017-01-01

    Abstract Deferasirox is an iron chelator agent used in the treatment of diseases with iron overload, such as thalassemia and myelodysplastic syndrome. Although the majority of adverse reactions of deferasirox involve gastrointestinal symptoms and increase in serum creatinine and transaminases, skin rashes, such as maculopapular and urticarial eruptions, have also been reported. This study reports a case of myelodysplastic syndrome with urticarial vasculitis due to deferasirox therapy. Drug er...

  1. [Prostatic vasculitis, an unusual onset of panarteritis nodosa: presentation of 2 cases].

    Science.gov (United States)

    Corralés Torres, A J; el Amrani, A; Aranegui Lasuen, P; Rivera Cívico, F; Arrebola Nacle, J P; Jiménez-Alonso, J

    1993-06-01

    Prostatic involvement in nodose panarteritis is considered as exceptional, especially as the onset of the disease. We discuss two cases which made their debut with fever, weight loss and urinary symptomatology, simulating a prostatic neoplasia. The pathology study of the ressected prostatic sample showed in both cases a necrotizing vasculitis which yielded the diagnosis of nodose panarteritis, and to establish the specific treatment with steroids an immunosuppressants.

  2. Hypocomplementemic urticarial vasculitis arising in a patient with immunoglobulin G4-related disease.

    Science.gov (United States)

    Takao, Mariko; Hamada, Toshihisa; Kaji, Tatsuya; Ikeda-Mizuno, Kazuko; Takehara-Yasuhara, Chinatsu; Ichimura, Kouichi; Yanai, Hiroyuki; Yshino, Tadashi; Iwatsuki, Keiji

    2016-04-01

    Hypocomplementemic urticarial vasculitis (HUV) has been defined as a syndrome associated with urticarial lesions caused by leukocytoclastic vasculitis. It has been observed in patients with systemic lupus erythematosus and related conditions. Immunoglobulin G4 (IgG4)-related disease is a lymphoproliferative disorder characterized by sclerosing pancreatitis, retroperitoneal fibrosis, sclerotic cholangitis, acute interstitial nephritis, and Mikulicz's disease, and associated with elevated levels of IgG4 and hypocomplementemia. Various cutaneous lesions may occur in IgG4-related disease. We report a 74-year-old woman with HUV, followed by systemic organ involvement suggestive of IgG4-related disease. Symptoms included submandibular gland swelling, lymphadenopathy, interstitial nephritis, and a thyroid nodule. Laboratory examinations revealed an elevated serum IgG4 level of 637 mg/dl in a total IgG level of 2176 mg/dl, and the production of autoantibodies such as rheumatoid factor and antiphospholipid antibodies. In the swollen lymph node, neither clonal proliferation of T or B cells nor Epstein-Barr virus infection was detected, although the structure had been destroyed by the infiltration of lymphocytes and plasma cells. Among total IgG-producing cells, approximately 40% were positive for IgG4. All symptoms and abnormal laboratory findings improved in response to oral prednisolone. Hypocomplementemic urticarial vasculitis may represent a clinical symptom of IgG4-related disease. © 2015 The International Society of Dermatology.

  3. Varicella Zoster Virus and Large Vessel Vasculitis, the Absence of an Association

    Science.gov (United States)

    Procop, Gary W.; Eng, Charis; Clifford, Alison; Villa-Forte, Alexandra; Calabrese, Leonard H.; Roselli, Eric; Svensson, Lars; Johnston, Douglas; Pettersson, Gosta; Soltesz, Edward; Lystad, Lisa; Perry, Julian D.; Blandford, Alexander; Wilson, Deborah A.; Hoffman, Gary S.

    2017-01-01

    Objective It is controversial whether microorganisms play a role in the pathogenesis of large and medium vessel vasculitides (eg, giant cell arteritis [GCA], Takayasu arteritis [TAK] and focal idiopathic aortitis [FIA]). Recent studies have reported the presence of Varicella Zoster Virus (VZV) within formalin-fixed, paraffin-embedded temporal arteries and aortas of about three-quarters or more of patients with these conditions, and in a minority of controls. In a prospective study, we sought to confirm these findings using DNA extracted from vessels that were harvested under surgically aseptic conditions and snap frozen. Methods and Results DNA samples extracted from 11 surgically sterile temporal arteries and 31 surgically sterile thoracic aortas were used in an attempt to identify the vessel-associated VZV genome. Two different validated PCR methods were used. Thirty-one thoracic aorta aneurysm specimens included biopsies from 8 patients with GCA, 2 from patients with TAK, 6 from patients with FIA, and 15 from patients without vasculitis, who had non-inflammatory aneurysms. Eleven temporal artery biopsies were collected from 5 patients with GCA and 6 controls. The presence of VZV was not identified in either the specimens from patients with large vessel vasculitis or from the controls. Conclusions Using surgically sterile snap-frozen specimens, we were unable to confirm recent reports of the presence of VZV in either aortas or temporal arteries from patients with large vessel vasculitis or controls. PMID:28758156

  4. An 'inflammatory' variant of solar purpura: a simulant of leukocytoclastic vasculitis and neutrophilic dermatoses.

    Science.gov (United States)

    Wood, Benjamin A; LeBoit, Philip E

    2013-08-01

    To study the clinical and pathological features of cases of apparent solar purpura, with attention to the recently described phenomenon of inflammatory changes within otherwise typical lesions. We studied 95 cases diagnosed as solar purpura and identified 10 cases (10.5%) in which significant neutrophilic inflammation was present, potentially simulating a leukocytoclastic vasculitis or neutrophilic dermatosis. An additional three cases were identified in subsequent routine practice. The clinical features, including follow-up for subsequent development of vasculitis and histological features were studied. In all cases the histological features were typical of solar purpura, with the exception of inflammatory changes, typically associated with clefting of elastotic stroma. Clinical follow-up information was available for all patients and none developed subsequent evidence of a cutaneous or systemic vasculitis or neutrophilic dermatosis. Inflammatory changes appear to be more frequent in solar purpura than is generally recognised. Awareness of this histological variation and correlation with the clinical findings and evolution is important in avoiding misdiagnosis.

  5. Kidney Lesions Associated with Systemic Vasculitis: Report of 25 Cases in Morocco

    Directory of Open Access Journals (Sweden)

    Abdou Niang

    2001-01-01

    Full Text Available In this retrospective study, we report 25 patients with renal injury caused by systemic vasculitis. These patients were hospitalized at the department of nephrology-hemodialysis in Ibn Rochd hospital from 1985 to 1998. The mean age of the patients was 36 years (range 3 to 57 years with male predominance (68%. The clinical presentations included cutaneous purpura in 80% of the patients, arthritis in 60% and pulmonary hemorrhage in 20%. The renal injury manifested as abnormal urinary sediment in 20 of the study patients (80%, nephritic syndrome in 14 (56%, renal failure in nine (36%. Three patients (12% had renal failure as the initial presentation followed by the other manifestations of vasculitis. The anti-neutrophil cytoplasmic antibodies (ANCA have been checked in only six patients of whom four were positive. Henoch-Schonlein purpura was the most common clinical sign in 11 patients (44% followed by Behcet′s disease in five (20%. The treatment was variable in type and duration but generally included corticoids alone or in combination with immunosuppressants. The prognosis of the renal injury was variable. The renal function remained stable in those with Henoch-Schonlein purpura, but was less favorable with the other etiologies with more predominance of renal failure and end-stage renal disease. This retrospective analysis of our experience is given to throw light on the pattern of vasculitis in our region.

  6. Eosinophilic Granulomatosis with Polyangiitis Presenting with Skin Rashes, Eosinophilic Cholecystitis, and Retinal Vasculitis.

    Science.gov (United States)

    Zeng, Mingbing; Liu, Xialin; Liu, Yizhi

    2016-11-18

    BACKGROUND Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome (CSS), is a rare vasculitis of unknown etiology. Most of the patients have a long history of asthma and then develop autoimmune inflammation of small and medium-sized blood vessels, with consequent reduction of blood flow to various organs and tissues. EGPA can cause disorders in multiple systems; the most seriously affected organs are the retina, kidney, brain, cardiovascular system, and skin. CASE REPORT The patient was hospitalized for high fever and skin rashes and then developed right upper abdominal pain, decreased visual acuity, coma, and convulsions. Laboratory investigations showed marked eosinophilia (9412/mm3). Following cholecystectomy, histopathological examination revealed a marked inflammatory cell infiltrate composed mainly of eosinophils. Retinal vasculitis and medium and peripheral vascular closure were confirmed by fundus fluorescence angiography (FFA). The coma and convulsions were controlled successfully by high-dose methylprednisolone. After gradual tapering of the methylprednisolone, the patient's blood count recovered to a normal level, and the other systematic disorders disappeared; however, she was left with irreversible blindness. CONCLUSIONS EGPA can cause eosinophilic cholecystitis, retinal vasculitis, and neuropathy in the short term and calls for effective treatments in order to avoid binocular blindness.

  7. Phospholipid Syndrome and Vasculitis as a presentation of Systemic Lupus Erythematosus. Case report.

    Directory of Open Access Journals (Sweden)

    Sila Castellón Mortera

    2013-09-01

    Full Text Available The systemic Lupus Erythematosus is presented, generally, as a poli articular syndrome, with a long period of fever nephritico or nephrotico; other clinical ways are: neuropsychiatry, vasculitis, etc. They appeared in a progressive manner; but in rare cases as a sickness debutant. It has not being reported in Sancti Spiritus Province patients in which matches the debut of the systemic Lupus Erythematosus with the manifestations of phospholipid syndrome. A Woman with 24 years of age is hospitalized having vasculitis, articular pains, thrombose in her right foot, detecting anticoagulante lupico and possitive Rematoideo factor with periferic pattern diffused in the Inmunoelectroforesis. 5 years later was hospitalized again with poliserositis. She had a positive evolution with a dose in a month of Intacglobin and anticoagulante treatment. Two years later she was hospitalized with articular pains proving she had livedo reticular on her left knee and Raynaud phenomenon on her foot. Beta Prebeta Index and high triglycerides. Lupico anticoagulant positive again. A treatment with Intacglobin and Prednisona was given to the patient with a better clinic without being hospitalized again. There is no evidence (at 17 years of age of a sickness debut of renal dissorder. It is about a Systemic Lupus Eritematoso which debut was a vasculitis and a Phospholipid Syndrome associated.

  8. Imaging large vessel vasculitis with fully integrated PET/MRI: a pilot study

    International Nuclear Information System (INIS)

    Einspieler, Ingo; Pyka, Thomas; Eiber, Matthias; Thuermel, Klaus; Wolfram, Sabine; Moog, Philipp; Reeps, Christian; Essler, Markus

    2015-01-01

    The aim of this study was to evaluate the feasibility of hybrid [ 18 F]fluorodeoxyglucose (FDG) positron emission tomography (PET)/MRI in patients with large vessel vasculitis (LVV) by comparing visual and quantitative parameters to that of PET/CT. Furthermore, the value of PET/MRI in disease activity and extent of LVV was assessed. A total of 16 [ 18 F]FDG PET/MRI and 12 [ 18 F]-FDG PET/CT examinations were performed in 12 patients with LVV. MRI of the vessel wall by T1-weighted and T2-weighted sequences was used for anatomical localization of FDG uptake and identification of morphological changes associated with LVV. In addition, contrast-enhanced (CE) magnetic resonance angiography (MRA) was performed. The vascular FDG uptake in the vasculitis group was compared to a reference group of 16 patients using a four-point visual score. Visual scores and quantitative parameters [maximum standardized uptake value (SUV max ) and target to background ratio (TBR)] were compared between PET/MRI and PET/CT. Furthermore, correlations between C-reactive protein (CRP) and quantitative PET results, as well the extent of vasculitis in PET, MRI/CE-MRA and combined PET/MRI, were analysed. TBRs, SUV max values and visual scores correlated well between PET/MRI and PET/CT (r = 0.92, r = 0.91; r = 0.84, p < 0.05). There was no significant difference between both modalities concerning SUV max measurements and visual scores. In PET/MRI, PET alone revealed abnormal FDG uptake in 86 vascular regions. MRI/CE-MRA indicated 49 vessel segments with morphological changes related to vasculitis, leading to a total number of 95 vasculitis regions in combination with PET. Strong and significant correlations between CRP and disease extent in PET alone (r = 0.75, p = 0.0067) and PET/MRI (r = 0.92, p < 0.0001) in contrast to MRI/CE-MRA only were observed. Regarding disease activity, no significant correlations were seen between quantitative PET results and CRP, although there was a trend towards

  9. Imaging large vessel vasculitis with fully integrated PET/MRI: a pilot study

    Energy Technology Data Exchange (ETDEWEB)

    Einspieler, Ingo; Pyka, Thomas; Eiber, Matthias [Technische Universitaet Muenchen, Klinikum rechts der Isar, Department of Nuclear Medicine, Munich (Germany); Thuermel, Klaus; Wolfram, Sabine; Moog, Philipp [Technische Universitaet Muenchen, Klinikum rechts der Isar, Department of Nephrology, Munich (Germany); Reeps, Christian [Technische Universitaet Muenchen, Department of Vascular Surgery, Klinikum rechts der Isar, Munich (Germany); Essler, Markus [Rheinische Friedrich-Wilhelms-Universitaet, Department of Nuclear Medicine, Universitaetsklinikum Bonn, Bonn (Germany)

    2015-04-16

    The aim of this study was to evaluate the feasibility of hybrid [{sup 18}F]fluorodeoxyglucose (FDG) positron emission tomography (PET)/MRI in patients with large vessel vasculitis (LVV) by comparing visual and quantitative parameters to that of PET/CT. Furthermore, the value of PET/MRI in disease activity and extent of LVV was assessed. A total of 16 [{sup 18}F]FDG PET/MRI and 12 [{sup 18}F]-FDG PET/CT examinations were performed in 12 patients with LVV. MRI of the vessel wall by T1-weighted and T2-weighted sequences was used for anatomical localization of FDG uptake and identification of morphological changes associated with LVV. In addition, contrast-enhanced (CE) magnetic resonance angiography (MRA) was performed. The vascular FDG uptake in the vasculitis group was compared to a reference group of 16 patients using a four-point visual score. Visual scores and quantitative parameters [maximum standardized uptake value (SUV{sub max}) and target to background ratio (TBR)] were compared between PET/MRI and PET/CT. Furthermore, correlations between C-reactive protein (CRP) and quantitative PET results, as well the extent of vasculitis in PET, MRI/CE-MRA and combined PET/MRI, were analysed. TBRs, SUV{sub max} values and visual scores correlated well between PET/MRI and PET/CT (r = 0.92, r = 0.91; r = 0.84, p < 0.05). There was no significant difference between both modalities concerning SUV{sub max} measurements and visual scores. In PET/MRI, PET alone revealed abnormal FDG uptake in 86 vascular regions. MRI/CE-MRA indicated 49 vessel segments with morphological changes related to vasculitis, leading to a total number of 95 vasculitis regions in combination with PET. Strong and significant correlations between CRP and disease extent in PET alone (r = 0.75, p = 0.0067) and PET/MRI (r = 0.92, p < 0.0001) in contrast to MRI/CE-MRA only were observed. Regarding disease activity, no significant correlations were seen between quantitative PET results and CRP, although there

  10. Monocyte chemoattractant protein-1 promoter -2518 polymorphism and susceptibility to vasculitis, rheumatoid arthritis, and multiple sclerosis: A meta-analysis.

    Science.gov (United States)

    Lee, Y H; Bae, S-C

    2016-03-20

    The purpose of this study was to examine whether the monocyte chemoattractant protein-1 (MCP-1) promoter -2518 A/G polymorphism (rs1024611) is associated with susceptibility to vasculitis, rheumatoid arthritis (RA), or multiple sclerosis (MS). A meta-analysis was conducted on the association between the MCP-1 -2518 A/G polymorphism and vasculitis, RA, and MS. Fourteen studies from 13 articles, including six on vasculitis, five on RA, and three on MS, consisting of 3,038 patients and 3,545 controls were available for the meta-analysis. The meta-analysis revealed no association between the MCP-1 -2518 G allele and vasculitis (odds ratio [OR] = 0.990, 95% confidence interval [CI] = 0.749-1.309, p = 0.943). Stratification by ethnicity indicated no association between the G allele of the MCP-1 -2518 A/G polymorphism and vasculitis in Asians and Caucasians. Meta-analysis by vasculitis type revealed an association between the GG+GA genotype of the MCP-1 -2518 A/G polymorphism and Behçet's disease (BD; OR = 1.349, 95% CI = 1.013-1.796, p = 0.040). However, sensitivity analysis showed that the association was not statistically significant after removing a study that was conducted in China (OR = 1.030, 95% CI = 0.667-1.590, p = 0.895), which indicated that the association was not statistically robust. The meta-analysis revealed no association between the MCP-1 -2518 G allele and RA (OR = 0.986, 95% CI = 0.890-1.093, p = 0.793) or MS (OR = 1.281, 95% CI = 0.802-2.046, p = 0.301). Our meta-analysis demonstrates that the MCP-1 -2518 A/G polymorphism is not associated with susceptibility to vasculitis, RA, or MS.

