Hypersensitivity vasculitis

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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 ...

Vasculitis mimics.

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Molloy, Eamonn S; Langford, Carol A

2008-01-01

There are many disorders that may closely resemble the clinical, radiologic and/or pathologic features of the primary vasculitides. In this review, we focus on recently described and under-recognized syndromes that may mimic vasculitis. Hereditary causes of large-artery aneurysms such as Marfan's syndrome have long been recognized; recent years have seen a greater understanding of the genetics of Marfan's and other such disorders, including Loeys-Dietz syndrome and Ehler-Danlos syndrome type IV. Under-recognized mimics of medium-vessel vasculitis include segmental arterial mediolysis and Grange syndrome. A large number of entities can mimic small-vessel vasculitis. Recent descriptions of antibodies to human neutrophil elastase have provided insight into the occurrence of antineutrophil cytoplasmic antibodies in cocaine-induced midline destructive lesions. The differential diagnosis of cerebral vasculitis can be particularly difficult. Reversible cerebral vasoconstriction syndromes represent an important class of entities that can readily mimic cerebral vasculitis but have a very different management approach and outcome. The diagnosis of vasculitis requires careful assessment of all available clinical, laboratory, radiologic and pathologic information, and consideration of many competing differential diagnoses. Awareness of noninflammatory mimics of vasculitis is essential to avoid unnecessary and potentially harmful treatment with immunosuppressive agents.

Antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis

NARCIS (Netherlands)

Kallenberg, Cees G. M.

Purpose of reviews This review focuses on recent advance in the diagnosis pathogenesis and treatment of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis. Recent findings Antineutrophil cytoplasmic autoantibodies are closely associated with Wegener's granulomatosis and

Clinical Spectrum of Medium-Sized Vessel Vasculitis.

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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.

[Large vessel vasculitides].

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Morović-Vergles, Jadranka; Puksić, Silva; Gracanin, Ana Gudelj

2013-01-01

Large vessel vasculitis includes Giant cell arteritis and Takayasu arteritis. Giant cell arteritis is the most common form of vasculitis affect patients aged 50 years or over. The diagnosis should be considered in older patients who present with new onset of headache, visual disturbance, polymyalgia rheumatica and/or fever unknown cause. Glucocorticoides remain the cornerstone of therapy. Takayasu arteritis is a chronic panarteritis of the aorta ant its major branches presenting commonly in young ages. Although all large arteries can be affected, the aorta, subclavian and carotid arteries are most commonly involved. The most common symptoms included upper extremity claudication, hypertension, pain over the carotid arteries (carotidynia), dizziness and visual disturbances. Early diagnosis and treatment has improved the outcome in patients with TA.

Radioisotopic imaging in large vessel vasculitis; Apport de l'imagerie radioisotopique dans l'arterite inflammatoire des gros vaisseaux

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Huglo, D.; Lambert, M.; Hachulla, E.; Hatron, P.Y.; Steinling, M. [Lille Univ. Nord-de-France, 59 - Lille (France); Huglo, D.; Raynaud, F.; Petyt, G.; Prangere, T.; Steinling, M. [CHU de Lille, Service de Medecine Nucleaire et Imagerie Fonctionnelle, Hopital Huriez, 59 - Lille (France); Huglo, D. [Inserm U703, 59 - Lille (France); Lambert, M.; Hachulla, E.; Hatron, P.Y. [CHU de Lille, Service de Medecine Interne, 59 - Lille (France)

2009-08-15

The group of large vessel vasculitis includes giant cell arthritis and Takayasu disease. If mono photonic nuclear medicine can sometimes show some unusual patterns in these diseases, positron emission tomography with fluorodeoxyglucose seems have a special attract and provide some indications during the monitoring of these patients. However, most of these indications remain to be validated, specially to differentiate weak vascular wall inflammatory activities from physiological and/or atherosclerotic accumulation. (authors)

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

Cerebral Vasculitis

Directory of Open Access Journals (Sweden)

Fariborz Khorvash

2017-02-01

protocol for cerebral vasculitis should include initial MRI to assess the degree of parenchymal damage and to detect vessel wall changes, in particular in large-vessel vasculitis. If the results are ambiguous or medium-sized arteries are affected (beyond the spatial resolution of MRI DSA should follow. Small-vessel vasculitides entirely evade detection by vascular imaging and consequently require brain or leptomeningeal biopsy . Exclusion of differential diagnoses: Important differential diagnoses include RCVS,  intracranial atherosclerosis Moyamoy disease, autoimmune encephalopathies and infectious disorder such as varicella zoster virus (VZV vasculopathies or endocarditis. Therapy: Vasculitis is a serious disease that is potentially fatal or leads to permanent disability and requires rapid institution of immunosuppressive treatment. Possible therapeutic options include glucocorticoids, cyclophosphamide, azathioprine, intravenous immunoglobulins and mycophenolate mofetil.

Isolated pulmonary vasculitis: case report and literature review.

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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.

Large vessel involvement by IgG4-related disease

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Perugino, Cory A.; Wallace, Zachary S.; Meyersohn, Nandini; Oliveira, George; Stone, James R.; Stone, John H.

2016-01-01

Abstract Objectives: IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect multiple organs and lead to tumefactive, tissue-destructive lesions. Reports have described inflammatory aortitis and periaortitis, the latter in the setting of retroperitoneal fibrosis (RPF), but have not distinguished adequately between these 2 manifestations. The frequency, radiologic features, and response of vascular complications to B cell depletion remain poorly defined. We describe the clinical features, radiology findings, and treatment response in a cohort of 36 patients with IgG4-RD affecting large blood vessels. Methods: Clinical records of all patients diagnosed with IgG4-RD in our center were reviewed. All radiologic studies were reviewed. We distinguished between primary large blood vessel inflammation and secondary vascular involvement. Primary involvement was defined as inflammation in the blood vessel wall as a principal focus of disease. Secondary vascular involvement was defined as disease caused by the effects of adjacent inflammation on the blood vessel wall. Results: Of the 160 IgG4-RD patients in this cohort, 36 (22.5%) had large-vessel involvement. The mean age at disease onset of the patients with large-vessel IgG4-RD was 54.6 years. Twenty-eight patients (78%) were male and 8 (22%) were female. Thirteen patients (36%) had primary IgG4-related vasculitis and aortitis with aneurysm formation comprised the most common manifestation. This affected 5.6% of the entire IgG4-RD cohort and was observed in the thoracic aorta in 8 patients, the abdominal aorta in 4, and both the thoracic and abdominal aorta in 3. Three of these aneurysms were complicated by aortic dissection or contained perforation. Periaortitis secondary to RPF accounted for 27 of 29 patients (93%) of secondary vascular involvement by IgG4-RD. Only 5 patients demonstrated evidence of both primary and secondary blood vessel involvement. Of those treated with

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 ...

[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.

[Large vessels vasculopathy in systemic sclerosis].

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Tejera Segura, Beatriz; Ferraz-Amaro, Iván

2015-12-07

Vasculopathy in systemic sclerosis is a severe, in many cases irreversible, manifestation that can lead to amputation. While the classical clinical manifestations of the disease have to do with the involvement of microcirculation, proximal vessels of upper and lower limbs can also be affected. This involvement of large vessels may be related to systemic sclerosis, vasculitis or atherosclerotic, and the differential diagnosis is not easy. To conduct a proper and early diagnosis, it is essential to start prompt appropriate treatment. In this review, we examine the involvement of large vessels in scleroderma, an understudied manifestation with important prognostic and therapeutic implications. Copyright © 2015 Elsevier España, S.L.U. All rights reserved.

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

Adult primary angiitis of the central nervous system: isolated small-vessel vasculitis represents distinct disease pattern.

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de Boysson, Hubert; Boulouis, Grégoire; Aouba, Achille; Bienvenu, Boris; Guillevin, Loïc; Zuber, Mathieu; Touzé, Emmanuel; Naggara, Olivier; Pagnoux, Christian

2017-03-01

We aimed to identify whether presentations and outcomes in adult patients with isolated small-vessel primary angiitis of the CNS (PACNS) would differ from other patients with large/medium-vessel involvement. In the French PACNS cohort, we compared the characteristics, treatments and outcomes of patients with isolated small-vessel disease (normal CT, MR and/or conventional angiograms, brain biopsy positive for vasculitis) with other patients who had large/medium-vessel involvement (vessel abnormalities on CT, MR or conventional angiograms). A good functional outcome was defined as a modified Rankin scale ⩽2 at last follow-up, regardless of the occurrence of relapse. Among the 102 patients in the cohort, 26 (25%) had isolated small-vessel PACNS, whereas the 76 others demonstrated large/medium-vessel involvement. Patients with isolated small-vessel PACNS had more seizures (P adult patients with isolated small-vessel PACNS presented some distinct disease features and relapsed more often than other PACNS patients who had large/medium-vessel involvement. Functional outcomes and mortality did not differ. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com

Vasculitis

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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 ...

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

Vasculitis in the autoinflammatory diseases.

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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.

Central Nervous System Vasculitis

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

Endoscopic Findings of Upper Gastrointestinal Involvement in Primary Vasculitis.

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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.

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

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

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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.