  11. Location of Urban Logistic Terminals as Hub Location Problem

    Directory of Open Access Journals (Sweden)

    Jasmina Pašagić Škrinjar

    2012-09-01

    Full Text Available In this paper the problems of locating urban logistic terminals are studied as hub location problems that due to a large number of potential nodes in big cities belong to hard non-polynomial problems, the so-called NP-problems. The hub location problems have found wide application in physical planning of transport and telecommunication systems, especially systems of fast delivery, networks of logistic and distribution centres and cargo traffic terminals of the big cities, etc. The paper defines single and multiple allocations and studies the numerical examples. The capacitated single allocation hub location problems have been studied, with the provision of a mathematical model of selecting the location for the hubs on the network. The paper also presents the differences in the possibilities of implementing the exact and heuristic methods to solve the actual location problems of big dimensions i.e. hub problems of the big cities.

  12. Necrotizing sialometaplasia: Manifestation of a localized unclassified vasculitis

    Directory of Open Access Journals (Sweden)

    Swagatika Senapati

    2016-01-01

    Full Text Available Necrotizing sialometaplasia is a rare benign and self-limiting disease, which commonly affects the minor salivary glands. Typically, it involves the seromucinous glands located at palate, buccal mucosa, tongue, tonsil, nasal cavity, trachea, larynx, maxillary sinus, and retromolar trigone. We report two such cases of necrotizing sialometaplasia to create awareness among the pathologists and surgeons because of its close morphological and clinical resemblance to squamous cell carcinoma. We have also documented that, the ischemic necrosis of salivary gland is the result of a vasculitic process.

  13. Hull properties in location problems

    DEFF Research Database (Denmark)

    Juel, Henrik; Love, Robert F.

    1983-01-01

    Some properties of the solution set for single and multifacility continuous location problems with lp distances are given. A set reduction algorithm is developed for problems in k-dimensional space having rectangular distances.......Some properties of the solution set for single and multifacility continuous location problems with lp distances are given. A set reduction algorithm is developed for problems in k-dimensional space having rectangular distances....

  14. The Effect of Elevated CO2 and Increased Temperature on in Vitro Fertilization Success and Initial Embryonic Development of Single Male:Female Crosses of Broad-Cast Spawning Corals at Mid- and High-Latitude Locations

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    Miriam Schutter

    2015-05-01

    Full Text Available The impact of global climate change on coral reefs is expected to be most profound at the sea surface, where fertilization and embryonic development of broadcast-spawning corals takes place. We examined the effect of increased temperature and elevated CO2 levels on the in vitro fertilization success and initial embryonic development of broadcast-spawning corals using a single male:female cross of three different species from mid- and high-latitude locations: Lyudao, Taiwan (22° N and Kochi, Japan (32° N. Eggs were fertilized under ambient conditions (27 °C and 500 μatm CO2 and under conditions predicted for 2100 (IPCC worst case scenario, 31 °C and 1000 μatm CO2. Fertilization success, abnormal development and early developmental success were determined for each sample. Increased temperature had a more profound influence than elevated CO2. In most cases, near-future warming caused a significant drop in early developmental success as a result of decreased fertilization success and/or increased abnormal development. The embryonic development of the male:female cross of A. hyacinthus from the high-latitude location was more sensitive to the increased temperature (+4 °C than the male:female cross of A. hyacinthus from the mid-latitude location. The response to the elevated CO2 level was small and highly variable, ranging from positive to negative responses. These results suggest that global warming is a more significant and universal stressor than ocean acidification on the early embryonic development of corals from mid- and high-latitude locations.

  15. New aspects of MRI for diagnostics of large vessel vasculitis and primary angiitis of the central nervous system

    International Nuclear Information System (INIS)

    Saam, T.; Habs, M.; Cyran, C.C.; Grimm, J.; Reiser, M.F.; Nikolaou, K.; Pfefferkorn, T.; Schueller, U.

    2010-01-01

    Vasculitis is a rare disease and clinical symptoms are often unspecific. Accurate and early diagnosis is mandatory in order to prevent complications, such as loss of vision or stroke. Imaging techniques can contribute to establishing a definite diagnosis and to evaluate disease activity and the extent of the disease in various vascular regions. Conventional imaging methods, such as computed tomography (CT) and magnetic resonance (MR) angiography, as well as digital subtraction angiography allow the vessel lumen but not the vessel wall to be depicted. However, vasculitis is a disease which primarily affects the vessel wall, therefore conventional imaging modalities often fail to make a definite diagnosis. Recently black-blood high resolution MR in vivo imaging has been used to visualize cervical and intracranial vasculitis. This review article presents imaging protocols for intracranial and cervical black-blood MR imaging and clinical cases with large vessel vasculitis and vasculitis of the central nervous system. Furthermore the current literature, examples of the most common differential diagnoses of cervical and cranial arteriopathy and the potential of other imaging modalities, such as PET/CT and ultrasound will be discussed. (orig.) [de

  16. Fulminant Vasculitis Associated with Extracranial Dissections and Occlusion, Ischemic Strokes, and Aneurysm Rupture: Case Report and Review of the Literature.

    Science.gov (United States)

    Jamil, Osama; Taussky, Philipp; Schmidt, Richard H; Park, Min S

    2016-07-01

    Central nervous system vasculitis has multiple presentations, including stroke, seizures, cranial nerve palsies, and encephalopathy. We present the case of an unresponsive 45-year-old woman with vasculitis associated with fulminant intracranial vessel dissection and occlusion with ischemic strokes and subarachnoid hemorrhage secondary to aneurysm rupture. Imaging studies demonstrated both ischemic and hemorrhagic strokes. She had a ruptured right internal carotid artery dorsal variant aneurysm, right vertebral artery dissection with occlusion and posterior inferior cerebellar artery infarct, left vertebral artery dissection, and severe vasculitis involving intracranial and extracranial vessels. She initially was treated for her vasculitis with high-dose steroids followed by clip wrapping of the dorsal variant aneurysm. Unfortunately, her surgery was complicated by intraprocedural rupture, and the patient died during her hospitalization. Fulminant central nervous system vasculitis can occur with critical vascular anomalies that require emergent intervention and should be part of the differential diagnosis of patients presenting with these multiple vascular pathologies. Copyright © 2016 Elsevier Inc. All rights reserved.

  17. Anti-proteinase 3 anti-neutrophil cytoplasm autoantibodies recapitulate systemic vasculitis in mice with a humanized immune system.

    LENUS (Irish Health Repository)

    Little, Mark A

    2012-01-01

    Evidence is lacking for direct pathogenicity of human anti-proteinase-3 (PR3) antibodies in development of systemic vasculitis and granulomatosis with polyangiitis (GPA, Wegener\\'s granulomatosis). Progress in study of these antibodies in rodents has been hampered by lack of PR3 expression on murine neutrophils, and by different Fc-receptor affinities for IgG across species. Therefore, we tested whether human anti-PR3 antibodies can induce acute vasculitis in mice with a human immune system. Chimeric mice were generated by injecting human haematopoietic stem cells into irradiated NOD-scid-IL2Rγ⁻\\/⁻ mice. Matched chimera mice were treated with human IgG from patients with: anti-PR3 positive renal and lung vasculitis; patients with non-vasculitic renal disease; or healthy controls. Six-days later, 39% of anti-PR3 treated mice had haematuria, compared with none of controls. There was punctate bleeding on the surface of lungs of anti-PR3 treated animals, with histological evidence of vasculitis and haemorrhage. Anti-PR3 treated mice had mild pauci-immune proliferative glomerulonephritis, with infiltration of human and mouse leukocytes. In 3 mice (17%) more severe glomerular injury was present. There were no glomerular changes in controls. Human IgG from patients with anti-PR3 autoantibodies is therefore pathogenic. This model of anti-PR3 antibody-mediated vasculitis may be useful in dissecting mechanisms of microvascular injury.

  18. Histopathology of the Inner Ear in a Case With Recent Onset of Cogan's Syndrome: Evidence for Vasculitis.

    Science.gov (United States)

    Jung, David H; Nadol, Joseph B; Folkerth, Rebecca D; Merola, Joseph F

    2016-01-01

    The association of sensorineural hearing loss and vertigo with inflammatory eye disease, usually interstitial keratitis, has been called Cogan's syndrome. The pathogenesis of Cogan's syndrome is unknown, but it has been assumed to be an immune mediated disorder with vasculitis. The histopathology of the inner ear in Cogan's syndrome has been described in 6 case reports. Although common pathologic findings in these reports include degeneration of the auditory and vestibular neuroepithelium, endolymphatic hydrops, fibrosis, and new bone formation, direct pathologic evidence of a vasculitis has not been published. A possible reason for this failure to identify vasculitis was a substantial delay (range, 4-40 years) between the onset of symptoms and examination of the otopathology. In the current case report, the patient had both auditory and vestibular symptoms and interstitial keratitis with a time delay of only 2 to 4 weeks between symptoms and death. Evidence of a vasculitis as a possible underlying etiology included H&E histopathology and anti-CD45 immunostaining of vessels both in the auditory and vestibular systems, supporting the hypothesis of a vasculitis as a mechanism in this disorder. © The Author(s) 2015.

  19. Anti-proteinase 3 anti-neutrophil cytoplasm autoantibodies recapitulate systemic vasculitis in mice with a humanized immune system.

    Directory of Open Access Journals (Sweden)

    Mark A Little

    Full Text Available Evidence is lacking for direct pathogenicity of human anti-proteinase-3 (PR3 antibodies in development of systemic vasculitis and granulomatosis with polyangiitis (GPA, Wegener's granulomatosis. Progress in study of these antibodies in rodents has been hampered by lack of PR3 expression on murine neutrophils, and by different Fc-receptor affinities for IgG across species. Therefore, we tested whether human anti-PR3 antibodies can induce acute vasculitis in mice with a human immune system. Chimeric mice were generated by injecting human haematopoietic stem cells into irradiated NOD-scid-IL2Rγ⁻/⁻ mice. Matched chimera mice were treated with human IgG from patients with: anti-PR3 positive renal and lung vasculitis; patients with non-vasculitic renal disease; or healthy controls. Six-days later, 39% of anti-PR3 treated mice had haematuria, compared with none of controls. There was punctate bleeding on the surface of lungs of anti-PR3 treated animals, with histological evidence of vasculitis and haemorrhage. Anti-PR3 treated mice had mild pauci-immune proliferative glomerulonephritis, with infiltration of human and mouse leukocytes. In 3 mice (17% more severe glomerular injury was present. There were no glomerular changes in controls. Human IgG from patients with anti-PR3 autoantibodies is therefore pathogenic. This model of anti-PR3 antibody-mediated vasculitis may be useful in dissecting mechanisms of microvascular injury.

  20. Bronchial lesions of mouse model of asthma are preceded by immune complex vasculitis and induced bronchial associated lymphoid tissue (iBALT).

    Science.gov (United States)

    Guest, Ian C; Sell, Stewart

    2015-08-01

    We systematically examined by immune histology the lungs of some widely used mouse models of asthma. These models include sensitization by multiple intraperitoneal injections of soluble ovalbumin (OVA) or of OVA with alum, followed by three intranasal or aerosol challenges 3 days apart. Within 24 h after a single challenge there is fibrinoid necrosis of arterial walls with deposition of immunoglobulin (Ig) and OVA and infiltration of eosinophilic polymorphonuclear cells that lasts for about 3 days followed by peribronchial B-cell infiltration and slight reversible goblet cell hypertrophy (GCHT). After two challenges, severe eosinophilic vasculitis is present at 6 h, increases by 72 h, and then declines; B-cell proliferation and significant GCHT and hyperplasia (GCHTH) and bronchial smooth muscle hypertrophy recur more prominently. After three challenges, there is significantly increased induced bronchus-associated lymphoid tissue (iBALT) formation, GCHTH, and smooth muscle hypertrophy. Elevated levels of Th2 cytokines, IL-4, IL-5, and IL-13, are present in bronchial lavage fluids. Sensitized mice have precipitating antibody and positive Arthus skin reactions but also develop significant levels of IgE antibody to OVA but only 1 week after challenge. We conclude that the asthma like lung lesions induced in these models is preceded by immune complex-mediated eosinophilic vasculitis and iBALT formation. There are elevations of Th2 cytokines that most likely produce bronchial lesions that resemble human asthma. However, it is unlikely that mast cell-activated atopic mechanisms are responsible as we found only a few presumed mast cells by toluidine blue and metachromatic staining limited to the most proximal part of the main stem bronchus, and none in the remaining main stem bronchus or in the lung periphery.

  1. Traumatic endophthalmitis presenting as isolated retinal vasculitis and white-centered hemorrhages: Case report and review of literature

    Directory of Open Access Journals (Sweden)

    Nidhi Relhan

    2012-01-01

    Full Text Available The article reports a case and review of the literature of endophthalmitis presenting as isolated retinal vasculitis. A 26-year-old male was observed to have white-centered retinal hemorrhages and retinal vasculitis following an occult scleral perforation. At presentation, the visual acuity was 20/60. With clinical suspicion of early endophthalmitis, he underwent wound exploration, scleral tear repair, vitreous biopsy and administration of intravitreal antibiotics. Microbiology evaluation revealed significant presence of methicillin-resistant coagulase-negative Staphylococcus epidermidis. Final visual acuity improved to 20/20 at 6 weeks postoperatively. Literature search revealed eight similar cases, all of them due to Staphylococcus species. Retinal vasculitis and white-centered retinal hemorrhages can be a presenting sign of early endophthalmitis, especially with non-fulminant pathogens like S. epidermidis.

  2. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial

    DEFF Research Database (Denmark)

    de Groot, Kirsten; Harper, Lorraine; Jayne, David R W

    2009-01-01

    BACKGROUND: Current therapies for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis are limited by toxicity. OBJECTIVE: To compare pulse cyclophosphamide with daily oral cyclophosphamide for induction of remission. DESIGN: Randomized, controlled trial. Random assignments were...... generalized ANCA-associated vasculitis with renal involvement but not immediately life-threatening disease. INTERVENTION: Pulse cyclophosphamide, 15 mg/kg every 2 to 3 weeks (76 patients), or daily oral cyclophosphamide, 2 mg/kg per day (73 patients), plus prednisolone. MEASUREMENT: Time to remission (primary...... regimen induced remission of ANCA-associated vasculitis as well as the daily oral regimen at a reduced cumulative cyclophosphamide dose and caused fewer cases of leukopenia. PRIMARY FUNDING SOURCE: The European Union....

  3. Fulminant systemic vasculitis in systemic lupus erythematosus. Case report and review of the literature.

    Science.gov (United States)

    Medina, G; González-Pérez, D; Vázquez-Juárez, C; Sánchez-Uribe, M; Saavedra, M A; Jara, L J

    2014-11-01

    Vasculitis in systemic lupus erythematosus (SLE) has a broad spectrum of clinical manifestations from cutaneous to visceral involvement and its prognosis ranges from mild to life-threatening. We report the case of a previously healthy 17-year-old woman with eight months' history of arthralgias and myalgias. Subsequently, she developed facial and lower limbs edema, and hair loss. Two weeks before admission to a secondary level hospital, she developed fever up to 40°C followed by abdominal pain, rectal bleeding, hematemesis and blisters on both legs, reason for which she was hospitalized. With active bullous SLE with rapidly progressive glomerulonephritis suspected, she was treated with methylprednisolone pulses without response. After one week of treatment, she was transferred to a tertiary level hospital. On admission she presented acute arterial insufficiency of the lower extremities, respiratory failure with apnea, metabolic acidosis and shock; six hours later she died. Autopsy findings showed active diffuse lupus nephritis and diffuse systemic vasculitis that involved vessels from the skin, brain, myocardium, spleen, iliac and renal arteries. In addition, serositis of the small intestine and colon, acute and chronic pericarditis, pericardial effusion and myocarditis were found. Immunologic tests confirmed SLE diagnosis. In this case the fulminant course was the result of SLE high disease activity, visceral vasculitis of several organs and late diagnosis, referral and treatment. Early diagnosis, and opportune referral to the rheumatologist for intensive treatment can improve the outlook in these patients. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  4. Vancomycin-Induced Leukocytoclastic Vasculitis and Acute Renal Failure Due to Tubulointerstitial Nephritis.