The value of [18F]FDG-PET in the diagnosis of large-vessel vasculitis and the assessment of activity and extent of disease

International Nuclear Information System (INIS)

Walter, Martin A.; Mueller-Brand, Jan; Nitzsche, Egbert U.; Melzer, Ralph A.; Tyndall, Alan; Schindler, Christian

2005-01-01

This study was performed to investigate the value of 18 F-fluorodeoxyglucose positron emission tomography ([ 18 F]FDG-PET) in the diagnosis of large-vessel vasculitis and the assessment of activity and extent of disease. Twenty-six consecutive patients (21 females, 5 males; median age - years, range 17-86 years) with giant cell arteritis or Takayasu's arteritis were examined with [ 18 F]FDG-PET. Follow-up scans were performed in four patients. Twenty-six age- and gender-matched controls (21 females, 5 males; median age 71 years, range 17-86 years) were included. The severity of large-vessel [ 18 F]FDG uptake was visually graded using a four-point scale. C-reactive protein (CRP) and the erythrocyte sedimentation rate (ESR) were measured and correlated with [ 18 F]FDG-PET results by logistic regression. [ 18 F]FDG-PET revealed pathological findings in 18 of 26 patients. Three scans were categorised as grade I, 12 as grade II and 3 as grade III arteritis. Visual grade was significantly correlated with both CRP and ESR levels (p=0.002 and 0.007 respectively; grade I: CRP 4.0 mg/l, ESR 6 mm/h; grade II: CRP 37 mg/l, ESR 46 mm/h; grade III: CRP 172 mg/l, ESR 90 mm/h). Overall sensitivity was 60% (95% CI 40.6-77.3%), specificity 99.8% (95% CI 89.1-100%), positive predictive value 99.7% (95% CI 77-100%), negative predictive value 67.9% (95% CI 49.8-80.9%) and accuracy 78.6% (95% CI 65.6-88.4%). In patients presenting with a CRP 18 F]FDG-PET is highly effective in assessing the activity and the extent of large-vessel vasculitis. Visual grading was validated as representing the severity of inflammation. Its use is simple and provides high specificity, while high sensitivity is achieved by scanning in the state of active inflammation. (orig.)

The prognostic value of baseline 18F-FDG PET/CT in steroid-naive large-vessel vasculitis: introduction of volume-based parameters

International Nuclear Information System (INIS)

Dellavedova, L.; Carletto, M.; Maffioli, L.S.; Faggioli, P.; Sciascera, A.; Mazzone, A.; Del Sole, A.

2016-01-01

The aim of this study was to analyse if the result of a baseline 18 F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT scan, in large-vessel vasculitis (LVV) patients, is able to predict the course of the disease, not only in terms of presence/absence of final complications but also in terms of favourable/complicated progress (response to steroid therapy, time to steroid suspension, relapses, etc.). A total of 46 consecutive patients, who underwent 18 F-FDG PET/CT between May 2010 and March 2013 for fever of unknown origin (FUO) or suspected vasculitis (before starting corticosteroid therapy), were enrolled. The diagnosis of LVV was confirmed in 17 patients. Considering follow-up results, positive LVV patients were divided into two groups, one characterized by favourable (nine) and the other by complicated progress (eight), on the basis of presence/absence of vascular complications, presence/absence of at least another positive PET/CT during follow-up and impossibility to comply with the tapering schedule of the steroid due to biochemical/symptomatic relapse. Vessel uptake in subjects of the two groups was compared in terms of intensity and extension. To evaluate the extent of active disease, we introduced two volume-based parameters: ''volume of increased uptake'' (VIU) and ''total lesion glycolysis'' (TLG). The threshold used to calculate VIU on vessel walls was obtained by the ''vessel to liver'' ratio by means of receiver-operating characteristic analysis and was set at 0.92 x liver maximum standardized uptake value in each patient. Measures of tracer uptake intensity were significantly higher in patients with complicated progress compared to those with a favourable one (p < 0.05). Measures of disease extension were even more significant and TLG emerged as the best parameter to separate the two groups of patients (p = 0.01). This pilot study shows that, in LVV patients, the combined

Development of outcome measures for large-vessel vasculitis for use in clinical trials: opportunities, challenges, and research agenda.

Science.gov (United States)

Direskeneli, Haner; Aydin, Sibel Z; Kermani, Tanaz A; Matteson, Eric L; Boers, Maarten; Herlyn, Karen; Luqmani, Raashid A; Neogi, Tuhina; Seo, Philip; Suppiah, Ravi; Tomasson, Gunnar; Merkel, Peter A

2011-07-01

Giant cell (GCA) and Takayasu's arteritis (TAK) are 2 forms of large-vessel vasculitis (LVV) that involve the aorta and its major branches. GCA has a predilection for the cranial branches, while TAK tends to affect the extracranial branches. Both disorders may also cause nonspecific constitutional symptoms. Although some clinical features are more common in one or the other disorder and the ages of initial presentation differ substantially, there is enough clinical and histopathologic overlap between these disorders that some investigators suggest GCA and TAK may be 2 processes within the spectrum of a single disease. There have been few randomized therapeutic trials completed in GCA, and none in TAK. The lack of therapeutic trials in LVV is only partially explained by the rarity of these diseases. It is likely that the lack of well validated outcome measures for LVV and uncertainties regarding trial design contribute to the paucity of trials for these diseases. An initiative to develop a core set of outcome measures for use in clinical trials of LVV was launched by the international OMERACT Vasculitis Working Group in 2009 and subsequently endorsed by the OMERACT community at the OMERACT 10 meeting. Aims of this initiative include: (1) to review the literature and existing data related to outcome assessments in LVV; (2) to obtain the opinion of experts and patients on disease content; and (3) to formulate a research agenda to facilitate a more data-based approach to outcomes development.

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.

The prognostic value of baseline {sup 18}F-FDG PET/CT in steroid-naive large-vessel vasculitis: introduction of volume-based parameters

Energy Technology Data Exchange (ETDEWEB)

Dellavedova, L. [Ospedale Civile di Legnano, PET/CT Center - Nuclear Medicine Department, Legnano (Italy); University of Milan, Department of Health Sciences, Milan (Italy); Carletto, M.; Maffioli, L.S. [Ospedale Civile di Legnano, PET/CT Center - Nuclear Medicine Department, Legnano (Italy); Faggioli, P.; Sciascera, A.; Mazzone, A. [Ospedale Civile di Legnano, Internal Medicine Department, Legnano (Italy); Del Sole, A. [University of Milan, Department of Health Sciences, Milan (Italy)

2016-02-15

The aim of this study was to analyse if the result of a baseline {sup 18}F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT scan, in large-vessel vasculitis (LVV) patients, is able to predict the course of the disease, not only in terms of presence/absence of final complications but also in terms of favourable/complicated progress (response to steroid therapy, time to steroid suspension, relapses, etc.). A total of 46 consecutive patients, who underwent {sup 18}F-FDG PET/CT between May 2010 and March 2013 for fever of unknown origin (FUO) or suspected vasculitis (before starting corticosteroid therapy), were enrolled. The diagnosis of LVV was confirmed in 17 patients. Considering follow-up results, positive LVV patients were divided into two groups, one characterized by favourable (nine) and the other by complicated progress (eight), on the basis of presence/absence of vascular complications, presence/absence of at least another positive PET/CT during follow-up and impossibility to comply with the tapering schedule of the steroid due to biochemical/symptomatic relapse. Vessel uptake in subjects of the two groups was compared in terms of intensity and extension. To evaluate the extent of active disease, we introduced two volume-based parameters: ''volume of increased uptake'' (VIU) and ''total lesion glycolysis'' (TLG). The threshold used to calculate VIU on vessel walls was obtained by the ''vessel to liver'' ratio by means of receiver-operating characteristic analysis and was set at 0.92 x liver maximum standardized uptake value in each patient. Measures of tracer uptake intensity were significantly higher in patients with complicated progress compared to those with a favourable one (p < 0.05). Measures of disease extension were even more significant and TLG emerged as the best parameter to separate the two groups of patients (p = 0.01). This pilot study shows that, in LVV patients, the

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

High-resolution MRI vessel wall imaging: spatial and temporal patterns of reversible cerebral vasoconstriction syndrome and central nervous system vasculitis.

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Obusez, E C; Hui, F; Hajj-Ali, R A; Cerejo, R; Calabrese, L H; Hammad, T; Jones, S E

2014-08-01

High-resolution MR imaging is an emerging tool for evaluating intracranial artery disease. It has an advantage of defining vessel wall characteristics of intracranial vascular diseases. We investigated high-resolution MR imaging arterial wall characteristics of CNS vasculitis and reversible cerebral vasoconstriction syndrome to determine wall pattern changes during a follow-up period. We retrospectively reviewed 3T-high-resolution MR imaging vessel wall studies performed on 26 patients with a confirmed diagnosis of CNS vasculitis and reversible cerebral vasoconstriction syndrome during a follow-up period. Vessel wall imaging protocol included black-blood contrast-enhanced T1-weighted sequences with fat suppression and a saturation band, and time-of-flight MRA of the circle of Willis. Vessel wall characteristics including enhancement, wall thickening, and lumen narrowing were collected. Thirteen patients with CNS vasculitis and 13 patients with reversible cerebral vasoconstriction syndrome were included. In the CNS vasculitis group, 9 patients showed smooth, concentric wall enhancement and thickening; 3 patients had smooth, eccentric wall enhancement and thickening; and 1 patient was without wall enhancement and thickening. Six of 13 patients had follow-up imaging; 4 patients showed stable smooth, concentric enhancement and thickening; and 2 patients had resoluton of initial imaging findings. In the reversible cerebral vasoconstriction syndrome group, 10 patients showed diffuse, uniform wall thickening with negligible-to-mild enhancement. Nine patients had follow-up imaging, with 8 patients showing complete resolution of the initial findings. Postgadolinium 3T-high-resolution MR imaging appears to be a feasible tool in differentiating vessel wall patterns of CNS vasculitis and reversible cerebral vasoconstriction syndrome changes during a follow-up period. © 2014 by American Journal of Neuroradiology.

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)

Vasculitis associated with connective tissue diseases.

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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.

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

The value of [{sup 18}F]FDG-PET in the diagnosis of large-vessel vasculitis and the assessment of activity and extent of disease

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Walter, Martin A.; Mueller-Brand, Jan; Nitzsche, Egbert U. [University Hospital Basel, Institute of Nuclear Medicine, Basel (Switzerland); Melzer, Ralph A.; Tyndall, Alan [University Hospital Basel, Division of Rheumatology (Switzerland); Schindler, Christian [University Hospital Basel, Institute of Social and Preventive Medicine (Switzerland)

2005-06-01

This study was performed to investigate the value of{sup 18}F-fluorodeoxyglucose positron emission tomography ([{sup 18}F]FDG-PET) in the diagnosis of large-vessel vasculitis and the assessment of activity and extent of disease. Twenty-six consecutive patients (21 females, 5 males; median age - years, range 17-86 years) with giant cell arteritis or Takayasu's arteritis were examined with [{sup 18}F]FDG-PET. Follow-up scans were performed in four patients. Twenty-six age- and gender-matched controls (21 females, 5 males; median age 71 years, range 17-86 years) were included. The severity of large-vessel [{sup 18}F]FDG uptake was visually graded using a four-point scale. C-reactive protein (CRP) and the erythrocyte sedimentation rate (ESR) were measured and correlated with [{sup 18}F]FDG-PET results by logistic regression. [{sup 18}F]FDG-PET revealed pathological findings in 18 of 26 patients. Three scans were categorised as grade I, 12 as grade II and 3 as grade III arteritis. Visual grade was significantly correlated with both CRP and ESR levels (p=0.002 and 0.007 respectively; grade I: CRP 4.0 mg/l, ESR 6 mm/h; grade II: CRP 37 mg/l, ESR 46 mm/h; grade III: CRP 172 mg/l, ESR 90 mm/h). Overall sensitivity was 60% (95% CI 40.6-77.3%), specificity 99.8% (95% CI 89.1-100%), positive predictive value 99.7% (95% CI 77-100%), negative predictive value 67.9% (95% CI 49.8-80.9%) and accuracy 78.6% (95% CI 65.6-88.4%). In patients presenting with a CRP <12 mg/l or an ESR <12 mm/h, logistic regression revealed a sensitivity of less than 50%. In patients with high CRP/ESR levels, sensitivity was 95.5%/80.7%. [{sup 18}F]FDG-PET is highly effective in assessing the activity and the extent of large-vessel vasculitis. Visual grading was validated as representing the severity of inflammation. Its use is simple and provides high specificity, while high sensitivity is achieved by scanning in the state of active inflammation. (orig.)

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.

[{sup 18}F]FDG-PET in large vessel vasculitis; [{sup 18}F]FDG-PET bei Grossgefaess-Vaskulitiden

Energy Technology Data Exchange (ETDEWEB)

Hauser, A.S.D.; Walter, M.A. [Universitaetsspital Basel (Switzerland). Inst. fuer Nuklearmedizin

2007-06-15

[{sup 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 [{sup 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. [{sup 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 [{sup 18}F]FDG-PET in patients with suspected large vessel vasculitis. (orig.)

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

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

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.

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.

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.

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

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

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.

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.

New aspects of MRI for diagnostics of large vessel vasculitis and primary angiitis of the central nervous system; Neue Aspekte der MRT-Bildgebung zur Diagnostik der Grossgefaessvaskulitiden sowie der primaeren Angiitis des zentralen Nervensystems

Energy Technology Data Exchange (ETDEWEB)

Saam, T.; Habs, M.; Cyran, C.C.; Grimm, J.; Reiser, M.F.; Nikolaou, K. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Institut fuer Klinische Radiologie, Muenchen (Germany); Pfefferkorn, T. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Klinik und Poliklinik fuer Neurologie, Muenchen (Germany); Schueller, U. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Zentrum fuer Neuropathologie, Muenchen (Germany)

2010-10-15

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.) [German] Vaskulitiden sind seltene Erkrankungen, deren klinische Symptome oft unspezifisch sind und deren genaue und fruehzeitige Diagnose daher eine besondere Herausforderung fuer jeden Kliniker darstellt. Hierzu kann die Bildgebung einen wertvollen Beitrag leisten und ist insbesondere in der Lage, das Ausmass der Erkrankung und die Anzahl der betroffenen Gefaesse zu bestimmen. Die klassischen bildgebenden Verfahren wie CT- (CTA) oder MR-Angiographie (MRA) sowie die digitale Subtraktionsangiographie (DSA) fokussieren dabei hauptsaechlich auf Veraenderungen des Lumendurchmessers, die Gefaesswand wird mit diesen Verfahren in der Regel nur unzureichend dargestellt. Ultraschalluntersuchungen lassen zwar eine Beurteilung des Lumens und

Imaging of systemic vasculitis in childhood

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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.)

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.)

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.

FDG-PET/CT(A) imaging in large vessel vasculitis and polymyalgia rheumatica: joint procedural recommendation of the EANM, SNMMI, and the PET Interest Group (PIG), and endorsed by the ASNC.

Science.gov (United States)

Slart, Riemer H J A

2018-07-01

Large vessel vasculitis (LVV) is defined as a disease mainly affecting the large arteries, with two major variants, Takayasu arteritis (TA) and giant cell arteritis (GCA). GCA often coexists with polymyalgia rheumatica (PMR) in the same patient, since both belong to the same disease spectrum. FDG-PET/CT is a functional imaging technique which is an established tool in oncology, and has also demonstrated a role in the field of inflammatory diseases. Functional FDG-PET combined with anatomical CT angiography, FDG-PET/CT(A), may be of synergistic value for optimal diagnosis, monitoring of disease activity, and evaluating damage progression in LVV. There are currently no guidelines regarding PET imaging acquisition for LVV and PMR, even though standardization is of the utmost importance in order to facilitate clinical studies and for daily clinical practice. This work constitutes a joint procedural recommendation on FDG-PET/CT(A) imaging in large vessel vasculitis (LVV) and PMR from the Cardiovascular and Inflammation & Infection Committees of the European Association of Nuclear Medicine (EANM), the Cardiovascular Council of the Society of Nuclear Medicine and Molecular Imaging (SNMMI), and the PET Interest Group (PIG), and endorsed by the American Society of Nuclear Cardiology (ASNC). The aim of this joint paper is to provide recommendations and statements, based on the available evidence in the literature and consensus of experts in the field, for patient preparation, and FDG-PET/CT(A) acquisition and interpretation for the diagnosis and follow-up of patients with suspected or diagnosed LVV and/or PMR. This position paper aims to set an internationally accepted standard for FDG-PET/CT(A) imaging and reporting of LVV and PMR.

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.

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

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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.

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.

[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...

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.)

[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.

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

Treatment of renal manifestations of ANCA-associated vasculitis.

Science.gov (United States)

Galesic, Kresimir; Ljubanovic, Danica; Horvatic, Ivica

2013-01-01

Vasculitis is a clinicopathological entity characterized by inflammation and necrosis of blood vessels. Directory of Open Access Journals (DOAJ), Google Scholar, Pubmed (NLM), LISTA (EBSCO) and Web of Science have been searched. Two major autoantigens for ANCA are myeloperoxidase (MPO) and proteinase 3 (PR3), which are proteins in the primary granules of neutrophils and in the lysosomes of monocytes. They are expressed in mature neutrophils of patients with ANCA, while absent in healthy subjects. The kidney is the most commonly affected vital organ in ANCA-associated vasculitis, and patient outcomes are largely determined by the severity of renal disease at diagnosis and by its response to treatment.

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

Dense Deposit Disease Mimicking a Renal Small Vessel Vasculitis

Science.gov (United States)

Singh, Lavleen; Bhardwaj, Swati; Sinha, Aditi; Bagga, Arvind; Dinda, Amit

2016-01-01

Dense deposit disease is caused by fluid-phase dysregulation of the alternative complement pathway and frequently deviates from the classic membranoproliferative pattern of injury on light microscopy. Other patterns of injury described for dense deposit disease include mesangioproliferative, acute proliferative/exudative, and crescentic GN. Regardless of the histologic pattern, C3 glomerulopathy, which includes dense deposit disease and C3 GN, is defined by immunofluorescence intensity of C3c two or more orders of magnitude greater than any other immune reactant (on a 0–3 scale). Ultrastructural appearances distinguish dense deposit disease and C3 GN. Focal and segmental necrotizing glomerular lesions with crescents, mimicking a small vessel vasculitis such as ANCA-associated GN, are a very rare manifestation of dense deposit disease. We describe our experience with this unusual histologic presentation and distinct clinical course of dense deposit disease, discuss the pitfalls in diagnosis, examine differential diagnoses, and review the relevant literature. PMID:26361799

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

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.