    Science.gov (United States)

    Pingili, Chandra Shekar; Okon, Emmanuel E

    2017-09-25

    BACKGROUND Methicillin-resistant Staphylococcus aureus (MRSA) bacteremia and sepsis are commonly treated with intravenous vancomycin. However, vancomycin treatment is associated adverse reactions, including skin rashes and nephrotoxicity. We present a case of acute renal failure due to acute tubulointerstitial nephritis associated with a diffuse leukocytoclastic vasculitic skin eruption following intravenous vancomycin treatment. CASE REPORT A 79-year-old Caucasian male patient was treated with intravenous vancomycin for MRSA bacteremia. Prior to treatment, his creatinine was normal at 0.6 mg/dl. He presented one week later with shortness of breath, lower limb edema, and acute renal failure. He had a diffuse maculopapular rash involving the trunk and both upper and lower extremities. A renal biopsy and left arm skin biopsy were examined histologically. The skin biopsy showed leukocytoclastic vasculitis. Renal biopsy showed some sclerosed glomeruli, some with mesangial proliferation, and tubulointerstitial inflammation with eosinophils and plasma cells and mild interstitial fibrosis. Although there was some renal arteriolosclerosis, no vasculitic changes were seen, and no vascular thrombosis was present. A diagnosis of leukocytoclastic vasculitis and acute tubulointerstitial nephritis secondary to intravenous vancomycin therapy was made. CONCLUSIONS Although skin reactions associated with drug therapy are common, vancomycin-associated dermal vasculitis is rare. Tubulointerstitial nephritis is also a rare association with vancomycin treatment. This case report has highlighted that patients being treated with intravenous vancomycin should be carefully observed for acute skin rashes and deterioration in renal function, which can be managed by ceasing treatment with vancomycin, steroid challenge, and preventing future exposure to similar antimicrobial agents.

  5. Cocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis

    Directory of Open Access Journals (Sweden)

    F.V. Veronese

    2016-01-01

    Full Text Available Levamisole has been increasingly used as an adulterant of cocaine in recent years, emerging as a public health challenge worldwide. Levamisole-associated toxicity manifests clinically as a systemic vasculitis, consisting of cutaneous, hematological, and renal lesions, among others. Purpura retiform, cutaneous necrosis, intravascular thrombosis, neutropenia, and less commonly crescentic nephritis have been described in association with anti-neutrophil cytoplasmic antibodies (ANCAs and other autoantibodies. Here we report the case of a 49-year-old male who was a chronic cocaine user, and who presented spontaneous weight loss, arthralgia, and 3 weeks before admission purpuric skin lesions in the earlobes and in the anterior thighs. His laboratory tests on admission showed serum creatinine of 4.56 mg/dL, white blood count 3,800/μL, hemoglobin 7.3 g/dL, urinalysis with 51 white blood cells/μL and 960 red blood cells/μL, and urine protein-to-creatinine ratio 1.20. Serum ANCA testing was positive (>1:320, as well as serum anti-myeloperoxidase and anti-proteinase 3 antibodies. Urine toxicology screen was positive for cocaine and levamisole, with 62.8% of cocaine, 32.2% of levamisole, and 5% of an unidentified substance. Skin and renal biopsies were diagnostic for leukocytoclastic vasculitis and pauci-immune crescentic glomerulonephritis, respectively. The patient showed a good clinical response to cocaine abstinence, and use of corticosteroids and intravenous cyclophosphamide. Last serum creatinine was 1.97 mg/dL, white blood cell count 7,420/μL, and hemoglobin level 10.8 g/dL. In levamisole-induced systemic vasculitis, the early institution of cocaine abstinence, concomitant with the use of immunosuppressive drugs in severe cases, may prevent permanent end organ damage and associate with better clinical outcomes.

  6. Cocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis.

    Science.gov (United States)

    Veronese, F V; Dode, R S O; Friderichs, M; Thomé, G G; da Silva, D R; Schaefer, P G; Sebben, V C; Nicolella, A R; Barros, E J G

    2016-01-01

    Levamisole has been increasingly used as an adulterant of cocaine in recent years, emerging as a public health challenge worldwide. Levamisole-associated toxicity manifests clinically as a systemic vasculitis, consisting of cutaneous, hematological, and renal lesions, among others. Purpura retiform, cutaneous necrosis, intravascular thrombosis, neutropenia, and less commonly crescentic nephritis have been described in association with anti-neutrophil cytoplasmic antibodies (ANCAs) and other autoantibodies. Here we report the case of a 49-year-old male who was a chronic cocaine user, and who presented spontaneous weight loss, arthralgia, and 3 weeks before admission purpuric skin lesions in the earlobes and in the anterior thighs. His laboratory tests on admission showed serum creatinine of 4.56 mg/dL, white blood count 3,800/μL, hemoglobin 7.3 g/dL, urinalysis with 51 white blood cells/μL and 960 red blood cells/μL, and urine protein-to-creatinine ratio 1.20. Serum ANCA testing was positive (>1:320), as well as serum anti-myeloperoxidase and anti-proteinase 3 antibodies. Urine toxicology screen was positive for cocaine and levamisole, with 62.8% of cocaine, 32.2% of levamisole, and 5% of an unidentified substance. Skin and renal biopsies were diagnostic for leukocytoclastic vasculitis and pauci-immune crescentic glomerulonephritis, respectively. The patient showed a good clinical response to cocaine abstinence, and use of corticosteroids and intravenous cyclophosphamide. Last serum creatinine was 1.97 mg/dL, white blood cell count 7,420/μL, and hemoglobin level 10.8 g/dL. In levamisole-induced systemic vasculitis, the early institution of cocaine abstinence, concomitant with the use of immunosuppressive drugs in severe cases, may prevent permanent end organ damage and associate with better clinical outcomes.

  7. Circulating Markers of Vascular Injury and Angiogenesis in ANCA-Associated Vasculitis

    Science.gov (United States)

    Monach, Paul A; Tomasson, Gunnar; Specks, Ulrich; Stone, John H; Cuthbertson, David; Krischer, Jeffrey; Ding, Linna; Fervenza, Fernando C; Fessler, Barri J; Hoffman, Gary S; Ikle, David; Kallenberg, Cees GM; Langford, Carol A; Mueller, Mark; Seo, Philip; St.Clair, E William; Spiera, Robert; Tchao, Nadia; Ytterberg, Steven R; Gu, Yi-Zhong; Snyder, Ronald D; Merkel, Peter A

    2011-01-01

    Objective To identify biomarkers that distinguish between active ANCA-associated vasculitis (AAV) and remission in a manner superior or complementary to established markers of systemic inflammation. Methods Markers of vascular injury and angiogenesis were measured before and after treatment in a large clinical trial in AAV. 163 subjects enrolled in the Rituximab in ANCA-Associated Vasculitis (RAVE) trial were studied. Serum levels of E-selectin, ICAM-3, MMP1, MMP3, MMP9, P-selectin, thrombomodulin, and VEGF were measured at study screening (time of active disease) and at month 6. ESR and CRP levels had been measured at the time of the clinical visit. The primary outcome was the difference in marker level between screening and month 6 among patients in remission (BVAS/WG score of 0) at month 6. Results All subjects had severe active vasculitis (mean BVAS/WG score 8.6 +/− 3.2 SD) at screening. Among the 123 subjects clinically in remission at month 6, levels of all markers except E-selectin showed significant declines. MMP3 levels were also higher among the 23 subjects with active disease at month 6 than among the 123 subjects in remission. MMP3 levels correlated weakly with ESR and CRP. Conclusion Many markers of vascular injury and angiogenesis are elevated in severe active AAV and decline with treatment, but MMP3 appears to distinguish active AAV from remission better than the other markers studied. Further study of MMP3 is warranted to determine its clinical utility in combination with conventional markers of inflammation and ANCA titers. PMID:21953143

  8. Central Diabetes Insipidus in Refractory Antineutrophil Cytoplasmic Antibody-associated Vasculitis.

    Science.gov (United States)

    Ohashi, Keiji; Morishita, Michiko; Watanabe, Haruki; Sada, Ken-Ei; Katsuyama, Takayuki; Miyawaki, Yoshia; Katsuyama, Eri; Narazaki, Mariko; Tatebe, Noriko; Watanabe, Katsue; Kawabata, Tomoko; Wada, Jun

    2017-11-01

    We herein describe two cases of refractory antineutrophil cytoplasmic antibody-associated vasculitis (AAV) complicated with diabetes insipidus (DI) possibly related to hypertrophic pachymeningitis (HP). One patient had microscopic polyangiitis and HP, which were refractory to cyclophosphamide, azathioprine, rituximab, mycophenolate mofetil (MMF), and mizoribine. Remission was finally achieved with the use of etanercept, but DI occurred 5 years later. The other patient had granulomatosis with polyangiitis, which that was refractory to cyclophosphamide, methotrexate, MMF, and rituximab. DI subsequently developed, but was successfully treated with etanercept. Dura mater hypertrophy was macroscopically observed in the latter case.

  9. Mesenteric vasculitis in adults with Henoch-Schonlein purpura: a not-so-benign condition.

    LENUS (Irish Health Repository)

    Sibartie, V

    2009-02-07

    INTRODUCTION: The gastrointestinal manifestations of Henoch-Schonlein purpura (HSP) are well characterised, but their recognition can be difficult when they occur in isolation. Furthermore, HSP can run a more serious course in adults, compared to children, in whom the disease usually occurs. MATERIALS AND METHODS: We describe two cases that illustrate the challenges of HSP with mesenteric vasculitis and the outcome in adults. CONCLUSION: Although self-limiting in most patients, the outcome of HSP in adults can be far from benign and even fatal.

  10. Benign Atrophic Papulosis (Degos Disease) With Lymphocytic Vasculitis and Lichen Sclerosus-Like Features.

    Science.gov (United States)

    Kim, Esther; Motaparthi, Kiran

    2018-04-01

    Degos disease manifests as 2 distinct clinical variants, malignant atrophic papulosis and benign atrophic papulosis, which are distinguished by the presence or absence of systemic disease. Both forms feature cutaneous involvement typified by erythematous papules with scar-like centers, and the classic histologic picture is described as wedge-shaped dermal necrosis overlying thrombotic vasculopathy. However, the histopathology of early lesions is distinct and more variable. A case of benign atrophic papulosis with lymphocytic vasculitis and lichen sclerosus-like features is described, and the spectrum of histologic findings in Degos disease is reviewed.

  11. Upper Respiratory Tract Symptoms, Renal Involvement and Vasculitis: A Case Report and Review of Wegener Granulomatosis

    Science.gov (United States)

    Said, Mohd Shahrir Mohamed

    2010-01-01

    Wegener’s Granulomatosis is a condition associated with systemic vasculitis which can present with upper respiratory tract symptoms initially. On September 2001, a 15-year-old girl presented with symptoms of nasal block for 3 weeks. She later developed joint pains and worsening renal status requiring dialysis. A renal biopsy was performed which showed pauci-immune cresentric glomerulonephritis. Her cANCA levels were positive. She was treated with oral cyclophosphamide and steroids and later responded. Keywords Wegener granulomatosis; Young girl; Cyclophophamide; cANCA PMID:21629538

  12. Prospective study of radioimmunoassay for antibodies against neutrophil cytoplasm in diagnosis of systemic vasculitis

    Energy Technology Data Exchange (ETDEWEB)

    Savage, C.O.S.; Winearls, C.G.; Jones, S.; Marshall, P.D.; Lockwood, C.M.

    1987-06-20

    The diagnosis and management of Wegener's granulomatosis and microscopic polyarteritis are complicated by the lack of specific diagnostic tests. The diagnostic performance of a solid-phase radioimmunoassay, which detects the autoantibodies against neutrophil cytoplasm present in these disorders, was assessed in a prospective study of patients with suspected vasculitis and/or rapidly progressive nephritis. The assay had a sensitivity and specificity of 96% when carried out in combination with a specific inhibition stage and indirect immunofluorescence staining of alcohol-fixed normal neutrophils.

  13. Prospective study of radioimmunoassay for antibodies against neutrophil cytoplasm in diagnosis of systemic vasculitis

    International Nuclear Information System (INIS)

    Savage, C.O.S.; Winearls, C.G.; Jones, S.; Marshall, P.D.; Lockwood, C.M.

    1987-01-01

    The diagnosis and management of Wegener's granulomatosis and microscopic polyarteritis are complicated by the lack of specific diagnostic tests. The diagnostic performance of a solid-phase radioimmunoassay, which detects the autoantibodies against neutrophil cytoplasm present in these disorders, was assessed in a prospective study of patients with suspected vasculitis and/or rapidly progressive nephritis. The assay had a sensitivity and specificity of 96% when carried out in combination with a specific inhibition stage and indirect immunofluorescence staining of alcohol-fixed normal neutrophils. (author)

  14. Recurrence of ANCA-associated vasculitis in a patient with kidney trasplant

    Directory of Open Access Journals (Sweden)

    Pedro García Cosmes

    2016-03-01

    Full Text Available Renal disease secondary to vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA can lead to chronic renal disease requiring renal replacement therapy. In these patients, kidney transplantation offers excellent long-term rates of allograft and patient survival; consequently, they can be trasplanted when the clinical disease activity has remitted. However, the risk of disease relapses in the renal allograft remains, although at lower rates due to modern immunosuppressive regimes. We describe the case of a male patient with extracapillary glomerulonephritis type III C-ANCA (+ who developed a recurrence in the renal allograft 8 years after transplantation. Intensive immunosupression with plasmapheresis controlled the disease.

  15. Location Systems An Introduction to the Technology Behind Location Awareness

    CERN Document Server

    LaMarca, Anthony

    2008-01-01

    Advances in electronic location technology and the coming of age of mobile computing have opened the door for location-aware applications to permeate all aspects of everyday life. Location is at the core of a large number of high-value applications ranging from the life-and-death context of emergency response to serendipitous social meet-ups. For example, the market for GPS products and services alone is expected to grow to US200 billion by 2015. Unfortunately, there is no single location technology that is good for every situation and exhibits high accuracy, low cost, and universal coverage.

  16. Leukocytoclastic Vasculitis as a Complication of Recombinant Granulocyte Colony-Stimulating Factor Therapy in a Heart Transplant Patient

    Directory of Open Access Journals (Sweden)

    Giovanbattista Ippoliti

    2014-01-01

    Full Text Available Recombinant granulocyte colony-stimulating factor (rG-CSF is a myeloid growth factor that is widely used in haematology to recover neutropenia secondary to myelosuppressive chemotherapy. Leukocytoclastic vasculitis is an acknowledged side effect of the above therapy. Its pathogenesis involves many mechanisms that collectively induce an increase in neutrophil function and a subsequent release of cytokines. Here, we report a case of leukocytoclastic vasculitis proven by skin biopsy, following the use of rG-CSF in a heart transplant patient with leukopenia secondary to immunosuppressive therapy.

  17. Polymyalgia rheumatica and giant cell arteritis-three challenges-consequences of the vasculitis process, osteoporosis, and malignancy

    DEFF Research Database (Denmark)

    Emamifar, Amir; Hess, Søren; Gerke, Oke

    2017-01-01

    as follows: vasculitis process/vascular stiffness, malignancy, and osteoporosis. METHODS AND ANALYSIS: All patients with suspected PMR/GCR referred to the Rheumatology section of Medicine Department at Svendborg Hospital, Denmark. The 4 separate studies in the current protocol focus on: the association...... compared with conventional workup (ie, chest X-ray/abdominal ultrasound); and the impact of disease process, and also steroid treatment on bone mineral density, body composition, and vasculitis/vascular stiffness in PMR/GCA patients. ETHICS AND DISSEMINATION: The study has been approved by the Regional...

  18. Long-term follow-up of cyclophosphamide compared with azathioprine for initial maintenance therapy in ANCA-associated vasculitis

    DEFF Research Database (Denmark)

    Walsh, M.; Faurschou, M.; Berden, A.

    2014-01-01

    BACKGROUND AND OBJECTIVES: Treatment with azathioprine within 3 months of remission induction with cyclophosphamide is a common treatment strategy for patients with ANCA-associated vasculitis. This study comprised patients undergoing long-term follow-up who were randomly allocated to azathioprine...... after 3-6 months or after 12 months of cyclophosphamide treatment. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Patients from 39 European centers between 1995 and 1997 with a new diagnosis of ANCA-associated vasculitis that involved the kidneys or another vital organ were eligible. At the time...

  19. Invasive Candida krusei infection and Candida vasculitis of a leg ulcer in an immunocompetent patient: A case report

    Directory of Open Access Journals (Sweden)

    Philipp Jud

    2017-02-01

    Full Text Available A 71 year old female Caucasian farmer without any known immunosuppression presented with a painful ulcer of her right lower leg after a trauma caused by a wood billet. There was no response to empirical antibacterial treatment. An ulcer biopsy showed an invasive Candida infection of the soft tissue and leucocytoclastic vasculitis. Voriconazole treatment was followed by wound healing. Invasive Candida infection and localized Candida vasculitis represent a rare cause of persisting leg ulcers. The similar clinical picture of chronic venous leg ulcers might blur the true cause and refractory cases should therefore promptly be processed by histopathological diagnostics.

  20. Invasive Candida krusei infection and Candida vasculitis of a leg ulcer in an immunocompetent patient: A case report.