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

3D-black-blood 3T-MRI for the diagnosis of thoracic large vessel vasculitis: A feasibility study

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Treitl, Karla Maria; Saam, Tobias [Institute for Clinical Radiology, LMU Munich, Munich (Germany); German Center for Cardiovascular Disease Research (DZHK e.V.), Munich (Germany); Maurus, Stefan; Sommer, Nora Narvina; Coppenrath, Eva; Treitl, Marcus [Institute for Clinical Radiology, LMU Munich, Munich (Germany); Kooijman-Kurfuerst, Hendrik [Philips Healthcare, Hamburg (Germany); Czihal, Michael; Hoffmann, Ulrich [LMU Munich, Division of Vascular Medicine, Medical Clinic and Policlinic IV, Munich (Germany); Dechant, Claudia; Schulze-Koops, Hendrik [LMU Munich, Division of Rheumatology and Clinical Immunology, Medical Clinic and Policlinic IV, Munich (Germany)

2017-05-15

To evaluate the feasibility of T1w-3D black-blood turbo spin echo (TSE) sequence with variable flip angles for the diagnosis of thoracic large vessel vasculitis (LVV). Thirty-five patients with LVV, diagnosed according to the current standard of reference, and 35 controls were imaged at 3.0T using 1.2 x 1.3 x 2.0 mm{sup 3} fat-suppressed, T1w-3D, modified Volumetric Isotropic TSE Acquisition (mVISTA) pre- and post-contrast. Applying a navigator and peripheral pulse unit triggering (PPU), the total scan time was 10-12 min. Thoracic aorta and subclavian and pulmonary arteries were evaluated for image quality (IQ), flow artefact intensity, diagnostic confidence, concentric wall thickening and contrast enhancement (CWT, CCE) using a 4-point scale. IQ was good in all examinations (3.25 ± 0.72) and good to excellent in 342 of 408 evaluated segments (83.8 %), while 84.1 % showed no or minor flow artefacts. The interobserver reproducibility for the identification of CCE and CWT was 0.969 and 0.971 (p < 0.001) with an average diagnostic confidence of 3.47 ± 0.64. CCE and CWT were strongly correlated (Cohen's k = 0.87; P < 0.001) and significantly more frequent in the LVV-group (52.8 % vs. 1.0 %; 59.8 % vs. 2.4 %; P < 0.001). Navigated fat-suppressed T1w-3D black-blood MRI with PPU-triggering allows diagnosis of thoracic LVV. (orig.)

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.

Proton density differences in signal characteristics of multiple sclerosis plaques versus white matter lesions of small vessel disease and vasculitis on high-field strength MR images

International Nuclear Information System (INIS)

Peyster, R.G.; Siegal, T.L.

1990-01-01

This paper determines if variations in signal intensity characteristics on multi-spin-echo images obtained with a high-field-strength magnet can be useful in differentiating demyelinating plaques of multiple sclerosis from other pathologic white matter processes due to small vessel disease and vasculities. Using the first of two multi-spin-echo images obtained with a General Electric 1.5-T magnet, the investigators compared signal intensity characteristics in 30 patients with a firm clinical diagnosis of multiple sclerosis versus a control group of 30 patients with a known clinical history of small-vessel disease and vasculitis are isodense to gray matter on proton-density images

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

Vasculitis : Clinical approach, pathophysiology and treatment

NARCIS (Netherlands)

Kallenberg, GGM

2000-01-01

Systemic vasculitides constitute a heterogeneous group of diseases characterized by inflammation of blood vessels. Classification of the disease is mandatory for the assessment of prognosis and the institution of treatment. Secondary forms of vasculitis should be distinguished from primary

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.

Bilateral Testicular Infarction from IgA Vasculitis of the Spermatic Cords

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Mazen Toushan

2017-01-01

Full Text Available A 51-year-old man with type 2 diabetes mellitus and chronic obstructive pulmonary disease presented to the emergency room with increasing bilateral leg pain, rash, and scrotal swelling with pain. Skin biopsy from his thigh revealed IgA-associated vasculitis. Due to hematuria, a renal biopsy was performed and showed an IgA glomerulonephritis with focal fibrinoid necrosis and neutrophil accumulation. Bilateral orchiectomies were performed in two separate procedures ten and thirteen days after the renal biopsy, as a result of uncontrolled abscess formation in testicles. Microscopically, both testicles revealed large abscess formation destroying almost the entire testicular parenchyma without tumor cells. Spermatic cord margins were further scrutinized microscopically to show bilateral vasculitis in many small size vessels, confirmed by positive endothelial staining for IgA. Some of the affected arteries revealed central organizing thrombi with recanalization features, highly suggestive of vasculitis-associated thrombi formation, resulting in testicular ischemic infarction and abscess formation. We conclude that this adult patient developed a severe form of Henoch-Schönlein purpura, with vasculitis affecting multiple organs, including the most serious and unusual complication of bilateral testicular infarction.

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

Single-Organ Gallbladder Vasculitis

Science.gov (United States)

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

2014-01-01

GB-SV was higher than in GB-SOV (35.5% vs 10%; p = 0.05). Nongranulomatous inflammation with fibrinoid necrosis of medium-sized vessels occurred equally in both groups (>90%). Forms of SV affecting the gallbladder included polyarteritis nodosa (n = 10), hepatitis B virus-associated vasculitis (n = 8), cryoglobulinemic (essential or hepatitis C virus-associated) vasculitis (n = 6), vasculitis associated with autoimmune diseases (n = 6), microscopic polyangiitis (n = 4), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (n = 4), IgA vasculitis (Henoch-Schönlein) (n = 2), and giant cell arteritis (n = 1). GV is uncommon. Its histology most often consists of a nongranulomatous necrotizing vasculitis affecting medium-sized vessels. GB-SOV is usually discovered after routine cholecystectomy performed because of the presence of local symptoms, gallstone-associated cholecystitis, and contrary to GB-SV, GB-SOV is usually not associated with systemic symptoms. Acute phase reactants and surrogate markers of autoimmunity are usually normal or negative in GB-SOV. GB-SOV does not require systemic antiinflammatory or immunosuppressive therapy; surgery is adequate to achieve cure. GB-SV always warrants immunosuppressant therapy and is associated with high mortality. The finding of GV may precede the generalized manifestations of SV. Therefore, once GV is discovered, studies to determine disease extent and a vigilant follow-up are mandatory. PMID:25500710

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.

Livedo vasculitis Livedo vasculitis

Directory of Open Access Journals (Sweden)

RE Achenbach

Full Text Available La livedo vasculitis representa un patrón de respuesta a múltiples causas, que confluyen en un estado procoagulante de los vasos de la dermis y subcutis superficial, resultando en úlceras dolorosas con localización en miembros inferiores y cicatrices características. Efectuamos una revisión de las múltiples sinonimias, aspectos clínicos e histológicos de tres casos, los que respondieron aceptablemente al tratamiento con pentoxifilina vía bucal.Livedo is not a specific disease, but a distinctive clinicopathologic pattern of changes, that may occur in a variety of different circunstances. The main pathogenic mechanism is considered to be intraluminal thrombosis of dermal vessels, with a common pathway and a prothrombotic state. A glossary, clinical, histological and therapeutic aspects are reviewed.

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.

Cutaneous polyarteritis nodosa: A rare isolated cutaneous vasculitis

Directory of Open Access Journals (Sweden)

Praveen Kumar A Subbanna

2012-01-01

Full Text Available Cutaneous polyarteritis nodosa (CPAN is a rare form of cutaneous vasculitis that involves small and medium sized arteries of the dermis and subcutaneous tissue without systemic involvement. It presents with tender subcutaneous nodules, digital gangrene, livedo reticularis and subcutaneous ulcerations. The diagnosis is by skin biopsy and characteristic pathologic feature is a leukocytoclastic vasculitis in the small to medium-sized arterioles of the dermis. We report a rare case of benign cutaneous PAN in a 14-year-old girl who presented with history of fever, subcutaneous nodules with cutaneous ulcer and digital gangrene. The skin biopsy showed leukocytoclastic vasculitis with fibrinoid necrosis in the dermal vessels. She received treatment with steroids and lesions resolved completely over a period of month.

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.

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

A project by the SIDeMaST Immunopathology Group on cutaneous vasculitis.

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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

Neutrophil extracellular traps in vasculitis, friend or foe?

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Söderberg, Daniel; Segelmark, Mårten

2018-01-01

Neutrophil extracellular traps (NETs) can be found at the sites of vascular lesions and in the circulation of patients with active small vessel vasculitis. Neutrophils from vasculitis patients release more NETs in vitro, and NETs have properties that can harm the vasculature both directly and indirectly. There are several ways to interfere with NET formation, which open for new therapeutic options. However, there are several types of NETs and different mechanisms of NET formation, and these might have different effects on inflammation. Here we review recent findings regarding the pathogenesis and therapeutic potentials of NETs in vasculitis. Experimental mouse models support a role for NETs in promoting vascular damage, where histones and mitochondrial DNA appear to be driving forces. Impaired formation of NETs, however, in an SLE-like mouse model leads to more severe disease, suggesting that NETs can be important in limiting inflammation. Studies on drug-induced vasculitis reveal that levamisole can induce NETosis via muscarinic receptors, predisposing for the generation of autoantibodies, including antineutrophil cytoplasmic autoantibodies (ANCA). This supports the notion that NETs can bridge the innate and adaptive immune systems. NETs can participate in the pathogenesis of vasculitis, but in some models there also seem to be protective effects of NETs. This complexity needs further evaluation with experimental models that are as specific as possible for human primary vasculitis.

Churg-Strauss syndrome with coexistence of eosinophilic vasculitis, granulomatous phlebitis and granulomatous dermatitis in bullous pemphigoid-like blisters.

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Ishibashi, Masafumi; Kudo, Saori; Yamamoto, Kyoko; Shimai, Nobuko; Chen, Ko-Ron

2011-03-01

The main histopathological features in the cutaneous lesions of Churg-Strauss syndrome (CSS) are dermal leukocytoclastic vasculitis with a variable eosinophilic infiltrate and non-vasculitic tissue eosinophilia with granuloma formation. This wide histopathological spectrum may account for the various skin manifestations of CSS. However, the unique histopathological combination of dermal eosinophilic vasculitis and subcutaneous granulomatous phlebitis accompanied by bulla formation has not been previously described. We report an unusual CSS case showing dermal necrotizing eosinophilic vasculitis and granulomatous phlebitis in purpuric lesions coupled with subepidermal blistering. The blisters showed dermal granulomatous dermatitis and eosinophilia without evidence of vasculitis. Dermal necrotizing eosinophilic vasculitis was characterized by fibrinoid alteration of the vessel wall, a prominent perivascular eosinophilic infiltrate, a few infiltrating histiocytes along the affected vessel wall, and the absence of neutrophilic infiltration. The underlying subcutaneous granulomatous phlebitis was characterized by an angiocentric histiocytic infiltrate surrounded by marked eosinophilic infiltrate. Deposition of cytotoxic proteins and radicals derived from eosinophils in the vessel walls and papillary dermis followed by a secondary granulomatous response may account for the unique clinical and histopathological features in this case. Copyright © 2010 John Wiley & Sons A/S.

Vasculitis Pregnancy Registry

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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

A pragmatic approach to vasculitis in the gastrointestinal tract.

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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/.

Hypertrophic cardiomyopathy secondary to hepatitis C virus-related vasculitis.

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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.

Cryoglobulinemia Vasculitis.

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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.

PF-1355, a mechanism-based myeloperoxidase inhibitor, prevents immune complex vasculitis and anti-glomerular basement membrane glomerulonephritis.

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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.

Cerebral vasculitis associated with Schistosoma mansoni infection

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Camuset Guillaume

2012-09-01

Full Text Available Abstract Background Cerebral involvement in schistosomiasis is not rare, but it is underdiagnosed because of the lack of clinical suspicion and the frequency of asymptomatic forms. Neurologic complications are generally supported by granuloma formation around ectopic eggs which have migrated to the brain. Moreover, vascular lesions and cerebral arteritis have been well documented in histopathological studies. Nevertheless, cerebral vasculitis in later stages of the Schistosoma mansoni infection have not yet been described in living subjects. Case presentation A 28-year-old french woman had a stroke linked with cerebral vasculitis, 6 monthes after returning from Burkina-Faso. At the same time, a S. mansoni disseminated infection was diagnosed. She suffered from a new stroke after undertaking praziquantel therapy, which lead us to associate the S. mansoni infection and cerebral vasculitis. Conclusion This is the first report of such association, since cerebral vasculitis has never been described in later stages of the S. mansoni infection. Although the causal link between the two pathologies could not be proved, we suggest that S. mansoni is able to cause severe vascular damage in cerebral vessels. Schistosomiasis must be investigated in the event of a brain infarct in young people, particularly in patients originating or returning from an endemic area.

Retinal Vasculitis

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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

Pediatric vasculitis.

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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.

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

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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.

Fulminant Vasculitis Associated with Extracranial Dissections and Occlusion, Ischemic Strokes, and Aneurysm Rupture: Case Report and Review of the Literature.

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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.

Vasculitis assessment with [{sup 18}F]F.D.G. positron emission tomography; Place de la tomographie par emission de positons (TEP) au [{sup 18}F]FDG dans l'exploration des vascularites

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Liozon, E. [CHU Dupuytren, Services de Medecine Interne A, 87 - Limoges (France); Monteil, J. [CHU Dupuytren, Services de Medecine Nucleaire, 87 - Limoges (France)

2008-10-15

[{sup 18}F]fluorodeoxyglucose ({sup 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 [{sup 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.

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

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

2014-11-01

higher than in GB-SOV (35.5% vs 10%; p = 0.05). Nongranulomatous inflammation with fibrinoid necrosis of medium-sized vessels occurred equally in both groups (>90%). Forms of SV affecting the gallbladder included polyarteritis nodosa (n = 10), hepatitis B virus-associated vasculitis (n = 8), cryoglobulinemic (essential or hepatitis C virus-associated) vasculitis (n = 6), vasculitis associated with autoimmune diseases (n = 6), microscopic polyangiitis (n = 4), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (n = 4), IgA vasculitis (Henoch-Schönlein) (n = 2), and giant cell arteritis (n = 1).GV is uncommon. Its histology most often consists of a nongranulomatous necrotizing vasculitis affecting medium-sized vessels. GB-SOV is usually discovered after routine cholecystectomy performed because of the presence of local symptoms, gallstone-associated cholecystitis, and contrary to GB-SV, GB-SOV is usually not associated with systemic symptoms. Acute phase reactants and surrogate markers of autoimmunity are usually normal or negative in GB-SOV. GB-SOV does not require systemic antiinflammatory or immunosuppressive therapy; surgery is adequate to achieve cure. GB-SV always warrants immunosuppressant therapy and is associated with high mortality. The finding of GV may precede the generalized manifestations of SV. Therefore, once GV is discovered, studies to determine disease extent and a vigilant follow-up are mandatory.

Eosinophils in vasculitis: characteristics and roles in pathogenesis

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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

Pentraxin-3 levels are associated with vasculitis and disease activity in childhood-onset systemic lupus erythematosus.

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Sahin, S; Adrovic, A; Barut, K; Durmus, S; Gelisgen, R; Uzun, H; Kasapcopur, O

2017-09-01

Objectives Childhood-onset systemic lupus erythematosus (cSLE) is a multisystemic autoimmune disease characterized by inflammatory organ damage by means of vasculitis. Pentraxin-3 (PTX3) is expressed locally at the sites of inflammatory processes, predominantly from endothelial cells. In adult studies, PTX3 has shown to be an indicator of active vasculitis both in large-vessel and small-vessel vasculitides, as well as in SLE. Moreover, in SLE it has found to be correlated with disease activity, and with some of the clinical manifestations and laboratory parameters. We aimed to ascertain if PTX3 might be a significant mediator in cSLE and if it might indicate active vasculitis during the course of the disease. Methods Serum PTX3 levels were measured in 76 patients with cSLE and 41 healthy subjects. We have investigated its relation with disease activity, damage, clinical features, laboratory parameters and medications. Results Serum levels of PTX3 were found to be increased in cSLE compared to healthy controls (mean ± SD; 10.6 ± 8.2 ng/mL vs 2.7 ± 1.3 ng/mL, p Lupus International Collaborating Clinics/American College of Rheumatology Damage Index), ESR, CRP, procalcitonin levels, anti-ds DNA antibody, anticardiolipin antibodies was not detected. Conclusions Patients with cSLE have increased levels of serum PTX3 compared to healthy controls. Thus, serum PTX-3 level might be a significant mediator in cSLE. Apart from these, the results support that PTX3 reflects active cutaneous vasculitis in cSLE and correlates with disease activity.

Vasculitis and inflammatory arthritis.

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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.

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

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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.

Biomarkers in Vasculitis

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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

A STUDY OF AETIOLOGICAL FACTORS IN THE OCCURRENCE OF CUTANEOUS VASCULITIS IN A TERTIARY CARE CENTRE IN NORTH KERALA

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Praveen Malayath

2017-03-01

Full Text Available BACKGROUND Cutaneous vasculitis is a condition caused by various aetiologies. They can be primary or secondary. Cutaneous lesions maybe a pointer to systemic diseases. So, it is important to identify the various aetiological factors in the occurrence of the various types of cutaneous vasculitis. The patterns and the various aetiologies of cutaneous vasculitis in Kerala is not well documented in the existing literature. MATERIALS AND METHODS The present study is a cross-sectional descriptive study of patients with a clinical diagnosis of cutaneous small vessel vasculitis admitted in Medicine and Dermatology Wards of Calicut, Government Medical College during January 2013 to December 2013. A detailed history and clinical examination of patients along with histopathological examination of skin biopsy was done. RESULTS Out of the 70 cases of cutaneous vasculitis studied, idiopathic cutaneous small vessel vasculitis was the most common type followed by Henoch-Schonlein purpura. The most common aetiology identified was drugs followed by infections. No aetiological factor was identified in 42.8% of the cases. CONCLUSION An aetiological association could be found in 57.8% of cases. The causes identified include drugs, infections, malignancy, connective tissue disorder associated, chronic systemic diseases and Behcet’s disease in decreasing order of frequency.

Ongoing Cerebral Vasculitis During Treatment of Rocky Mountain Spotted Fever.