    Science.gov (United States)

    Jud, Philipp; Valentin, Thomas; Regauer, Sigrid; Gary, Thomas; Hackl, Gerald; Rief, Peter; Brodmann, Marianne; Hafner, Franz

    2017-02-01

    A 71year old female Caucasian farmer without any known immunosuppression presented with a painful ulcer of her right lower leg after a trauma caused by a wood billet. There was no response to empirical antibacterial treatment. An ulcer biopsy showed an invasive Candida infection of the soft tissue and leucocytoclastic vasculitis. Voriconazole treatment was followed by wound healing. Invasive Candida infection and localized Candida vasculitis represent a rare cause of persisting leg ulcers. The similar clinical picture of chronic venous leg ulcers might blur the true cause and refractory cases should therefore promptly be processed by histopathological diagnostics. Copyright © 2017 The Author(s). Published by Elsevier Ltd.. All rights reserved.

  1. Vasculite granulomatosa necrosante isolada de colo uterino associada à infecção por papilomavírus humano Isolated necrotizing granulomatous vasculitis of the uterine cervix associated with human papillomavirus infection

    Directory of Open Access Journals (Sweden)

    Nilton Salles Rosa Neto

    2009-10-01

    Full Text Available Vasculites de órgão único, ou isoladas, já foram descritas em diversos órgãos e seu achado pode ser acidental. Relatamos um caso de vasculite granulomatosa necrosante isolada de colo uterino em uma paciente de meia-idade, previamente hígida, sexualmente ativa, e cuja pesquisa de DNA de papilomavírus humano (Human Papiloma Virus - HPV por captura híbrida foi positiva. Não foi identificado comprometimento sistêmico e, como houve excisão completa da lesão, optou-se pelo acompanhamento clínico. Há poucos relatos, na literatura, de acometimento do trato genital feminino de forma isolada, alguns com presença simultânea de lesões que podem ser causadas pelo HPV, postulando-se uma associação patogênica.Single organ vasculitis (SOV, or isolated vasculitis, has been described in several organs and it can be an accidental finding. We report a case of isolated necrotizing granulomatous vasculitis of the uterine cervix in a middle-aged woman, previously healthy, and sexually active, and whose human papillomavirus (HPV DNA hybrid capture assay was positive. Systemic involvement was not detected and, since the lesion was completely removed, we opted for a clinical follow-up. The literature has very few reports on the isolated involvement of the female genital tract, and some had concomitant lesions that could be caused by the HPV, indicati.

  2. Análisis Histórico de la Vasculitis. Clasificación y propuestas para un entendimiento.

    Directory of Open Access Journals (Sweden)

    Antonio Iglesias Gamarra

    2002-04-01

    Full Text Available

    Apartir de 1978, siendo residente de Reumatología iniciamos el estudio de las vasculitis en el Hospital San Juan de Dios de Bogotá. Se escribió el primer texto de vasculitis necrotizante que ganó el premio Salvat en 1980 y se publicó en 1982: Este libro tenía como objeto informar el tema a la comunidad médica.

    Durante mi estadía en Barranquilla, se realizó una descripción de 9 pacientes con los doctores Eduardo Egea, Gloria Garavito y Silvio Severini sobre vasculitis linfomonocítica. Previamente se conocían algunos informes en la literatura médica como el realizado por Mc’Duffie fundador del Laboratorio de la Clínica Mayo en Rochester y Jorge Maldonado quienes describieron una vasculitis asociada a urticaria y se documentó una precipitina C1q, pero que no reunía los criterios de lupus eritematoso sistémico.

    Otra publicación era la de Soter quien describió dos tipos de vasculitis, la leucocitoclastica y la linfomonocitica. Con estos estudios previos, Iglesias y col estudiamos 9 pacientes que consultaron por urticaria, lesiones eritematosas, papulas, etc. Las lesiones de urticaria tenían una característica importante y era que permanecían por más de 24 horas en el sitio de la piel comprometida, eran de tipo urente y al tomar una biopsia de esta lesión se observaba un infiltrado linfoóplasmocitario; posiblemente su patogénesis está relacionada con un mecanismo de tipo celular y participan una serie de proteínas (como integrinas, selectinas y moléculas de adhesión.

    Se documenta que las vasculitis linfomonocíticas se asocian a algunos medicamentos, procesos infecciosos, o son secundarias a algunas enfermedades del tejido conjuntivo como el lupus y el síndrome de Sjögren primario.

    Desde hace más o menos cinco años estamos estudiando en las unidades de reumatología, dermatología y patología del Hospital San Juan de Dios, a los pacientes con lesiones nodulares, paniculitis y eritema

  3. Necrotizing mycotic vasculitis with cerebral infarction caused by Aspergillus niger in a horse with acute typholocolitis.

    Science.gov (United States)

    Tunev, S S; Ehrhart, E J; Jensen, H E; Foreman, J H; Richter, R A; Messick, J B

    1999-07-01

    An 18-year-old Morgan mare was presented to the Veterinary Medical Teaching Hospital, University of Illinois, with a 10-day history of watery diarrhea, depression, and dysphagia. On admission, the animal was severely dehydrated, depressed, and unable to swallow and had no clinical signs of diarrhea. The respiratory and heart rate and body temperature were within normal limits. Following fluid therapy, the mare developed severe watery diarrhea and continued to be depressed, incoordinated, and dysphagic. The animal died on the fourth day after admission and was sent to the Laboratories of Veterinary Diagnostic Medicine for necropsy. Gross postmortem findings were consistent with an acute cerebral infarction in the right cerebral hemisphere, an acute necrotizing typhlocolitis, multifocal petechial and ecchymotic hemorrhages, enlarged and congested pars intermedia of the pituitary gland, and marked bilateral adrenocortical hyperplasia with multifocal areas of necrosis and hemorrhage. Histologic evaluation of the affected brain demonstrated an area of coagulative necrosis of the gray matter, with hemorrhage, vasculitis, and thrombosis. There were many fungal hyphae 3.5-6.0 microm, pale basophilic, septate, and occasionally branching at 45 degrees present in the arterial walls and throughout the necrotic tissue. Immunohistochemical analysis revealed Aspergillus niger as the etiologic agent responsible for the mycotic vasculitis and infarction in the brain. Bacteria culture and immunohistochemical staining of the colon and cecum failed to demonstrate specific pathogens.

  4. Syndrome of cocaine-levamisole-induced cutaneous vasculitis and immune-mediated leukopenia.

    Science.gov (United States)

    Poon, Samuel H; Baliog, Crisostomo R; Sams, Ralph N; Robinson-Bostom, Leslie; Telang, Gladys H; Reginato, Anthony M

    2011-12-01

    We describe 4 patients who presented with palpable purpura, arthralgia or arthritis, leukopenia, and antineutrophil cytoplasmic antigen (ANCA) positivity most likely as a result of a hypersensitivity reaction to cocaine-levamisole induced vasculopathy. Cases were seen and reviewed in both the inpatient consult service and the outpatient clinics at Rhode Island Hospital from August 2009 to August 2010. Clinical characteristics as well as pertinent laboratory parameters were also reviewed and corroborated with a review of the present literature. We describe 3 cases of cocaine-levamisole-related cutaneous vasculitis with or without associated neutropenia, and 1 case of severe neutropenia with oral mucosal ulceration. Further serologic studies revealed maximum titers of ANCA mostly in a perinuclear pattern. Antimyeloperoxidase tested negative or mildly elevated in our cohort. Three patients with neutropenia had positive antigranulocyte IgM antibody. Nonsteroidal anti-inflammatory drugs were effective as first-line treatment for joint pain. The use of colchicine and systemic corticosteroid was employed to manage severe and persistent skin lesions. Cocaine-levamisole-related cutaneous vasculitis with leukopenia is a diagnosis of exclusion, but this diagnosis should be strongly considered in patients with a history of cocaine abuse who present with a tetrad of cutaneous manifestations consisting of palpable purpura or bullae with ear involvement, arthralgias, leukopenia, and positive ANCA in high titers and negative Antimyeloperoxidase, when other infectious or idiopathic vasculitic entities have been excluded. Published by Elsevier Inc.

  5. Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Ana Paula Sakamoto

    Full Text Available Abstract Objectives: To assess clinical digital vasculitis (DV as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE within a large population. Methods: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. Results: DV was observed in 25/852 (3% cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%, periungual infarction in 7 (28%, tip finger ulceration in 4 (16%, painful nodules in 1 (4% and gangrene in 1 (4%. A poor outcome, with digital resorption, occurred in 5 (20%. Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p = 0.008, discoid rash (16% vs. 4%, p = 0.017, photosensitivity (76% vs. 45%, p = 0.002 and other cutaneous vasculitides (80% vs. 19%, p 0.05. SLEDAI-2K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10 (0-28 vs. 14 (0-58, p = 0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p = 0.014 was significantly lower in the DV group. Conclusion: Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients.

  6. [Systemic necrotizing vasculitis presenting as gangrene combined with diabetes insipidus: a case report].

    Science.gov (United States)

    Huang, Qing; Liu, Yu-lan

    2015-12-18

    The male patient reported here presented as gangrene and central diabetes insipidus (CDI), who had characteristics of vasculitis. The patient complained about polydipsia and polyuria half a year ago, and then developed tingling, pain and blackish discoloration of some fingers and toes 3 month ago. He also had Raynaud's phenomenon. After admission, his laboratory examination showed the rise of erythrocyte sedimentation rate, C-reactive protein, immunoglobulin, β2-glycoprotein I and the activity of rheumatoid factors, lupus anticoagulant test. his pituitary gland showed loss of posterior signal on magnetic resonance imaging. In addition, his vasopressin test was active. However, there was no sufficient evidence to diagnose any specific disease; as a consequence the patient was diagnosed as idiopathic systemic necrotizing vasculitis (SNV). For SNV, the patient was treated with glucocorticoid 40 mg/d and impact therapy of cyclophosphamide 0.4 g every 2 weeks. He also received symptomatic treatment for gangrene and CDI. Cutaneous involvement leading to gangrene was widely reported in SNV, however pituitary involvement in SNV leading to CDI was rare. The prognosis of this patient was poor.

  7. Cutaneous vasculitis as a presenting feature of multiple myeloma: A report of 2 cases

    Directory of Open Access Journals (Sweden)

    Kembre Priyam

    2006-01-01

    Full Text Available We report two male patients who presented with symmetrical, painful purpura that evolved into bullae and necrotic ulcers, predominantly on the extremities, over two months in spite of conventional therapy including oral steroids. Examination showed livedoid and purpuric patches with necrotic centers in starburst pattern over the extremities and buttocks. The first case also had similar lesions over the ears. The clinical presentation and the histopathological examination suggested a diagnosis of necrotizing leukocytoclastic vasculitis (LCV. Blood testing ruled out connective tissue disease, hepatitis B or C infection or streptococcal infection as underlying cause of vasculitis. Serum antinuclear factor, antineutrophilic cytoplasmic antibody and anticardiolipin anticoagulant were negative in both cases. Cryoglobulins were positive in case 2. An incidental finding was raised serum proteins and globulins in case 2. Further investigations revealed M band on electrophoresis and features of multiple myeloma on bone marrow biopsy in both cases. These cases emphasize the importance of simple investigations like serum proteins in the evaluation of LCV.

  8. Systematic review of the role of rituximab in treatment of antineutrophil cytoplasmic autoantibody-associated vasculitis, hepatitis C virus-related cryoglobulinemic vasculitis, Henoch–Schönlein purpura, ankylosing spondylitis, and Raynaud's phenomenon

    Directory of Open Access Journals (Sweden)

    Taha R

    2017-12-01

    Full Text Available Rbab Taha,1 Hadeel El-Haddad,1 Abdulqader Almuallim,2 Fatma Alshaiki,3 Elaf Obaid,2 Hani Almoallim1,2,4 1Department of Medicine, Dr Soliman Fakeeh Hospital, Jeddah, 2Department of Medicine, Faculty of Medicine, Umm Al-Qura University, Mecca, 3Department of Medicine, East Jeddah Hospital, Jeddah, 4Rheumatic Diseases, Umm Al-Qura University, Mecca, Saudi Arabia Abstract: Rituximab (RTX is established for the treatment of rheumatoid arthritis. This systematic review of the literature since 2006 summarizes evidence for the use of RTX in the treatment of additional rheumatological diseases: antineutrophil cytoplasmic autoantibody-associated vasculitis (AAV, hepatitis C virus-related cryoglobulinemic vasculitis, Henoch–Schönlein purpura, ankylosing spondylitis, and Raynaud’s phenomenon. Data from randomized controlled trials are available only for AAV, confirming efficacy for remission induction, including in disease resistant to conventional treatment, and maintenance of remission. Further studies are required to confirm optimal maintenance regimens in AAV, important questions needing to be addressed including protocol administration versus treatment in response to clinical relapse and the importance of maintaining B-cell depletion. Sufficient data are available in other diseases to suggest RTX to be useful and that randomized controlled trials should be conducted. Keywords: anti-CD20 monoclonal antibody, anti-neutrophil cytoplasmic antibody (ANCA-associated vasculitis, refractory ankylosing spondylitis, resistant cryoglobulinemic vasculitis, refractory rheumatological diseases 

  9. Increased Expression of Toll-Like Receptors by Monocytes and Natural Killer Cells in ANCA-Associated Vasculitis

    NARCIS (Netherlands)

    Tadema, Henko; Abdulahad, Wayel H.; Stegeman, Coen A.; Kallenberg, Cees G. M.; Heeringa, Peter

    2011-01-01

    Introduction: Toll-like receptors (TLRs) are a family of receptors that sense pathogen associated patterns such as bacterial cell wall proteins. Bacterial infections are associated with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). Here, we assessed the expression of

  10. Imaging Findings of Central Nervous System Vasculitis Associated with Goodpasture's Syndrome: a Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jee Young; Ahn, Kook Jin; Jung, Jung Im; Jung, So Lyung; Kim, Bum Soo; Hahn, Seong Tae [College of Medicine, The Catholic University of Korea, Seoul (Korea, Republic of)

    2007-12-15

    We report a rare case of CNS vasculitis associated with Goodpasture's syndrome in a 34-year-old man, who presented with a seizure and sudden onset of right sided weakness. He also had recurrent hemoptysis of one month's duration. Goodpasture's syndrome is histologically diagnosed by intense linear deposits of IgG along the glomerular basement membrane in both renal and lung tissues. oodpasture's syndrome is a rare disease, characterized by rapidly progressive glomerulonephritis, diffuse pulmonary hemorrhage and circulating antiglomerular basement membrane antibody (anti-GBM antibody). Central nervous system (CNS) manifestations in Goodpasture's syndrome are extremely rare, with only a few cases having been reported in the literature (8 10). Therefore, we present our imaging findings of CNS vasculitis associated with Goodpasture's syndrome, together with a review of the relevant literature. In summary, CNS vasculitis associated with Goodpasture's syndrome is extremely rare. Awareness of the imaging findings, as well as the clinical significance of CNS vasculitis associated with Goodpasture's syndrome, can be helpful in making the correct diagnosis and subsequent management of this rare condition.

  11. Biopsy-proven case of Epstein-Barr virus (EBV)-associated vasculitis of the central nervous system.

    Science.gov (United States)

    Kano, Kohei; Katayama, Takayuki; Takeguchi, Shiori; Asanome, Asuka; Takahashi, Kae; Saito, Tsukasa; Sawada, Jun; Saito, Masato; Anei, Ryogo; Kamada, Kyousuke; Miyokawa, Naoyuki; Nishihara, Hiroshi; Hasebe, Naoyuki

    2017-06-01

    A 75-year-old woman was admitted to our hospital with rapidly deteriorating consciousness disturbance. She had a 7-year history of rheumatoid arthritis (RA), which had been treated with methotrexate (MTX) and prednisolone. Brain T2-weighted MRI showed diffuse high-intensity lesions in the cerebral subcortical and deep white matter, bilateral basal ganglia and thalamus. A cerebrospinal fluid examination revealed elevated protein levels and positive Epstein-Barr virus (EBV) DNA. Human immunodeficiency virus was negative. Brain biopsy showed perivascular lymphocytic infiltration in the parenchyma and meninx with EBV-encoded small RNA (EBER). Since this case did not fulfill the criteria for chronic active EBV infection (CAEBV), she was diagnosed with Epstein-Barr virus (EBV)-associated vasculitis of the central nervous system. High-dose methylprednisolone, acyclovir, ganciclovir and foscarnet were not effective. Although EBV is a causative agent of infectious mononucleosis (IM), lymphomas and nasopharyngeal carcinomas, vasculitic pathology of the central nervous system with EBV reactivation in the elderly is rare. Immunosuppressive drugs such as steroids and MTX are widely used to treat autoimmune disorders, but may exacerbate the reactivation of EBV. This is the first case of biopsy-proven EBV-positive/HIV-negative vasculitis during the treatment of RA with MTX and steroids. This case indicates that EBV-associated vasculitis needs to be considered as a differential diagnosis of CNS vasculitis. © 2016 Japanese Society of Neuropathology.

  12. Long-term Prognosis of Anti-Neutrophil Cytoplasmic Antibody-Negative Renal Vasculitis: Cohort Study in Korea.

    Science.gov (United States)

    Lee, Sung Woo; Yu, Mi-Yeon; Baek, Seon Ha; Ahn, Shin-Young; Kim, Sejoong; Na, Ki Young; Chae, Dong-Wan; Chin, Ho Jun

    2016-04-01

    Few studies have reported on the long-term prognosis of anti-neutrophil cytoplasmic antibody (ANCA)-negative renal vasculitis. Between April 2003 and December 2013, 48 patients were diagnosed with renal vasculitis. Their ANCA status was tested using indirect immunofluorescence and enzyme-linked immunosorbent assays. During a median (interquartile range) follow-up duration of 933.5 (257.5-2,079.0) days, 41.7% of patients progressed to end stage renal disease (ESRD) and 43.8% died from any cause. Of 48 patients, 6 and 42 were ANCA-negative and positive, respectively. The rate of ESRD within 3 months was higher in ANCA-negative patients than in ANCA-positive patients (P = 0.038). In Kaplan-Meier survival analysis, ANCA-negative patients showed shorter renal survival than did ANCA-positive patients (log-rank P = 0.033). In univariate Cox-proportional hazard regression analysis, ANCA-negative patients showed increased risk of ESRD, with a hazard ratio 3.190 (95% confidence interval, 1.028-9.895, P = 0.045). However, the effect of ANCA status on renal survival was not statistically significant in multivariate analysis. Finally, ANCA status did not significantly affect patient survival. In conclusion, long-term patient and renal survival of ANCA-negative renal vasculitis patients did not differ from those of ANCA-positive renal vasculitis patients. Therefore, different treatment strategy depending on ANCA status might be unnecessary.

  13. Location-based prospective memory.

    Science.gov (United States)

    O'Rear, Andrea E; Radvansky, Gabriel A

    2018-02-01

    This study explores location-based prospective memory. People often have to remember to do things when in a particular location, such as buying tissues the next time they are in the supermarket. For event cognition theory, location is important for structuring events. However, because event cognition has not been used to examine prospective memory, the question remains of how multiple events will influence prospective memory performance. In our experiments, people delivered messages from store to store in a virtual shopping mall as an ongoing task. The prospective tasks were to do certain activities in certain stores. For Experiment 1, each trial involved one prospective memory task to be done in a single location at one of three delays. The virtual environment and location cues were effective for prospective memory, and performance was unaffected by delay. For Experiment 2, each trial involved two prospective memory tasks, given in either one or two instruction locations, and to be done in either one or two store locations. There was improved performance when people received instructions from two locations and did both tasks in one location relative to other combinations. This demonstrates that location-based event structure influences how well people perform on prospective memory tasks.

  14. PF-1355, a mechanism-based myeloperoxidase inhibitor, prevents immune complex vasculitis and anti-glomerular basement membrane glomerulonephritis.

    Science.gov (United States)

    Zheng, Wei; Warner, Roscoe; Ruggeri, Roger; Su, Chunyan; Cortes, Christian; Skoura, Athanasia; Ward, Jessica; Ahn, Kay; Kalgutkar, Amit; Sun, Dexue; Maurer, Tristan S; Bonin, Paul D; Okerberg, Carlin; Bobrowski, Walter; Kawabe, Thomas; Zhang, Yanwei; Coskran, Timothy; Bell, Sammy; Kapoor, Bhupesh; Johnson, Kent; Buckbinder, Leonard

    2015-05-01

    Small vessel vasculitis is a life-threatening condition and patients typically present with renal and pulmonary injury. Disease pathogenesis is associated with neutrophil accumulation, activation, and oxidative damage, the latter being driven in large part by myeloperoxidase (MPO), which generates hypochlorous acid among other oxidants. MPO has been associated with vasculitis, disseminated vascular inflammation typically involving pulmonary and renal microvasculature and often resulting in critical consequences. MPO contributes to vascular injury by 1) catabolizing nitric oxide, impairing vasomotor function; 2) causing oxidative damage to lipoproteins and endothelial cells, leading to atherosclerosis; and 3) stimulating formation of neutrophil extracellular traps, resulting in vessel occlusion and thrombosis. Here we report a selective 2-thiouracil mechanism-based MPO inhibitor (PF-1355 [2-(6-(2,5-dimethoxyphenyl)-4-oxo-2-thioxo-3,4-dihydropyrimidin-1(2H)-yl)acetamide) and demonstrate that MPO is a critical mediator of vasculitis in mouse disease models. A pharmacokinetic/pharmacodynamic response model of PF-1355 exposure in relation with MPO activity was derived from mouse peritonitis. The contribution of MPO activity to vasculitis was then examined in an immune complex model of pulmonary disease. Oral administration of PF-1355 reduced plasma MPO activity, vascular edema, neutrophil recruitment, and elevated circulating cytokines. In a model of anti-glomerular basement membrane disease, formerly known as Goodpasture disease, albuminuria and chronic renal dysfunction were completely suppressed by PF-1355 treatment. This study shows that MPO activity is critical in driving immune complex vasculitis and provides confidence in testing the hypothesis that MPO inhibition will provide benefit in treating human vasculitic diseases. Copyright © 2015 by The American Society for Pharmacology and Experimental Therapeutics.

  15. The Birmingham Vasculitis Activity Score as a Measure of Disease Activity in Patients with Giant Cell Arteritis

    Science.gov (United States)

    Kermani, TA; Cuthbertson, D; Carette, S; Hoffman, GS; Khalidi, NA; Koening, CL; Langford, CA; McKinnon-Maksimowicz, K; McAlear, CA; Monach, PA; Seo, P; Warrington, KJ; Ytterberg, SR; Merkel, PA; Matteson, EL

    2016-01-01

    Objective To evaluate the performance of the Birmingham Vasculitis Activity Score (BVAS) in the assessment of disease activity in giant cell arteritis (GCA). Methods Patients with GCA enrolled in a prospective, multicenter, longitudinal study with symptoms of active vasculitis during any visit were included. Spearman’s rank correlation was used to explore the association of the BVAS with other measures of disease activity. Results During a mean (SD) follow-up of 2.3 (1.6) years, symptoms of active GCA were present in 236 visits in 136 subjects (100 female, 74%). Median (range) BVAS1 (new/worse symptoms) was 1 (0–10) and median (range) BVAS2 (persistent symptoms) was 0 (0–5). Median (range) physician global assessment (PGA) was 4 (0–9) for disease activity in the past 28 days and 2 (0–9) for activity on the day of the visit. Important ischemic manifestations of active vasculitis not captured by the BVAS included tongue/jaw claudication (27%), upper extremity claudication (15%), lower extremity claudication (5%), carotidynia (7%), ischemic retinopathy (5%). During 25 visits (11%) with active disease, all symptoms of active vasculitis were captured in the “Other” category yet still resulted in a BVAS 1 and BVAS 2 of 0. BVAS1 moderately correlated with PGA for the past 28 days (Spearman’s correlation 0.50) and physician-rated disease activity for the past 28 days (Spearman’s correlation 0.46). Conclusions The BVAS has limited utility in GCA. Patients with active GCA can have a BVAS of 0. Many important ischemic symptoms attributable to active vasculitis are not captured in the composite score. PMID:27036388

  16. Pressure Transducer Locations

    Data.gov (United States)

    National Aeronautics and Space Administration — Files are located here, defining the locations of the pressure transducers on the HIRENASD model. These locations also correspond to the locations that analysts...

  17. Efficacy and Safety of Sofosbuvir Plus Daclatasvir for Treatment of HCV-Associated Cryoglobulinemia Vasculitis.

    Science.gov (United States)

    Saadoun, David; Pol, Stanislas; Ferfar, Yasmina; Alric, Laurent; Hezode, Christophe; Si Ahmed, Si Nafa; de Saint Martin, Luc; Comarmond, Cloé; Bouyer, Anne Sophie; Musset, Lucile; Poynard, Thierry; Resche Rigon, Matthieu; Cacoub, Patrice

    2017-07-01

    Circulating mixed cryoglobulins are detected in 40%-60% of patients with hepatitis C virus (HCV) infection, and overt cryoglobulinemia vasculitis (CryoVas) develops in approximately 15% of patients. Remission of vasculitis has been associated with viral clearance, but few studies have reported the effectiveness of direct-acting antiviral drugs in these patients. We performed an open-label, prospective, multicenter study of the effectiveness and tolerance of an all-oral, interferon- and ribavirin-free regimen of sofosbuvir plus daclatasvir in patients with HCV-associated CryoVas. Forty-one consecutive patients with active HCV-associated CryoVas (median age, 56 y; 53.6% women) were recruited from hospitals in Paris, France, from 2014 through 2016. They received sofosbuvir (400 mg/day) plus daclatasvir (60 mg/day) for 12 weeks (n = 32) or 24 weeks (n = 9), and were evaluated every 4 weeks until week 24 and at week 36. Blood samples were analyzed for complete blood count, serum chemistry profile, level of alanine aminotransferase, rheumatoid factor activity, C4 fraction of complement, and cryoglobulin; peripheral blood mononuclear cells were isolated for flow cytometry analysis. Thirty-seven patients (90.2%) had a complete clinical response (defined by improvement of all the affected organs involved at baseline and no clinical relapse) after a median time of 12 weeks of therapy; all had a sustained virologic response (no detectable serum HCV RNA 12 weeks after the end of antiviral therapy). Patients' mean cryoglobulin level decreased from 0.56 ± 0.18 at baseline to 0.21 ± 0.14 g/L at week 36, and no cryoglobulin was detected in 50% of patients at this time point. After antiviral therapy, patients had increased numbers of T-regulatory cells, IgM+CD21-/low-memory B cells, CD4+CXCR5+ interleukin 21+ cells, and T-helper 17 cells, compared with before therapy. After a median follow-up period of 26 months (interquartile range, 20-30 mo), no patients had a serious adverse

  18. Clinical characteristics and outcomes of childhood-onset ANCA-associated vasculitis: a French nationwide study.

    Science.gov (United States)

    Sacri, Anne-Sylvia; Chambaraud, Tristan; Ranchin, Bruno; Florkin, Benoît; Sée, Hélène; Decramer, Stéphane; Flodrops, Hugues; Ulinski, Tim; Allain-Launay, Emma; Boyer, Olivia; Dunand, Olivier; Fischbach, Michel; Hachulla, Eric; Pietrement, Christine; Le Pogamp, Patrick; Stephan, Jean-Louis; Belot, Alexandre; Nivet, Hubert; Nobili, François; Guillevin, Loic; Quartier, Pierre; Deschênes, Georges; Salomon, Rémi; Essig, Marie; Harambat, Jérôme

    2015-04-01

    Data on anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis are scarce in children. The current study is aimed at describing the clinical features and outcomes of childhood-onset ANCA-associated vasculitis (AAV). We conducted a retrospective French multicentre study involving patients in whom AAV was diagnosed before the age of 18 years. Inclusion criteria were (i) granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) according to classification criteria of the European League Against Rheumatism/Paediatric Rheumatology European Society, and (ii) ANCA positivity. Patient and renal survival were analysed. Among 66 children included, 80% were female, 42% had GPA and 58% MPA including renal-limited vasculitis, 67% were pANCA+ and 33% cANCA+. The mean incidence of reported cases increased to 0.45 per million children/year in the period 2006-10. Median age at diagnosis was 11.5 years, and median time to diagnosis was 1 month. Initial symptoms included fever and fatigue (79%), skin lesions (41%), arthritis (42%), pulmonary (45%) and renal involvement (88%). Clinical features were similar between GPA and MPA with the exception of upper airway impairment (28%) specific of GPA. Ninety percent of the patients achieved remission after induction treatment. After a median follow-up of 5.2 years, 4 patients (6%) died, corresponding to a mortality rate of 1.2 per 100 person-years, and 22 patients (34%) developed end-stage renal disease (ESRD). Renal survival was 74, 70 and 59% at 1, 5 and 10 years, respectively. In a multivariable Cox regression model, baseline glomerular filtration rate, ethnic origin, histopathological classification and era of treatment were associated with the occurrence of ESRD. Relapse-free survival was 57% at 5 years and 34% at 10 years of follow-up. Patient and renal outcome did not significantly differ between GPA and MPA. Childhood-onset AAV is a rare disease characterized by female predominance, delayed diagnosis

  19. [Cavitating lung lesions in the course of ANCA-associated vasculitis: differential diagnostic aspects].

    Science.gov (United States)

    Kirchner, J; Raab, H P; Länger, F; Wigand, R; Mitrou, P; Jacobi, V

    1998-05-01

    Antineutrophil cytoplasmatic antibodies (ANCA)-associated vasculitides (Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome) show quite variable courses. Clinical features of the full blown generalized systemic vasculitis are usually found in the respiratory tract and the kidney. Pulmonary involvement of Wegener's granulomatosis shows commonly nodules and cavitations but also diffuse alveolar hemorrhage. We report the case of a 57 year-old man suffering from dyspnea, thoracal pain, arthralgia, purpura, scleritis and tinitus. Specimen of the kidney showed segmental glomerulosclerosis and tubulointerstitial nephritis. Because of the presence of cANCA Wegener's disease was assumed. Pulmonary infiltrates developed under immunosuppressive treatment with cyclophosphamid. As differential diagnosis of the pulmonary infiltrates, we considered invasive pulmonary aspergillosis as well as infiltrates due to Wegener's granulomatosis. In spite of maximal therapeutic management of patient died of respiratory and cardiovascular failure. The findings at autopsy showed distinct invasive pulmonary aspergillosis and perifocal hemorrhage.

  20. Recurrence of ANCA-associated vasculitis in a patient with kidney trasplant.

    Science.gov (United States)

    García Cosmes, Pedro; Fraile Gómez, Pilar; Lewczuk, Kamil; Rodríguez González, Marta; Ruiz Ferreras, Elena; Tabernero Fernández, Guadalupe

    2016-01-01

    Renal disease secondary to vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA) can lead to chronic renal disease requiring renal replacement therapy. In these patients, kidney transplantation offers excellent long-term rates of allograft and patient survival; consequently, they can be trasplanted when the clinical disease activity has remitted. However, the risk of disease relapses in the renal allograft remains, although at lower rates due to modern immunosuppressive regimens. We describe the case of a male patient with extracapillary glomerulonephritis type III C-ANCA (+) who developed a recurrence in the renal allograft 8 years after transplantation. Intensive immunosupression with plasmapheresis controlled the disease. Copyright © 2015 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

  1. Cytomegalovirus retinitis presenting as vasculitis in a patient with Wegener’s granulomatosis

    Directory of Open Access Journals (Sweden)

    Paris G Tranos

    2008-08-01

    Full Text Available Paris G Tranos1, Ilias Georgalas2, Panagiota Founti1, Ioannis Ladas21Papageorgiou University Hospital, Thessaloniki, Greece; 2Department of Ophthalmology, “G.Genimatas” Hospital, Athens, GreecePurpose: To present an unusual case of cytomegalovirus (CMV retinitis in a patient with Wegener’s granulomatosis.Results: A 54-year-old lady with Wegener’s granulomatosis presented with decreased vision in her left eye. Wegener’s retinal vasculitis was diagnosed initially and the patient received treatment with oral steroids. Three days later the patient developed typical CMV retinitis.Conclusion: The likelihood of CMV retinitis in patients with Wegener’s granulomatosis should not be overlooked. Increased awareness in such cases is very important since CMV retinitis may present with less typical manifestations, which makes the correct diagnosis more challenging.Keywords: cytomegalovirus retinitis, Wegener’s granulomatosis, ganciclovir

  2. Current literature and imaging techniques of aseptic lymphocyte-dominated vasculitis-associated lesions (ALVAL)

    International Nuclear Information System (INIS)

    Duggan, P.J.; Burke, C.J.; Saha, S.; Moonim, M.; George, M.; Desai, A.; Houghton, R.

    2013-01-01

    Aseptic lymphocyte-dominated vasculitis-associated lesions (ALVAL) are a recognized complication of metal-on-metal bearing hip prostheses. There is an impending concern regarding the future investigation and management of patients who have received such implants. The current literature is discussed, and the current guidelines for management of these patients in the UK are reviewed. The various imaging techniques available, such as computed tomography, metal artefact reduction magnetic resonance imaging, and ultrasound are discussed and evaluated with respect to the assessment of patients with suspected ALVAL. The histopathological findings are discussed with images of the tissue changes provided. Images of the radiological findings are also provided for all general radiological methods. ALVAL and its radiological presentation is an important issue that unfortunately may become a significant clinical problem

  3. [Humoral immune diseases: Cutaneous vasculitis and auto-immune bullous dermatoses].