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Sun, Lisa R; Huisman, Thierry A G M; Yeshokumar, Anusha K; Johnston, Michael V

2015-11-01

Rocky Mountain spotted fever is a tickborne infection that produces a systemic small-vessel vasculitis; its prognosis is excellent if appropriate treatment is initiated early. Because the advent of effective antirickettsial therapies predates the widespread use of brain magnetic resonance imaging, there are limited data on the effect of untreated Rocky Mountain spotted fever infection on neuroimaging studies. We describe a 7-year-old girl with delayed treatment of Rocky Mountain spotted fever who suffered severe neurological impairment. Serial brain magnetic resonance images revealed a progressive "starry sky appearance," which is proposed to result from the same small vessel vasculitis that causes the characteristic skin rash of this infection. Neurological injury can continue to occur despite specific antirickettsial therapy in Rocky Mountain spotted fever. This child's clinical features raise questions about the optimal management of this infection, particularly the utility of immune modulating therapies in cases of delayed treatment and neurological involvement. Copyright © 2015 Elsevier Inc. All rights reserved.

Urinary Soluble CD163 in Active Renal Vasculitis.

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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.

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

Clinical Transcriptomics in Systemic Vasculitis (CUTIS)

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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

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

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

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

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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.

Vasculitis and Pregnancy.

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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.

Pediatric Vasculitis Initiative

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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

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

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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.

Cocaine-Levamisole-Induced Vasculitis/Vasculopathy Syndrome.

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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.

Managing mesenteric vasculitis.

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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.

Recent advances in childhood vasculitis.

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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.

Systemic vasculitis and the gut.

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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.

Thromboembolic disease in vasculitis

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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

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

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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.

Patient perceptions about illness self-management in ANCA-associated small vessel vasculitis.

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Thorpe, C T; DeVellis, R F; Blalock, S J; Hogan, S L; Lewis, M A; DeVellis, B M

2008-06-01

To characterize patient perceptions, related to eight self-management behaviours relevant for adults with ANCA-associated small vessel vasculitis (ANCA-SVV), and to determine if these perceptions were associated with performance of each behaviour. Adults with ANCA-SVV (n = 202) completed a self-administered questionnaire that assessed eight self-management behaviours (adherence to recommendations for medication, health service use, diet, exercise, infection avoidance and symptom monitoring; prompt reporting of symptoms and side effects; and adjusting activities in response to symptoms), perceptions about these behaviours, socio-demographics, clinical factors and social desirability bias. Descriptive statistics were generated to characterize patients' perceptions about difficulty of, importance of, and specific barriers to performing each behaviour. Regression analyses explored whether these variables were associated with performing each behaviour, controlling for potential confounders. With few exceptions, higher perceived importance and lower perceived difficulty of each behaviour were associated with more frequent performance of the behaviour. For each behaviour, several specific barriers were frequently endorsed by patients and a number of these were associated with lower levels of self-management. This study reveals that patient perceptions about the illness and its treatment influence ANCA-SVV self-management. Perceived barriers to medication, health services, diet and exercise adherence were similar to those in other illnesses. This study also provides insight into barriers experienced by patients in performing behaviours (infection avoidance, symptom monitoring, reporting symptoms and side-effects and adjusting activities) not often previously studied. How the identification of these barriers can help inform future interventions for ANCA-SVV patients is to be discussed.

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

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

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.)

MRI in human immunodeficiency virus-associated cerebral vasculitis

Energy Technology Data Exchange (ETDEWEB)

Berkefeld, J.; Lanfermann, H. [Frankfurt Univ. (Germany). Abt. fuer Neuroradiologie; Enzensberger, W. [Klinik fuer Neurologie, Klinikum der Johann Wolfgang Goethe-Univ. Frankfurt am Main (Germany)

2000-07-01

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.)

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.

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.

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

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...

The Spectrum of Paraneoplastic Cutaneous Vasculitis in a Defined Population

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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

Pregnancy Outcomes Among Patients With Vasculitis

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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

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.

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.

Acute respiratory failure as primary manifestation of antineutrophil cytoplasmic antibodies-associated vasculitis

Directory of Open Access Journals (Sweden)

Evdokia Sourla

2014-07-01

Full Text Available The systemic vasculitides are multifocal diseases characterized by the presence of blood vessel inflammation in multiple organ systems. Their clinical presentation is variable extending from self-limited illness to critical complications including diffuse alveolar hemorrhage and glomerulonephritis. Alveolar hemorrhage is a lifethreatening manifestation of pulmonary vasculitis that can rapidly progress into acute respiratory failure requiring ventilatory support. We present the case of a 74-year-old patient admitted to the Intensive Care Unit with severe hypoxic respiratory failure and diffuse alveolar infiltrates in chest imaging that was later diagnosed as antineutrophil cytoplasmic antibodies-associated vasculitis. The report highlights the importance of differentiate between alveolar hemorrhage and acute respiratory distress syndrome of other etiology because alveolar hemorrhage is reversible with prompt initiation of treatment.

Histopathology of the Inner Ear in a Case With Recent Onset of Cogan's Syndrome: Evidence for Vasculitis.

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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.

Identification of Fetal Inflammatory Cells in Eosinophilic/T-cell Chorionic Vasculitis Using Fluorescent In Situ Hybridization.

Science.gov (United States)

Katzman, Philip J; Li, LiQiong; Wang, Nancy

2015-01-01

Eosinophilic/T-cell chorionic vasculitis (ETCV) is an inflammatory lesion of placental fetal vessels. In contrast to acute chorionic vasculitis, inflammation in ETCV is seen in chorionic vessel walls opposite the amnionic surface. It is not known whether inflammation in ETCV consists of maternal cells from the intervillous space or fetal cells migrating from the vessel. We used fluorescent in situ hybridization (FISH) to differentiate fetal versus maternal cells in ETCV. Placentas with ETCV, previously identified for a published study, were used. Infant sex in each case was identified using the electronic medical record. For male infants, 3-μm sections were cut from archived tissue blocks from placentas involving ETCV and stained with fluorescent X- and Y-chromosome centromeric probes. A consecutive hematoxylin/eosin-stained section was used for correlation. FISH analysis was performed on 400 interphase nuclei at the site of ETCV to determine the proportion of XX, XY, X, and Y cells. Of 31 ETCV cases, 20 were female and 10 were male (1 sex not recorded). Six of 10 cases with male infants had recuts with visible ETCV. In these 6 cases the average percentages (ranges) of XY cells, X-only cells, and Y-only cells in the region of inflammation were 81 (70-90), 11 (6-17), and 8 (2-14), respectively. There was a 2:1 female:male infant ratio in ETCV. Similar to acute chorionic vasculitis, the inflammation in ETCV is of fetal origin. It is still unknown, however, whether the stimulus for ETCV is of fetal or maternal origin.

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....

IgA vasculitis as a presentation of human immunodeficiency virus infection.

Science.gov (United States)

Brandy-García, Anahy M; Santos-Juanes, Jorge; Suarez, Silvia; Caminal-Montero, Luis

2018-05-15

IgA vasculitis is a small-vessel vasculitis mediated by immune complexes. In clinical terms, it is characterized by palpable purpura in the lower limbs, joint involvement in the form of arthralgia or arthritis, and gastrointestinal and renal involvement (this will mark a poorer prognosis in adults). Infectious processes, mainly in the upper respiratory tract, are frequently found to be triggers. On the other hand, human immunodeficiency virus (HIV) causes immune dysfunction, which triggers hypergammaglobulinemia and can trigger autoimmune disorders. At times, this can affect the vascular endothelium, giving rise to vasculitic manifestations, although there are few reports in the literature of its role in the presentation of HIV. Copyright © 2018 Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. Publicado por Elsevier España, S.L.U. All rights reserved.

Leukocytoclastic vasculitis associated with clarithromycin.

Science.gov (United States)

Gavura, S R; Nusinowitz, S

1998-05-01

To report a possible case of leukocytoclastic vasculitis associated with clarithromycin therapy. An 83-year-old white woman was prescribed clarithromycin for pneumonia. Six days after her initial presentation, she developed lesions on her palms. Clarithromycin was discontinued at that time. The following day she developed purpuric eccymotic nonblanching lesions that primarily appeared on the lower extremities, buttocks, and abdomen. Colonoscopy revealed generalized erythema and edema of the bowel mucosa. Gastroscopy revealed duodenitis and gastritis, but no bleeding or ulceration. Skin biopsy of the lesions was compatible with leukocytoclastic vasculitis. Renal function was not affected, although hematuria was noted. All symptoms resolved after drug withdrawal and a short course of corticosteroids. Searches were performed on MEDLINE, Embase, International Pharmaceutical Abstracts, and major adverse drug reaction databases to identify reports and articles discussing clarithromycin- and macrolide-induced leukocytoclastic vasculitis. Leukocytoclastic vasculitis is one category of drug hypersensitivity reactions characterized by distinctive patterns of perivascular inflammation. The case described here is consistent with the diagnosis of leukocytoclastic vasculitis, and is similar to the other single published case report associated with clarithromycin. Leukocytoclastic vasculitis induced by clarithromycin is a rare but serious potential adverse effect.