    Science.gov (United States)

    Wechsler, Janine

    2018-02-01

    Humoral immunity is the cause of multiple diseases related to antibodies (IgA, IgG, IgM) produced by the patient. Two groups of diseases are identified. The first group is related to circulating antigen-antibody complexes. The antigens are various. They are often unknown. These immune complexes cause a vascular inflammation due to the complement fixation. Consequently, this group is dominated by inflammatory vasculitis. In the second group, the pathology is due to the fixation in situ of antibodies to a target antigen of the skin that is no more recognized by the patient. This group is represented by the auto-immune bullous dermatoses. Copyright © 2017. Published by Elsevier Masson SAS.

  4. Intermediate monocytes in ANCA vasculitis : increased surface expression of ANCA autoantigens and IL-1 beta secretion in response to anti-MPO antibodies

    NARCIS (Netherlands)

    O'Brien, Ein C.; Abdulahad, Wayel H.; Rutgers, Abraham; Huitema, Minke G.; O'Reilly, Vincent P.; Coughlan, Alice M.; Harrington, Mark; Heeringa, Peter; Little, Mark A.; Hickey, Fionnuala B.

    2015-01-01

    ANCA vasculitis encompasses several autoimmune conditions characterised by destruction of small vessels, inflammation of the respiratory tract and glomerulonephritis. Most patients harbour autoantibodies to myeloperoxidase (MPO) or proteinase 3 (PR3). Clinical and experimental data suggest that

  5. A Location Privacy Aware Friend Locator

    DEFF Research Database (Denmark)

    Siksnys, Laurynas; Thomsen, Jeppe Rishede; Saltenis, Simonas

    2009-01-01

    A location-based service called friend-locator notifies a user if the user is geographically close to any of the user’s friends. Services of this kind are getting increasingly popular due to the penetration of GPS in mobile phones, but existing commercial friend-locator services require users...... to trade their location privacy for quality of service, limiting the attractiveness of the services. The challenge is to develop a communication-efficient solution such that (i) it detects proximity between a user and the user’s friends, (ii) any other party is not allowed to infer the location of the user......, and (iii) users have flexible choices of their proximity detection distances. To address this challenge, we develop a client-server solution for proximity detection based on an encrypted, grid-based mapping of locations. Experimental results show that our solution is indeed efficient and scalable...

  6. Granulomatous slack skin - seven years follow-up of a case with features of "parapsoriasis en plaques", muscle involvement, granulomatous vasculitis, and necrobiotic changes.

    Science.gov (United States)

    ChiriŢă, Aurel Doru; Mărgăritescu, Irina

    2016-01-01

    Granulomatous slack skin (GSS) represents an extremely rare variant of mycosis fungoides with only 70 cases reported in the literature to date. It is characterized clinically by the occurrence of bulky, pendulous skinfolds, usually located in flexural areas and histologically by an infiltrate composed of small neoplastic T-lymphocytes joined by granulomatous inflammation with scattered multinucleated giant cells containing nuclei arranged in a wreath-like fashion. Since its first description, very rare cases of GSS with muscle involvement, large vessels involvement, or necrobiotic changes have been reported. We present an extraordinary case of GSS with all these unusual features developing in the lesions of the same patient. The long follow-up of seven years allowed us to document the evolution of each lesion. Some lesions appeared and evolved in a manner very reminiscent of those of "parapsoriasis en plaques", others were classical GSS lesions, and still others developed large ulcerated lesions. These ulcerated lesions consistently failed to respond to conventional wound therapy, skin directed therapy [retinoids + psoralen combined with ultraviolet A (PUVA)-therapy], and interferon-alpha therapy. Remarkably, the ulcers completely healed when systemic corticosteroids were added. We hence postulate that the ulcers appeared because of large vessel vasculitis rather than tumoral direct destruction.

  7. Oxaliplatin-Induced Leukocytoclastic Vasculitis under Adjuvant Chemotherapy for Colorectal Cancer: Two Cases of a Rare Adverse Event

    Directory of Open Access Journals (Sweden)

    Henriette Quack

    2013-12-01

    Full Text Available Leukocytoclastic vasculitis is a multicausal systemic inflammatory disease of the small vessels, histologically characterized by inflammation and deposition of both nuclear debris and fibrin in dermal postcapillary venules. The clinical picture typically involves palpable purpura of the lower legs and may be associated with general symptoms such as fatigue, arthralgia and fever. Involvement of the internal organs, most notably the kidneys, the central nervous system or the eyes, is possible and determines the prognosis. Oxaliplatin-induced leukocytoclastic vasculitis is a very rare event that limits treatment options in affected patients. We report 2 patients who developed the condition under chemotherapy for advanced rectal and metastatic colon carcinoma, respectively; a termination of the therapy was therefore necessary. While current therapies for colorectal cancer include the combination of multimodal treatment with new and targeted agents, rare and unusual side effects elicited by established agents also need to be taken into account for the clinical management.

  8. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial

    DEFF Research Database (Denmark)

    de Groot, Kirsten; Harper, Lorraine; Jayne, David R W

    2009-01-01

    BACKGROUND: Current therapies for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis are limited by toxicity. OBJECTIVE: To compare pulse cyclophosphamide with daily oral cyclophosphamide for induction of remission. DESIGN: Randomized, controlled trial. Random assignments were...... generalized ANCA-associated vasculitis with renal involvement but not immediately life-threatening disease. INTERVENTION: Pulse cyclophosphamide, 15 mg/kg every 2 to 3 weeks (76 patients), or daily oral cyclophosphamide, 2 mg/kg per day (73 patients), plus prednisolone. MEASUREMENT: Time to remission (primary.......7%). Thirteen patients in the pulse group and 6 in the daily oral group achieved remission by 9 months and subsequently had relapse. Absolute cumulative cyclophosphamide dose in the daily oral group was greater than that in the pulse group (15.9 g [interquartile range, 11 to 22.5 g] vs. 8.2 g [interquartile...

  9. Imaging of large vessel vasculitis with 18FDG PET: illusion or reality? A critical review of the literature data

    International Nuclear Information System (INIS)

    Belhocine, Tarik; Vandevivere, Johan; Blockmans, Daniel; Hustinx, Roland; Mortelmans, Luc

    2003-01-01

    Fluorine-18 fluorodeoxyglucose positron emission tomography ( 18 FDG PET) plays a major role in the management of oncology patients. Owing to the singular properties of the glucose tracer, many patients suffering from non-malignant diseases such as inflammatory or infectious diseases may also derive clinical benefit from the appropriate use of metabolic imaging. Large vessel vasculitides such as giant cell arteritis and Takayasu arteritis are other examples that may potentially extend the field of 18 FDG PET indications. The purpose of the present article is to assess the feasibility of metabolic imaging in vasculitis on the basis of the current literature data. In particular, the clinical context and the 18 FDG imaging patterns seen in patients with large vessel vasculitis are analysed in order to identify potential indications for metabolic imaging. (orig.)

  10. Imaging of large vessel vasculitis with {sup 18}FDG PET: illusion or reality? A critical review of the literature data

    Energy Technology Data Exchange (ETDEWEB)

    Belhocine, Tarik; Vandevivere, Johan [Department of Nuclear Medicine, A.Z. Middelheim Hospital, 2020, Antwerp (Belgium); Blockmans, Daniel [Department of Internal Medicine, Gasthuisberg University Hospital, Leuven (Belgium); Hustinx, Roland [Department of Nuclear Medicine, University Hospital of Liege, Liege (Belgium); Mortelmans, Luc [Department of Nuclear Medicine, Gasthuisberg University Hospital, Leuven (Belgium)

    2003-09-01

    Fluorine-18 fluorodeoxyglucose positron emission tomography ({sup 18}FDG PET) plays a major role in the management of oncology patients. Owing to the singular properties of the glucose tracer, many patients suffering from non-malignant diseases such as inflammatory or infectious diseases may also derive clinical benefit from the appropriate use of metabolic imaging. Large vessel vasculitides such as giant cell arteritis and Takayasu arteritis are other examples that may potentially extend the field of {sup 18}FDG PET indications. The purpose of the present article is to assess the feasibility of metabolic imaging in vasculitis on the basis of the current literature data. In particular, the clinical context and the {sup 18}FDG imaging patterns seen in patients with large vessel vasculitis are analysed in order to identify potential indications for metabolic imaging. (orig.)

  11. Computed tomography of the brain in cases with venous vasculitis compared with an age-matched reference group

    International Nuclear Information System (INIS)

    Hannerz, J.; Ericson, K.; Bergstrand, G.; Berggren, B.M.; Edman, G.; Karolinska Sjukhuset, Stockholm; Karolinska Sjukhuset, Stockholm

    1988-01-01

    Patients with a particular, steroid-sensitive headache and often characteristic pathology at orbital phlebography, have been suggested to suffer from venous vasculitis. Fifty such patients were examined with computed tomography (CT) of the brain. The findings were compared with those of an age-matched reference group selected at random to represent normal subjects. The CT examinations were analyzed with respect to size of lateral ventricles and signs of atrophy. In both groups, there was a significant increase of atrophy with age. There was also a significantly higher degree of atrophy in the patient group as compared with the reference group. The findings indicate that the supposedly underlying venous vasculitis is related to early aging and atrophy of the brain. (orig.)

  12. Isolated breast vasculitis manifested as breast edema with suggestive sonographic findings: a case report with imaging findings.

    Science.gov (United States)

    Lee, Ji Young; Joo, Mee

    2017-04-01

    Early diagnosis of breast vasculitis (BV) is difficult because this condition is rare and occasionally mimics breast cancer clinically or radiologically. It may present as systemic disease or as an isolated lesion in the breast, without systemic evidence. When vasculitis appears in the breast, it also might manifest as a tumor-like lesion, and in previous cases, tissue acquisition was needed for confirmation of the diagnosis because of BV's resemblance to inflammatory breast cancer. We report a case of isolated BV that was suspected of being inflammatory breast cancer clinically, but manifested as bilateral breast edema on mammography. In this case, sonographic findings included not only nonspecific edema findings that might be seen in other cases, but also suggestive findings of hypoechoic circumferential arterial wall thickening with perivascular fat infiltrations that are similar to the halo sign in large arteries but have not been reported in the breast. These are helpful for presumptive diagnosis of BV using ultrasound.

  13. Early treatment with infliximab in bilateral occlusive vasculitis as a presenting manifestation of Behçet' disease.

    Science.gov (United States)

    Bañeros-Rojas, P; Berrozpe-Villabona, C; Peraza-Nieves, J E; Díaz-Valle, D

    2015-06-01

    A 24 year old woman who complained of maculo-papulo rash, genital and bucal aphthous ulcers, abdominal pain, minor dyspnea and visual loss in both eyes. Funduscopy revealed a bilateral occlusive vasculitis including central vessels. Treatment was initiated with a methylprednisolone bolus (1 g/24h) and infliximab 5mg/kg/day (0-2-6 weeks and every 8 weeks). The treatment prescribed induced a fast remission. Visual acuity improved. The patient did not suffer any other relapse after one year of follow-up. An initial treatment with Infliximab should be considered in Behçet disease for serious outbreaks, such as macular occlusive vasculitis with ischemia. Copyright © 2013 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.

  14. Crohns disease with central nervous system vasculitis causing subarachnoid hemorrhage due to aneurysm and cerebral ischemic stroke.

    Science.gov (United States)

    Garge, Shaileshkumar S; Vyas, Pooja D; Modi, Pranav D; Ghatge, Sharad

    2014-10-01

    Cerebral vasculitis secondary to Crohn's disease (CD) seems to be a very rare phenomenon. We report a 39-year-old male who presented with headache, vomiting, and left-sided weakness in the known case of CD. Cross-sectional imaging (computed tomography and magnetic resonance imaging,) showed right gangliocapsular acute infarct with supraclinoid cistern subarachnoid hemorrhage (SAH). Cerebral digital substraction angiography (DSA) showed dilatation and narrowing of right distal internal carotid artery (ICA). Left ICA was chronically occluded. His inflammatory markers were significantly raised. Imaging features are suggestive of cerebral vasculitis. Arterial and venous infarcts due to thrombosis are known in CD. Our case presented with acute subarachnoid hemorrhage in supraclinoid cistern due to rupture of tiny aneurysm of perforator arteries causing SAH and infarction in right basal ganglia. Patient was treated conservatively with immunosuppression along with medical management of SAH.

  15. Crohns disease with central nervous system vasculitis causing subarachnoid hemorrhage due to aneurysm and cerebral ischemic stroke

    Directory of Open Access Journals (Sweden)

    Shaileshkumar S Garge

    2014-01-01

    Full Text Available Cerebral vasculitis secondary to Crohn′s disease (CD seems to be a very rare phenomenon. We report a 39-year-old male who presented with headache, vomiting, and left-sided weakness in the known case of CD. Cross-sectional imaging (computed tomography and magnetic resonance imaging, showed right gangliocapsular acute infarct with supraclinoid cistern subarachnoid hemorrhage (SAH. Cerebral digital substraction angiography (DSA showed dilatation and narrowing of right distal internal carotid artery (ICA. Left ICA was chronically occluded. His inflammatory markers were significantly raised. Imaging features are suggestive of cerebral vasculitis. Arterial and venous infarcts due to thrombosis are known in CD. Our case presented with acute subarachnoid hemorrhage in supraclinoid cistern due to rupture of tiny aneurysm of perforator arteries causing SAH and infarction in right basal ganglia. Patient was treated conservatively with immunosuppression along with medical management of SAH.

  16. Controle de estoque de peças de reposição em local único: uma revisão da literatura Single location spare parts inventory control: the state of the art

    Directory of Open Access Journals (Sweden)

    José Roberto do Rego

    2011-01-01

    Full Text Available Estoques de peças de reposição atendem necessidades de manutenção e reparo de produtos de consumo, veículos, máquinas e equipamentos industriais, acarretando frequentemente altos custos de capital e forte impacto no nível de serviço aos clientes. A grande multiplicidade de componentes, com ciclos de vida mais curtos e baixas demandas dificultam a gestão destes estoques. Este artigo apresenta revisão da literatura sobre modelos de controle de estoques de peças de reposição em local único, abordando tanto a previsão de demanda quanto o controle de estoques nos diferentes estágios do ciclo de vida das peças. A partir do levantamento bibliográfico, identificam-se algumas lacunas referentes à decisão de estocagem ou não de um item, elaboração de pedidos iniciais e finais, na integração de modelos de previsão de demanda com o controle de estoque e também de estudos de caso na aplicação prática dos modelos.Spare parts inventory are needed for maintenance and repair of final products, vehicles, industrial machines and equipments, frequently requiring high investments and significantly affecting customer satisfaction. Inventory management is complex due to the large number of different items and low demands. This article presents a literature review on single location spare parts inventory control, embracing both demand forecasting techniques and inventory control decisions on the different life cycle stages. Overall, the literature review identified the following research opportunities on inventory management: criteria to decide to stock or not an item, how many to order in the first and the last batch, demand forecasting and inventory control models integration and case studies on real applications.

  17. Exploration, Development, and Validation of Patient-reported Outcomes in Antineutrophil Cytoplasmic Antibody–associated Vasculitis Using the OMERACT Process

    Science.gov (United States)

    Robson, Joanna C.; Milman, Nataliya; Tomasson, Gunnar; Dawson, Jill; Cronholm, Peter F.; Kellom, Katherine; Shea, Judy; Ashdown, Susan; Boers, Maarten; Boonen, Annelies; Casey, George C.; Farrar, John T.; Gebhart, Don; Krischer, Jeffrey; Lanier, Georgia; McAlear, Carol A.; Peck, Jacqueline; Sreih, Antoine G.; Tugwell, Peter; Luqmani, Raashid A.; Merkel, Peter A.