Clinically Apparent Arterial Thrombosis in Persons with Systemic Vasculitis

Directory of Open Access Journals (Sweden)

Alexander Tsoukas

2017-01-01

Full Text Available Objective. To estimate the incidence rate of clinically apparent arterial thrombotic events and associated comorbidities in patients with primary systemic vasculitis. Methods. Using large cohort administrative data from Quebec, Canada, we identified patients with vasculitis, including polyarteritis nodosa (PAN and granulomatosis with polyangiitis (GPA. Incident acute myocardial infarctions (AMIs and cerebrovascular accidents (CVAs after the diagnosis of vasculitis were ascertained in the PAN and GPA group via billing and hospitalization data. These were compared to rates of a general population comparator group. The incidences of comorbidities (type 2 diabetes mellitus, dyslipidemia, and hypertension were also collected. Results. Among the 626 patients identified with vasculitis, 19.7% had PAN, 2.9% had Kawasaki disease, 23.8% had GPA, 52.4% had GCA, and 1.3% had Takayasu arteritis. The AMI rate was substantially higher in males aged 18–44 with PAN, with rates up to 268.1 events per 10,000 patient years [95% CI 67.1–1070.2], approximately 30 times that in the age- and sex-matched control group. The CVA rate was also substantially higher, particularly in adults aged 45–65. Patients with vasculitis had elevated incidences of diabetes, dyslipidemia, and hypertension versus the general population. Conclusion. Atherothrombotic rates were elevated in patients identified as having primary systemic vasculitis. While incident rates of cardiovascular comorbidities were also increased, the substantial elevation in AMIs seen in young adults suggests a disease-specific component which requires further investigation.

Omalizumab for Urticarial Vasculitis

DEFF Research Database (Denmark)

Ghazanfar, Misbah Nasheela; Thomsen, Simon Francis

2015-01-01

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...

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.

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

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

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

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.

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

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.

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.

Systemic Vasculitis During the Course of Systemic Sclerosis

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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

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

Posterior reversible encephalopathy syndrome in IgA vasculitis: Neuroimaging of a 14-year-old child.

Science.gov (United States)

Arslan, Harun; Yavuz, Alpaslan; Arslan, Ayşe; Aycan, Abdurrahman

IgA vasculitis (IgAV) is a leukocytoclastic vasculitis and characterized by involvement of small vessels in skin, gastrointestinal system, joints, kidneys, and less frequently other organs. It is the commonest vasculitis in childhood and etiology is not completely known. Neurological manifestations of IgAV are very rare and usually seen in patients with severe hypertension or as an uncommon feature such as peripheral neuropathy. Posterior reversible encephalopathy syndrome (PRES) is a clinic-radiologic entity characterized with temporary vasogenic edema developing typically in posterior circulation of the brain and has been reported as a rare manifestation of IgAV. In this paper, a PRES case of 14-year-old male with IgAV is reported and etiopathogenesis was discussed with literature. Diagnosis was made by magnetic resonance imaging because of the existence of neurological symptoms (headache and visual loss) during the course of disease. His radiological findings have resolved with therapy. Although neurological involvement is a rare manifestation in IgAV, we recommend magnetic resonance imaging in such patients for diagnosis and evaluation of complications. Copyright © 2017. Published by Elsevier Urban & Partner Sp. z o.o.

Vasculitis and Thrombosis due to the Sea Lion Lungworm, Parafilaroides decorus, in a Guadalupe Fur Seal ( Arctocephalus philippii townsendi).

Science.gov (United States)

Seguel, Mauricio; Nadler, Steven; Field, Cara; Duignan, Padraig

2018-05-01

A free-ranging, male, yearling Guadalupe fur seal ( Arctocephalus philippii townsendi) died due to multifocal verminous vasculitis with thrombosis and several embolic infarcts in liver, kidney, and brain. Nematodes extracted from lung blood vessels were identified as Parafilaroides decorus, a parasite normally found in alveoli of California sea lions ( Zalophus californianus).

Urticarial Vasculitis

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Hypersensitivity Vasculitis

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Vitamin D Status in Small Vessel and Large Vessel Ischemic Stroke Patients: A Case–control Study

Directory of Open Access Journals (Sweden)

Navid Manouchehri

2017-01-01

Full Text Available Background: Vitamin D insufficiency is a globally widespread issue. Recent studies have reported a high prevalence of Vitamin D deficiency in Middle-East countries. Studies have shown negative effects of Vitamin D deficiency on endothelium and related diseases such as ischemic brain stroke. Here, we assessed Vitamin D status in patients with different types of ischemic brain stroke and control group. Materials and Methods: Seventy-five patients (49.3% small vessel, 50.7% large vessel and 75 controls, matched for age (68.01 ± 10.94 vs. 67.64 ± 10.24 and sex (42 male and 33 female were recruited. 25(OH D levels were measured by Chemiluminescence immunoassay. 25(OH D status was considered as severely, moderately, or mildly deficient and normal with 25(OH D levels of less than 5, 5-10, 10-16, and> 16 ng/ml, respectively. Results: Mean ± standard error concentration of 25(OH D in cases and controls were 17.7 ± 1.5 and 26.9 ± 1.6 (P = 0.0001, respectively. Mild, moderate, and severe Vitamin D deficiency were observed in 10.8%, 32.4%, 8.1% vs. 34.3%, 31.5%, 9.5% of small vessel and large vessel group, respectively. 21.7% of the controls were Vitamin D deficient. Vitamin D deficiency was significantly associated with higher risk for ischemic stroke, (P = 0.000, OR = 7.17, 95% confidence interval: 3.36–15.29. 25(OH D levels were significantly higher in control group comparing to small vessel (26.9 ± 1.6 vs. 20.59 ± 2.6 P < 0.05 and large vessel (26.9 ± 1.6 vs. 13.4 ± 1.3 P < 0.001 stroke patients. Small vessel group had significantly higher levels of Vitamin D than large vessel (P < 0.05. Conclusion: Vitamin D deficiency significantly increases the risk of ischemic stroke, favoring the types with the pathogenesis of large vessel strokes.

Vasculitis Foundation

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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.

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

Acral keratoses and leucocytoclastic vasculitis occurring during treatment of essential thrombocythaemia with hydroxyurea.

Science.gov (United States)

Worley, B; Glassman, S J

2016-03-01

Hydroxyurea is used in essential thrombocythaemia to lower thromboembolic risk. Cutaneous adverse effects from hydroxyurea are diverse. Small vessel vasculitis has been rarely reported, and the coexistence of several different morphologies has not been described. We report a case of acral keratoses, psoriasiform plaques and leucocytoclastic vasculitis (LCV) in a patient with essential thrombocythaemia. A 69-year-old woman developed a confusing array of skin lesions including keratotic papules, psoriasiform plaques and keratoderma 4 years after commencing hydroxyurea therapy. The initial diagnosis was hand and foot psoriasis, but lesions were resistant to therapy. With an increase in the dose of hydroxyurea, the lesions ulcerated. Skin biopsies taken from different sites indicated different diagnoses, including LCV. Discontinuation of hydroxyurea yielded rapid improvement. Although the most commonly reported cutaneous adverse effect from hydroxyurea is leg ulceration, this can be preceded or accompanied by less dramatic skin lesions. Unless recognized, delayed diagnosis and lesion progression can occur. © 2015 British Association of Dermatologists.

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.

Swarm Manipulation of Large Surface Vessels

National Research Council Canada - National Science Library

Smith, Erik T

2007-01-01

The goal of this Trident project was to develop an independent control scheme to allow a team of autonomous tugboats to move a large disabled vessel, such as a barge, to a desired position and orientation...

Recent advances in anti-neutrophil cytoplasmic antibody-associated vasculitis

Directory of Open Access Journals (Sweden)

B Lazarus

2016-01-01

Full Text Available Anti-neutrophil cytoplasmic antibody-associated vasculitis is an uncommon inflammatory disease of small to medium-sized vessels that frequently presents with rapidly progressive glomerulonephritis and renal failure though it can affect any organ system. If untreated, the vast majority of patients will die within a year. Current treatments improve prognosis but affected patients remain at a substantially higher risk of death and adverse outcomes. We review the classification of the disease, our understanding of the pathogenesis and epidemiology, and propose future directions for research. We also evaluate the evidence supporting established treatment regimens and the progress of clinical trials for newer treatments to inform the design of future studies.

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.

Lysosomal-associated membrane protein-2 plays an important role in the pathogenesis of primary cutaneous vasculitis.