    2016-01-01

    Objective Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of linked multisystem life- and organ-threatening diseases. The Outcome Measures in Rheumatology (OMERACT) vasculitis working group has been at the forefront of outcome development in the field and has achieved OMERACT endorsement of a core set of outcomes for AAV. Patients with AAV report as important some manifestations of disease not routinely collected through physician-completed outcome tools; and they rate common manifestations differently from investigators. The core set includes the domain of patient-reported outcomes (PRO). However, PRO currently used in clinical trials of AAV do not fully characterize patients’ perspectives on their burden of disease. The OMERACT vasculitis working group is addressing the unmet needs for PRO in AAV. Methods Current activities of the working group include (1) evaluating the feasibility and construct validity of instruments within the PROMIS (Patient-Reported Outcome Measurement Information System) to record components of the disease experience among patients with AAV; (2) creating a disease-specific PRO measure for AAV; and (3) applying The International Classification of Functioning, Disability and Health to examine the scope of outcome measures used in AAV. Results The working group has developed a comprehensive research strategy, organized an investigative team, included patient research partners, obtained peer-reviewed funding, and is using a considerable research infrastructure to complete these interrelated projects to develop evidence-based validated outcome instruments that meet the OMERACT filter of truth, discrimination, and feasibility. Conclusion The OMERACT vasculitis working group is on schedule to achieve its goals of developing validated PRO for use in clinical trials of AAV. (First Release September 1 2015; J Rheumatol 2015;42:2204–9; doi:10.3899/jrheum.141143) PMID:26329344

  18. Serum proteins reflecting inflammation, injury and repair as biomarkers of disease activity in ANCA-associated vasculitis

    Science.gov (United States)

    Monach, Paul A; Warner, Roscoe L; Tomasson, Gunnar; Specks, Ulrich; Stone, John H; Ding, Linna; Fervenza, Fernando C; Fessler, Barri J; Hoffman, Gary S; Iklé, David; Kallenberg, Cees GM; Krischer, Jeffrey; Langford, Carol A; Mueller, Mark; Seo, Philip; St. Clair, E William; Spiera, Robert; Tchao, Nadia; Ytterberg, Steven R; Johnson, Kent J; Merkel, Peter A

    2016-01-01

    Objective To identify circulating proteins that distinguish between active anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and remission in a manner complementary to markers of systemic inflammation. Methods Twenty-eight serum proteins representing diverse aspects of the biology of AAV were measured before and 6 months after treatment in a large clinical trial of AAV. Subjects (n=186) enrolled in the Rituximab in ANCA-Associated Vasculitis (RAVE) trial were studied. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were available for comparison. The primary outcome was the ability of markers to distinguish severe AAV (Birmingham Vasculitis Activity Score for Wegener’s granulomatosis (BVAS/WG)≥3 at screening) from remission (BVAS/WG=0 at month 6), using areas under receiver operating characteristic (ROC) curve (AUC). Results All subjects had severe active vasculitis (median BVAS/WG=8) at screening. In the 137 subjects in remission at month 6, 24 of the 28 markers showed significant declines. ROC analysis indicated that levels of CXCL13 (BCA-1), matrix metalloproteinase-3 (MMP-3) and tissue inhibitor of metalloproteinases-1 (TIMP-1) best discriminated active AAV from remission (AUC>0.8) and from healthy controls (AUC>0.9). Correlations among these markers and with ESR or CRP were low. Conclusions Many markers are elevated in severe active AAV and decline with treatment, but CXCL13, MMP-3 and TIMP-1 distinguish active AAV from remission better than the other markers studied, including ESR and CRP. These proteins are particularly promising candidates for future studies to address unmet needs in the assessment of patients with AAV. PMID:22975753

  19. The impact of 18F-FDG PET on the management of patients with suspected large vessel vasculitis

    International Nuclear Information System (INIS)

    Fuchs, Martin; Rasch, Helmut; Berg, Scott; Ng, Quinn K.T.; Mueller-Brand, Jan; Walter, Martin A.; Briel, Matthias; Daikeler, Thomas; Tyndall, Alan; Walker, Ulrich A.; Raatz, Heike; Jayne, David; Koetter, Ina; Blockmans, Daniel; Cid, Maria C.; Prieto-Gonzalez, Sergio; Lamprecht, Peter; Salvarani, Carlo; Karageorgaki, Zaharenia; Watts, Richard; Luqmani, Raashid

    2012-01-01

    We aimed to assess the impact of 18 F-fluorodeoxyglucose (FDG) positron emission tomography (PET) on the management of patients with suspected large vessel vasculitis. An international expert panel determined diagnoses and clinical management in patients with suspected large vessel vasculitis, with and without the results of 18 F-FDG PET, respectively. The accuracy of the clinical diagnosis and the resulting clinical management with and without the 18 F-FDG PET results were compared using logistic regression models. The analysis included 30 patients referred to a tertiary care centre with large vessel vasculitis and 31 controls. 18 F-FDG PET had an overall sensitivity of 73.3% [95% confidence interval (CI) 54.1-87.7%], a specificity of 83.9% (95% CI 66.3-94.5%), a positive predictive value of 81.5% (95% CI 61.9-93.7%) and a negative predictive value of 76.5% (95% CI 58.8-89.3%). The diagnostic accuracy of 18 F-FDG PET was higher in patients not receiving immunosuppressive drugs (93.3 vs 64.5%, p = 0.006). Taken in context with other available diagnostic modalities, the addition of 18 F-FDG PET increased the clinical diagnostic accuracy from 54.1 to 70.5% (p = 0.04). The addition of 18 F-FDG PET increased the number of indicated biopsies from 22 of 61 patients (36.1%) to 25 of 61 patients (41.0%) and changed the treatment recommendation in 8 of 30 patients (26.7%) not receiving immunosuppressive medication and in 7 of 31 patients (22.6%) receiving immunosuppressive medication. 18 F-FDG PET is a sensitive and specific imaging tool for large vessel vasculitis, especially when performed in patients not receiving immunosuppressive drugs. It increases the overall diagnostic accuracy and has an impact on the clinical management in a significant proportion of patients. (orig.)

  20. Cutaneous vasculitis in patients with autoimmune polyendocrine syndrome type 1: report of a case and brief review of the literature.

    Science.gov (United States)

    Improda, Nicola; Capalbo, Donatella; Cirillo, Emilia; Cerbone, Manuela; Esposito, Andrea; Pignata, Claudio; Salerno, Mariacarolina

    2014-11-01

    Autoimmune polyendocrine syndrome type 1, also known as autoimmune polyendocrinopathy-candidiasis-ectodermal-dystrophy, is a rare autosomal recessive disease due to pathogenic variants in the AIRE gene. Classic features of the syndrome are mucocutaneous candidiasis, chronic idiopathic hypoparathyroidism and Addison disease. However, other endocrine and non-endocrine components, may occur with a different prevalence. In addition to ectodermal features, which are quite common features of the disease, APS 1 patients may experience other types of skin alterations, such as vasculitic skin rash. An early diagnosis of APS 1 can be very challenging, due to the high clinical heterogeneity, and a considerable delay may occur between the appearance of symptoms and the diagnosis. We report on a girl affected by APS 1 who presented with cutaneous vasculitis when she was seven-months old, some years before the onset of the common components of the disease. Clinical picture of APS 1 may be characterized by isolated rare or atypical autoimmune or immune-mediated manifestations, even years before the onset of the classic components of the disease. Among these uncommon features, skin rashes of variable form and duration may occur, most of them being associated with histopathological features of vasculitis. Our case suggests that cutaneous vasculitis may represent a first sign of APS 1. The clinical significance of cutaneous vasculitis in the context of APS 1 is still debated. It may represent a rare, unusual, early component of the disease or a clinical manifestation secondarily related to the typical APS 1 components (i.e. autoimmune thyroid disease), which are frequently associated with rheumatologic-like signs and symptoms. Alternatively, it may be the expression of an independent disease co-occuring with APS 1. In conclusion, our case suggests that children presenting with unexplained vasculitic skin rash should be followed-up in order to early identify APS 1.

  1. Onderzoek Location Based Marketing: Mobile = location = effect

    NARCIS (Netherlands)

    Gisbergen, M.S. van; Huhn, A.E.; Khan, V.J.; Ketelaar, P.E.

    2011-01-01

    Onderzoekers van de NHTV (Internationaa Hoger Onderwijs Breda, Radboud Universiteit, DVJ Insights en Popai Benelux lieten consumenten in een virtuele supermarkt advertenties via de smartphone ontvangen wanneer men langs het geadverteerde product liep. De uitkomsten laten zien dat 'location based

  2. Epstein-Barr virus-related cutaneous necrotizing vasculitis in a girl heterozygous for factor V Leiden.

    Science.gov (United States)

    Guerriero, Cristina; Moretta, Gaia; Bersani, Giulia; Valentini, Piero; Gatto, Antonio; Rigante, Donato

    2017-12-01

    Necrotizing vasculitides are basically characterized by vessel wall neutrophil infiltration and necrosis and they can occur as a primary process or secondary to an underlying disease. Although Henoch-Schönlein purpura (HSp) is the more frequent primary vasculitis in childhood, sometimes it has to be distinguished from other secondary vasculitides induced by infections, drugs, vaccines, or immune-mediated disorders. We report a case of a 14-year-old girl with cutaneous necrotizing vasculitis, appearing in the course of acute Epstein-Barr virus infection. Physical examination revealed highly aching erythematous-purple lesions with reticular edges localized on the back of feet. Pain was non-responsive to ibuprofen and required administration of tapentadol and pregabalin. The patient was also heterozygous for factor V Leiden that might have contributed to the development of cutaneous painful lesions. To our knowledge this is the first documented pediatric case of necrotizing vasculitis associated with acute EBV infection in a girl heterozygous for factor V Leiden. In this patient the severity of skin manifestations might have been influenced by the concomitant factor V Leiden, which gave rise to hypercoagulability and occlusive vasculopathy with markedly severe pain, a symptom rather infrequent in other childhood vasculitides.

  3. Parthenium dermatitis manifesting clinically as polymorphic light eruption and prurigo nodularis- like lesions with vasculitis-like picture on histopathology

    Directory of Open Access Journals (Sweden)

    Chembolli Lakshmi

    2011-01-01

    Full Text Available Parthenium dermatitis is a widespread and distressing dermatoses in rural and urban India caused by the air borne allergen of the Compositae weed Parthenium hysterophorus. Parthenium dermatitis has been thought to be mediated solely by type IV hypersensitivity, but recently a combined immediate (type I and delayed (type IV hypersensitivity mechanism has been postulated in the initiation and perpetuation of parthenium dermatitis, especially in sensitized subjects with an atopic diathesis. Initially, the exposed sites of the body are involved. Later in the course of the disease, unexposed sites may get involved. Various clinical presentations have been described in parthenium dermatitis. Typically, it presents as an air borne contact dermatitis (ABCD involving the eyelids and nasolabial folds Other presentations include a photodermatitis (essentially a pseudo photodermatitis, atopic dermatitis, seborrheic dermatitis, exfoliative dermatitis, hand dermatitis. Photosensitive lichenoid dermatitis and prurigo nodularis are rarer presentations. Uncommon presentations have been described in parthenium dermatitis. They include prurigo nodularis-like lesions and photosensitive lichenoid eruption. Three cases are presented, two of whom presented as polymorphic-like lesions and one as prurigo nodularis. All three patch tested positive to parthenium on Day 2. Prick testing was positive in two of the three patients. Parthenium dermatitis mimicking polymorphic light eruption has not been reported. Histopathology revealed vasculitis in the lesional skin in two of the patients. Although leukocytoclastic vasculitis has been reported earlier from the prick-tested site, this is the first report demonstrating the presence of vasculitis in lesional skin of parthenium dermatitis.

  4. Effect of rituximab on malignancy risk in patients with ANCA-associated vasculitis.

    Science.gov (United States)

    van Daalen, Emma E; Rizzo, Raffaella; Kronbichler, Andreas; Wolterbeek, Ron; Bruijn, Jan A; Jayne, David R; Bajema, Ingeborg M; Rahmattulla, Chinar

    2017-06-01

    Patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) treated with cyclophosphamide have an increased malignancy risk compared with the general population. We investigated whether treatment with rituximab instead of cyclophosphamide has decreased the malignancy risk in patients with AAV. The study included patients with AAV treated at a tertiary vasculitis referral centre between 2000 and 2014. The malignancy incidence in these patients was compared with the incidence in the general population by calculating standardised incidence ratios (SIRs), adjusted for sex, age and calendar year. Malignancy incidence was compared between rituximab-treated and cyclophosphamide-treated patients. Of the 323 included patients, 33 developed a total of 45 malignancies during a mean follow-up of 5.6 years. This represented a 1.89-fold increased (95% CI 1.38 to 2.53) malignancy risk, and a non-significantly increased risk if non-melanoma skin cancer was excluded (SIR, 1.09; 95% CI 0.67 to 1.69). The risk of non-melanoma skin cancer was 4.58-fold increased (95% CI 2.96 to 6.76). Cyclophosphamide-treated patients had an increased malignancy risk compared with the general population (SIR, 3.10; 95% CI 2.06 to 4.48). In contrast, rituximab-treated patients had a malignancy risk similar to the general population (SIR, 0.67; 95% CI 0.08 to 2.43). The malignancy risk in cyclophosphamide-treated patients was 4.61-fold higher (95% CI 1.16 to 39.98) than in rituximab-treated patients. The malignancy risk in patients with AAV was lower in rituximab-treated patients than in cyclophosphamide-treated patients. Notably, rituximab treatment was not associated with an increased malignancy risk compared with the general population. Rituximab could therefore be a safe alternative to cyclophosphamide in the treatment of AAV. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  5. Spotlight on rituximab in the treatment of antineutrophil cytoplasmic antibody-associated vasculitis: current perspectives

    Directory of Open Access Journals (Sweden)

    Moog P

    2015-11-01

    Full Text Available Philipp Moog, Klaus Thuermel Abteilung für Nephrologie, Klinikum rechts der Isar, Technische Universität München, Munich, Germany Abstract: A 54-year-old patient presented to his general practitioner because of strong muscle pain in both thighs. Inflammatory parameters (CRP 16.3 mg/dL and white blood cells (15 g/L were elevated. The patient reported a weight loss of 10 kg in 4 weeks. There was no fever or any other specific symptoms. Urine dipstick examination and computed tomography of the chest were unremarkable. Because of increasing symptoms, the patient was referred to our department. Magnetic resonance tomography showed diffuse inflammatory changes of the muscles of both thighs. Neurological examination and electrophysiology revealed axonal sensorimotor neuropathy and ground-glass opacities of both lungs had occurred. Serum creatinine increased to 229 µmol/L within a few days, with proteinuria of 3.3 g/g creatinine. Kidney biopsy showed diffuse pauci-immune proliferative glomerulonephritis. Proteinase 3-specific antineutrophil cytoplasmic antibodies were markedly increased. Birmingham Vasculitis Activity Score was 35. Within 2 days, serum creatinine further increased to 495 µmol/L. Plasma exchange, high-dose glucocorticosteroids, and hemodialysis were started. The patient received cyclophosphamide 1 g twice and rituximab 375 mg/m2 four times according to the RITUXVAS protocol. Despite ongoing therapy, hemodialysis could not be withdrawn and had to be continued over 3 weeks until diuresis normalized. Glucocorticosteroids were tapered to 20 mg after 2 months, and serum creatinine was 133 µmol/L. However, nephritic urinary sediment reappeared. Another dose of 1 g cyclophosphamide was given, and glucocorticosteroids were raised for another 4 weeks. After 6 months, the daily prednisolone dose was able to be tapered to 5 mg. Serum creatinine was 124 µmol/L, proteinuria further decreased to 382 mg/g creatinine, and the Birmingham

  6. Peripheral CD5+ B Cells in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

    Science.gov (United States)

    Unizony, Sebastian; Lim, Noha; Phippard, Deborah J.; Carey, Vincent J.; Miloslavsky, Eli M.; Tchao, Nadia K.; Iklé, David; Asare, Adam L.; Merkel, Peter A.; Monach, Paul A.; Seo, Philip; St Clair, E. William; Langford, Carol A.; Spiera, Robert; Hoffman, Gary S.; Kallenberg, Cees G. M.; Specks, Ulrich; Stone, John H.

    2015-01-01

    Objective CD5+ B cells have been conceptualized as a possible surrogate for Breg cells. The aim of the present study was to determine the utility of CD5+ B cells as biomarkers in antineutrophil cytoplasmic antibody–associated vasculitis (AAV). Methods The absolute and relative numbers (percentages) of CD5+ B cells (explanatory variables) were measured longitudinally during 18 months in 197 patients randomized to receive either rituximab (RTX) or cyclophosphamide (CYC) followed by azathioprine (AZA) for the treatment of AAV (Rituximab in ANCA-Associated Vasculitis [RAVE] trial). Outcome variables included disease activity (status of active disease versus complete remission), responsiveness to induction therapy, disease relapse, disease severity, and, in RTX-treated patients, relapse-free survival according to the percentage of CD5+ B cells detected upon B cell repopulation. Results CD5+ B cell numbers were comparable between the treatment groups at baseline. After an initial decline, absolute CD5+ B cell numbers progressively increased in patients in the RTX treatment arm, but remained low in CYC/AZA-treated patients. In both groups, the percentage of CD5+ B cells increased during remission induction and slowly declined thereafter. During relapse, the percentage of CD5+ B cells correlated inversely with disease activity in RTX-treated patients, but not in patients who received CYC/AZA. No significant association was observed between the numbers of CD5+ B cells and induction treatment failure or disease severity. The dynamics of the CD5+ B cell compartment did not anticipate disease relapse. Following B cell repopulation, the percentage of CD5+ B cells was not predictive of time to flare in RTX-treated patients. Conclusion The percentage of peripheral CD5+ B cells might reflect disease activity in RTX-treated patients. However, sole staining for CD5 as a putative surrogate marker for Breg cells did not identify a subpopulation of B cells with clear potential for

  7. Clinical Outcomes of Remission Induction Therapy for Severe Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

    Science.gov (United States)

    Miloslavsky, E. M.; Specks, U.; Merkel, P. A.; Seo, P.; Spiera, R.; Langford, C. A.; Hoffman, G. S.; Kallenberg, C. G. M.; St.Clair, E. W.; Tchao, N. K.; Viviano, L.; Ding, L.; Sejismundo, L. P.; Mieras, K.; Iklé, D.; Jepson, B.; Mueller, M.; Brunetta, P.; Allen, N. B.; Fervenza, F. C.; Geetha, D.; Keogh, K.; Kissin, E. Y.; Monach, P. A.; Peikert, T.; Stegeman, C.; Ytterberg, S. R.; Stone, J. H.

    2016-01-01

    Objective To evaluate the reasons that complete remission is not achieved or maintained with original treatment in some patients with antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) treated with rituximab (RTX) or with cyclophosphamide/azathioprine (CYC/AZA). Methods The Rituximab in AAV trial was a randomized, double-blind, placebo-controlled trial comparing the rate of remission induction among patients treated with RTX (n = 99) and patients treated with CYC followed by AZA (n = 98). Glucocorticoids were tapered over a period of 5 months. The primary outcome measure was lack of disease activity without glucocorticoid treatment at 6 months. To determine the most important reason for failure to achieve the primary outcome, 7 hierarchical categories of reasons were defined retrospectively (uncontrolled disease, adverse event leading to therapy discontinuation, severe flare, limited flare, Birmingham Vasculitis Activity Score for Wegener’s Granulomatosis >0, prednisone treatment at any dosage, and other). Results Although remission (lack of disease activity) was achieved in 170 of the 197 patients (86%) in the first 6 months, the primary outcome measure was not achieved in 42%. There were 3 deaths. Twenty-four percent of the patients failed to achieve the primary end point due to active disease: 10 (5%) experienced uncontrolled disease in the first month and 37 (19%) experienced flares after initial improvement. In the majority of such patients, treatment with blinded crossover or according to best medical judgment led to disease control. Ninety-one percent of patients who had uncontrolled disease or experienced a severe flare had proteinase 3 (PR3)–ANCA. When patients with uncontrolled disease were excluded from analysis, those who were PR3-ANCA positive were found to experience fewer flares when treated with RTX compared to CYC/AZA (8 of 59 [14%] versus 20 of 62 [32%]; P = 0.02). Neither ANCA titers nor B cell counts predicted disease

  8. Cutaneous vasculitis in systemic lupus erythematosus: association with anti-ribosomal P protein antibody and Raynaud phenomenon.

    Science.gov (United States)

    Shinjo, Samuel Katsuyuki; Bonfá, Eloísa

    2011-02-01

    Ninety-one consecutive systemic lupus erythematosus (SLE) patients (American College of Rheumatology criteria) with a history of cutaneous vasculitis were compared to 163 SLE controls without this clinical manifestation from July to December 2007 in order to determine the possible clinical and serological association of this manifestation. Data were obtained in an ongoing electronic database protocol and autoantibodies to anti-double-stranded DNA, anti-Sm, anti-RNP, anti-Ro/SS-A, anti-La/SS-B, and anticardiolipin and ribosomal P protein antibody (anti-P) were detected by standard techniques. Exclusion criteria were the presence of anti-phospholipid syndrome or antibodies, Sjögren syndrome, and a history of thrombosis. The mean age (38.5 ± 11.5 vs. 37.8 ± 11.6 years, p = 0.635), disease duration (12.5 ± 7.8 vs. 11.8 ± 7.9 years, p = 0.501), and frequency of white race (71.4% vs. 70.5%, p = 0.872) and female sex (96.8% vs. 93.7%, p = 0.272) were comparable in both groups. The vasculitis group had a higher frequency of malar rash (97.9% vs. 87.4%, p = 0.004), photosensitivity (91.4% vs. 81.6%, p = 0.030), and Raynaud phenomenon (RP; 27.7% vs. 7.5%, p < 0.001), whereas all other clinical manifestation including renal and central nervous system involvements were similar to the control group. Laboratorial data revealed that only anti-P (35.1% vs. 12.1%, p < 0.001) was more frequent in patients with vasculitis. In a multivariate logistic regression model, cutaneous vasculitis was associated to the presence of RP (OR = 3.70; 95% confidence interval [CI] = 1.73-8.00) and anti-P (OR = 3.42; 95% CI = 1.76-6.66). In summary, SLE cutaneous vasculitis characterizes a subgroup of patients with more RP and anti-P antibodies but not accompanied by a higher frequency of renal and central nervous system involvements.

  9. Facility Location with Double-peaked Preferences

    DEFF Research Database (Denmark)

    Filos-Ratsikas, Aris; Li, Minming; Zhang, Jie

    2015-01-01

    We study the problem of locating a single facility on a real line based on the reports of self-interested agents, when agents have double-peaked preferences, with the peaks being on opposite sides of their locations. We observe that double-peaked preferences capture real-life scenarios and thus...... complement the well-studied notion of single-peaked preferences. We mainly focus on the case where peaks are equidistant from the agents’ locations and discuss how our results extend to more general settings. We show that most of the results for single-peaked preferences do not directly apply to this setting...

  10. Vasculites pulmonares: novas visões de uma velha conhecida Pulmonary forms of vasculitis: new perspectives on an old acquaintance

    Directory of Open Access Journals (Sweden)

    Thais Thomaz Queluz

    2005-07-01

    Full Text Available A vasculite necrosante foi descrita em 1866 e seu espectro é muito amplo, uma vez que acomete vasos arteriais e venosos de todos os calibres e de vários órgãos, apresenta diversos tipos de infiltrados inflamatórios, tem um significante número de manifestações clínicas e pode ter ou não fatores desencadeantes identificáveis. A sempre controversa classificação das vasculites mudou radicalmente com a descoberta dos anticorpos anticitoplasma de neutrófilos em 1982, contemplando atualmente a doença de Goodpasture, as vasculites associadas aos anticorpos anticitoplasma de neutrófilos, as vasculites por imunocomplexos e outros tipos de vasculites. As evidências de que os anticorpos anticitoplasma de neutrófilos estão envolvidos na patogênese destas lesões trouxeram avanços consideráveis para o seu diagnóstico e tratamento. Granulomatose de Wegener, doença de Churg-Strauss e poliangeíte microscópica, todas vasculites associadas aos anticorpos anticitoplasma de neutrófilos, são as vasculites sistêmicas que mais acometem os pulmões. Suas manifestações clínicas comuns são tosse, hemorragia alveolar difusa ou asma de difícil controle. Na arterite de Takayasu, na doença de Behçet, na púrpura de Henoch-Schönlein e nas vasculites associadas às doenças do colágeno o acometimento pulmonar é mais raro. Em todos os casos há evidências de serem processos de origem imunológica e com base neste princípio são propostas as abordagens terapêuticas.Necrotizing vasculitis was first described in 1866. The condition encompasses a wide spectrum of symptoms, affecting arterial blood vessels of various calibers and in various organs. In addition, it is associated with many types of inflammatory infiltrate, and presents a significant number of clinical manifestations. The causative factor or factors may or may not be identifiable. The eternally controversial classification of the various forms of vasculitis changed radically

  11. Kaposi's sarcoma with HHV8 infection and ANCA-associated vasculitis in a hemodialysis patient.

    Science.gov (United States)

    Fatma, Lilia Ben; Rais, Lamia; Mebazza, Amel; Azzouz, Haifa; Beji, Somaya; Krid, Madiha; Smaoui, Wided; Maiz, Hedi Ben; Zouaghi, Karim; Zitouna, Moncef; Osmane, Amel Ben; Moussa, Fatma Ben

    2013-11-01

    The association between Kaposi's sarcoma (KS) and human herpes virus eight (HHV-8) infection is rarely reported in hemodialysis (HD) patients. We report here the rare association of KS, HHV-8 and hepatitis C virus (HCV) infection as well as syphilis in a HD patient. We report the case of a 72-year-old woman who presented with microscopic polyangiitis with alveolar hemorrhage and pauci-immune necrosing and crescentic glomerulonephritis as well as renal failure requiring HD. Biological tests showed positive HCV and syphilis tests. The patient was treated by HD and intravenous pulse, followed by oral corticosteroids and six cyclophosphamide monthly pulses with remission of the alveolar hemorrhage, but without renal functional recovery as the patient remained HD dependent. Five months after the first treatment administration, she developed extensive purpuric lesions on her lower limbs, abdomen face and neck. A skin biopsy showed KS. The HHV-8 test was positive, with positive polymerase chain reaction-HHV8 in the serum and skin. After immunosuppression withdrawal, the KS skin lesions regressed rapidly without relapse after 12 months of follow-up, but alveolar hemorrhage relapsed after 16 months of follow-up. Our case showed that the immunosuppressed state related to multiple factors such as aging, vasculitis, HHV-8, HCV, syphilis, immunosuppressive therapy and HD may all have contributed to the development of KS in our patient.

  12. Valvular heart disease in patients with hypocomplementemic urticarial vasculitis syndrome associated with Jaccoud's arthropathy.

    Science.gov (United States)

    Houser, S L; Askenase, P W; Palazzo, E; Bloch, K J

    2002-01-01

    Since 1973, more than 75 patients with hypocomplementemic urticarial vasculitis syndrome (HUVS) were reported, but valvular heart disease does not seem to have been noted in these patients. Since 1993, however, five patients with HUVS accompanied by Jaccoud's arthropathy (JA) were found to have serious valvular heart disease. To characterize the cardiac valvulopathy of the third patient with HUVS/JA to have undergone valve replacement, this study included the use of routine and special tissue stains, as well as immunohistochemical staining. We compared gross and histologic findings of this patient's valve to those of two other patients with this complex syndrome who underwent valve replacement. Pathologic findings of these latter two patients were described in separate earlier reports. Histologic examination of the resected valves in all three patients showed an acute necrotizing endocarditis and fibrin deposition on the surface of valve leaflets. Beneath the surfaces of the leaflets, there was evidence of chronic inflammation, consisting of lymphocytes and histiocytes. A fibrocalcific degenerative change was also present in all three valves. Positive staining for IgG, IgA, IgM, and light-chain determinant-bearing proteins was detected primarily at the valve surface in special studies of the aortic valve of the patient described in the current report. Patients with HUVS and associated JA should be evaluated for the presence of valvular heart disease. The latter is probably a nonrheumatic, inflammatory, and degenerative process, mediated by immune complex, as well as cellular immune mechanisms.

  13. Cutaneous vasculitis in systemic lupus erythematosus patients: potential key players and implications.

    Science.gov (United States)

    Gheita, T A; Abaza, N M; Sayed, S; El-Azkalany, G S; Fishawy, H S; Eissa, A H

    2017-01-01

    Objectives The aim of the present work was to study the clinical characteristics of cutaneous vasculitis (CV) in systemic lupus erythematosus (SLE) patients and find possible potential key players in its development and implicated associations with the disease manifestations. Patients and methods Fifty adult female SLE patients underwent full history taking, thorough clinical examination and laboratory investigations. The SLE Disease Activity Index (SLEDAI) and accumulated damage using the Systemic Lupus International Collaborative Clinics/American College of Rheumatology Damage Index (SLICC/ACR DI) were assessed. Results The mean age of the patients was 29.1 ± 6.1 years and was significantly lower in those with CV ( p = 0.018). The disease duration was 4.9 ± 3.7 years. CV was present in 30% of the patients. Musculoskeletal manifestations and hypocomplementemia were present in all patients with CV. The SLEDAI and SLICC/ACR DI tended to be higher in those with CV. Complement (C3 and C4) was significantly consumed in CV patients ( p Lupus nephritis, cardiovascular manifestations and Sjögren syndrome were significantly linked to the development of CV ( p = 0.025, p = 0.023 and p lupus nephritis, musculoskeletal manifestations and Sjögren syndrome.

  14. A Case of Presumed Tuberculosis Uveitis with Occlusive Vasculitis from an Endemic Region

    Directory of Open Access Journals (Sweden)

    Berna Başarır

    2017-06-01

    Full Text Available In this report, we present a case with presumed unilateral tuberculosis uveitis from an endemic region. A 23-year-old male presented with decreased vision in his left eye for 15 days. Visual acuities were 1.0 in his right eye and 0.3 in his left eye. Ophthalmologic examination was normal for the right eye. Slit-lamp examination revealed 2+ cells in the vitreous without anterior chamber reaction in his left eye. Fundus examination revealed occlusive vasculitis and granuloma. His history revealed that he had a respiratory infection with fever 3 months ago while visiting his native country, Rwanda, and was treated with non-specific antibiotic therapy. His visual symptom started 2 weeks after his systemic symptoms resolved. Laboratory findings included 15 mm induration in purified protein derivative tuberculin skin test, HIV negativity, and parenchymal lesions in chest X-ray. Bronchoalveolar lavage was negative for acid-fast bacillus. A pulmonary disease consultant reported presumed tuberculosis because of the patient’s history. Anti-tuberculosis treatment was initiated. The patient’s visual acuity improved rapidly and his signs regressed. A careful history should be taken from patients with uveitis. Travel to tuberculosis-endemic areas may be important for diagnosis and should be asked about directly.

  15. Renal manifestations in hypocomplementic urticarial vasculitis syndrome: Is it a distinct pathology?

    Directory of Open Access Journals (Sweden)

    Badriya AlHermi

    2017-01-01

    Full Text Available Hypocomplementic urticarial vasculitis syndrome (HUVS is an autoimmune disease characterized by recurrent urticaria, arthritis, and glomerulonephritis (GN. Anti-C1q antibody is the marker of HUVS together with low levels of classical pathway complements which are C2, C3, C4, and C1q. We report a case of a 6-year-old boy who presented with episodes of rashes, injected conjunctiva, abdominal pain, and arthritis, diagnosed as HUVS. He had low C3, low CH50, normal C4, and positive C1q antibody. His urinalysis showed intermittent microscopic hematuria only. One year later, his laboratories showed persistent low C3 and positive Anti-ds DNA. The urinalysis showed hematuria, pyuria, and nephrotic-range proteinuria. Urine protein to creatinine ratio was 101.8 h mg/mmol. Kidney biopsy showed mesangioproliferative GN consistent with the diagnosis of HUVS. The patient was treated initially with prednisolone then azathioprine was added to the regimen. He showed good response with the disappearance of hematuria and proteinuria. Nine months later, he had no skin rashes with normal urinalysis and normal anti-ds DNA antibody. We report a case with HUVS and GN with positive anti-dsDNA antibody that revealed good response to combination of immunosuppressive therapy.

  16. Law Enforcement Locations

    Data.gov (United States)

    Kansas Data Access and Support Center — Law Enforcement Locations in Kansas Any location where sworn officers of a law enforcement agency are regularly based or stationed. Law enforcement agencies "are...

  17. Appraising manufacturing location

    NARCIS (Netherlands)

    Steenhuis, H.J.; de Bruijn, E.J.

    2002-01-01

    International location of manufacturing activities is an issue for managers of manufacturing companies as well as public policy makers. For managers, the issue is relevant because international locations offer opportunities for lowering costs due to productivity improvements. For governments the

  18. Location | FNLCR Staging

    Science.gov (United States)

    The Frederick National Laboratory for Cancer Research campus is located 50 miles northwest of Washington, D.C., and 50 miles west of Baltimore, Maryland, in Frederick, Maryland. Satellite locations include leased and government facilities extending s

  19. Location, Location, Location: Where Do Location-Based Services Fit into Your Institution's Social Media Mix?

    Science.gov (United States)

    Nekritz, Tim

    2011-01-01

    Foursquare is a location-based social networking service that allows users to share their location with friends. Some college administrators have been thinking about whether and how to take the leap into location-based services, which are also known as geosocial networking services. These platforms, which often incorporate gaming elements like…

  20. Sleeping at work: not all about location, location, location.

    Science.gov (United States)

    Jay, Sarah M; Aisbett, Brad; Sprajcer, Madeline; Ferguson, Sally A

    2015-02-01

    Working arrangements in industries that use non-standard hours sometimes necessitate an 'onsite' workforce where workers sleep in accommodation within or adjacent to the workplace. Of particular relevance to these workers is the widely held (and largely anecdotal) assumption that sleep at home is better than sleep away, particularly when away for work. This narrative review explores the idea that sleep outcomes in these unique work situations are the product of an interaction between numerous factors including timing and duration of breaks, commute length, sleeping environment (noise, movement, vibration, light), circadian phase, demographic factors and familiarity with the sleep location. Based on the data presented in this review, it is our contention that the location of sleep, whilst important, is secondary to other factors such as the timing and duration of sleep periods. We suggest that future research should include measures that allow conceptualisation of other critical factors such as familiarity with the sleeping environment. Copyright © 2014 Elsevier Ltd. All rights reserved.