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Takeuchi, Sora; Kimura, Satoko; Soma, Yoshinao; Waki, Masashi; Yamaguchi, Madoka; Nakazawa, Daigo; Tomaru, Utano; Ishizu, Akihiro; Kawakami, Tamihiro

2013-09-01

Recent research suggests that lysosomal-associated membrane protein-2 (LAMP-2) could be one of the target antigens in the pathogenesis of vasculitides. We established a transgenic rat model, env-pX rats, with various vasculitides including cutaneous vasculitis. Human primary cutaneous vasculitis includes cutaneous polyarteritis nodosa (CPN) and Henoch-Schönlein purpura (HSP). We measured serum anti-LAMP-2 antibody levels in morbid env-pX rats and injected anti-LAMP-2 antibody into premorbid env-pX rats. We further measured serum anti-LAMP-2 antibody levels in patients with CPN and HSP. Cutaneous vasculitis was observed in ∼30% of 6-month-old morbid env-pX rats. In contrast, these findings were rare in premorbid env-pX rats under 3 months old. We also examined 85 patients with CPN and 36 adult patients with HSP. Serum anti-LAMP-2 antibody levels were determined using ELISA. Premorbid env-pX rats under 3 months old were given an i.v. injection of anti-LAMP-2 antibody at day 0 and day 7. At day 14, these rats underwent histopathological and direct immunofluorescence examination. Cell surface LAMP-2 expression of rat neutrophils was examined by flow cytometry. Serum anti-LAMP-2 antibody levels were significantly higher in morbid env-pX rats than in wild-type normal rats. In addition, the levels in the cutaneous vasculitis group of morbid env-pX rats were significantly higher than the no cutaneous vasculitis group. Intravenous anti-LAMP-2 antibody injection into premorbid env-pX rats under 3 months old induced infiltration of neutrophils into cutaneous small vessels. Anti-LAMP-2 antibody-binding neutrophils were detected there. LAMP-2 expression on the cell surface of neutrophils in premorbid env-pX rats under PMA stimulation was higher compared with controls. Serum anti-LAMP-2 antibody levels in CPN and HSP were significantly higher than those of healthy controls. These data support a positive relationship between anti-LAMP-2 antibody and cutaneous vasculitis.

Genetically Distinct Subsets within ANCA-Associated Vasculitis

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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

Cryofibrinogenemia: a marker of severity of cryoglobulinemic vasculitis.

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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.

Cryoglobulinemic vasculitis in a patient with CREST syndrome.

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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.

Myocytes of chorionic vessels from placentas with meconium-associated vascular necrosis exhibit apoptotic markers.

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King, Erin L; Redline, Raymond W; Smith, Steven D; Kraus, Frederick T; Sadovsky, Yoel; Nelson, D Michael

2004-04-01

Meconium-associated vascular necrosis (MAVN) is a histological abnormality of human placental chorionic vessels that is associated with poor neonatal outcome. We tested the hypothesis that MAVN shows apoptosis in the walls of chorionic vessels. Archival placental specimens with MAVN (n = 5) were compared with specimens from uncomplicated pregnancies at term (n = 5) and from placentas with intense chorionic vasculitis associated with acute chorioamnionitis with (n = 5) or without (n = 5) a clinical history of meconium in the amniotic fluid. Sections from all placentas were processed by the TUNEL method, and 2 observers who were blinded to specimen diagnosis quantified the immunofluorescent TUNEL staining in both the amnion-facing and villous-facing walls of the larger chorionic vessels in each specimen. Compared with the other 3 groups, only the amnion-facing wall of chorionic vessels in MAVN showed a significantly greater number of apoptotic cells. This was verified by morphological criteria and caspase 3 staining. There were limited or no detectable TUNEL-stained cells in either the villous-facing walls of vessels in the MAVN specimens or in any of the vessels of the placentas from uncomplicated pregnancies. There was a negligible level of apoptosis in chorionic vessels of placentas with intense chorionic vasculitis, with or without meconium, despite the inflammatory response or presence of meconium. We conclude that apoptosis contributes to the pathophysiology of MAVN.

Churg-Strauss syndrome and hemorragic vasculitis

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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.

Churg-Strauss syndrome and hemorrhagic vasculitis

Science.gov (United States)

Marques, Rui Moreira; Cabral, Ana Rita; Monteiro, Antonio; Henriques, Pedro

2011-01-01

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 left-hand 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. PMID:25386301

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

Large vessel imaging using cosmic-ray muons

International Nuclear Information System (INIS)

Jenneson, P.M.

2004-01-01

Cosmic-ray muons are assessed for their practical use in the tomographic imaging of the internal composition of large vessels over 2 m in diameter. The technique is based on the attenuation and scattering of cosmic-ray muons passing through a vessel and has advantages over photon-based methods of tomography that it is extendable to object containing high-density materials over many tens of metres. The main disadvantage is the length of time required to produce images of sufficient resolution and hence cosmic ray muon tomography will be most suited to the imaging of large structures whose internal composition is effectively static for the duration of the imaging period. Simulation and theoretical results are presented here which demonstrate the feasibility of cosmic ray muon tomography

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.

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

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.

Illness Perceptions and Fatigue in Systemic Vasculitis

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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

Clinical studies of the Research Committee on Intractable Vasculitides, the Ministry of Health, Labour and Welfare of Japan.

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Makino, Hirofumi; Sada, Ken-Ei

2013-10-01

In Japan, the Research Committee on Intractable Vasculitides, supported by the Ministry of Health, Labour and Welfare, has been promoting basic and clinical research on vasculitis since 1972. The present Research Committee on Intractable Vasculitides comprises 4 subcommittees under the direction of a Principal Investigator: Basic and Pathological Research Subcommittee, Clinical Research Subcommittee of Small and Medium-sized Vessel Vasculitis, Clinical Research Subcommittee of Large-sized Vessel Vasculitis, and International Cooperation Research Subcommittee. Since 2008, 9 nationwide clinical studies for vasculitis have been conducted and 8 clinical and basic studies are in progress.

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

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.

Perspective on future therapy of vasculitis.

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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.

Application of digital subtraction angiography in disease of large cardiac vessel

Energy Technology Data Exchange (ETDEWEB)

Arisawa, Jun; Sone, Shusuke; Morimoto, Shizuo; Ikezoe, Junpei; Higashibara, Tokuro; Hanayama, Masayuki

1983-06-01

Digital subtraction angiography (DSA) was performed in 31 cases of disease of large cardiac vessel. DSA was useful for the diagnosis of aortic aneurysm and malformation of large vessels, follow-up after A-C bypass operation and Blalock's shunt operation for tetralogy of Fallot and as an adjuvant modality in cardiac catheterization.

Application of digital subtraction angiography in disease of large cardiac vessel

International Nuclear Information System (INIS)

Arisawa, Jun; Sone, Shusuke; Morimoto, Shizuo; Ikezoe, Junpei; Higashibara, Tokuro; Hanayama, Masayuki

1983-01-01

Digital subtraction angiography (DSA) was performed in 31 cases of disease of large cardiac vessel. DSA was useful for the diagnosis of aortic aneurysm and malformation of large vessels, follow-up after A-C bypass operation and Blalock's shunt operation for tetralogy of Fallot and as an adjuvant modality in cardiac catheterization. (Chiba, N.)

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.

Oxaliplatin-Induced Leukocytoclastic Vasculitis under Adjuvant Chemotherapy for Colorectal Cancer: Two Cases of a Rare Adverse Event

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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.

Vasculite cutânea de pequenos vasos: etiologia, patogênese, classificação e critérios diagnósticos - Parte I Small vessel cutaneous vasculitis: etiology, pathogenesis, classification and diagnostic criteria - Part I

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Hebert Roberto Clivati Brandt

2007-10-01

Full Text Available Vasculite é a inflamação da parede dos vasos. Pode variar em gravidade desde doença autolimitada de um único órgão até doença grave com risco de morte por falência de múltiplos órgãos. Existem várias causas, embora só se apresente por poucos padrões histológicos de inflamação vascular. Vasos de qualquer tipo e em qualquer órgão podem ser afetados, resultando em ampla variedade de sinais e sintomas. Diferentes vasculites com apresentações clínicas indistinguíveis têm evolução e tratamento muito diferentes. Essa condição representa desafio para o médico, incluindo classificação, diagnóstico, exames laboratoriais pertinentes, tratamento e seguimento adequado. Neste artigo são revistos a classificação, a etiologia, a patogênese e os critérios diagnósticos das vasculites cutâneas.Vasculitis is an inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple-organ failure. It has many causes, although they result in only a few histological patterns of vascular inflammation. Vessels of any type in any organ can be affected, a fact that results in a broad variety of signs and symptoms. Different vasculitides with indistinguishable clinical presentations have very different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, treatment, and the need for careful follow-up. This article reviews the classification, etiology, pathology and diagnostic criteria of cutaneous vasculitis.

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

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....

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

18F-fluoro-deoxy-glucose positron emission tomography combined with computed tomography can reliably rule-out infection and cancer in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis suspected of disease relapse

DEFF Research Database (Denmark)

Frary, Evan C; Hess, Søren; Gerke, Oke

2017-01-01

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune diseases characterized by systemic inflammation in small- to medium-sized blood vessels. Although immunosuppressive therapy has greatly improved the prognosis for these patients, there are still...

Depressurization as a means of leak checking large vacuum vessels

International Nuclear Information System (INIS)

Callis, R.W.; Langhorn, A.; Petersen, P.I.; Ward, C.; Wesley, J.

1985-01-01

A common problem associated with large vacuum vessels used in magnetic confinement fusion experiments is that leak checking is hampered by the inaccessibility to most of the vacuum vessel surface. This inaccessibility is caused by the close proximity of magnetic coils, diagnostics and, for those vessels that are baked, the need to completely surround the vessel with a thermal insulation blanket. These obstructions reduce the effectiveness of the standard leak checking method of using a mass spectrometer and spraying a search gas such as helium on the vessel exterior. Even when the presence of helium is detected, its entry point into the vessel cannot always be pinpointed. This paper will describe a method of overcoming this problem. By slightly depressurizing the vessel, an influx of helium through the leak is created. The leak site can then be identified by personnel within the vessel using standard sniffing procedures. There are two conditions which make this method of leak checking practical. First, the vessel need only be depressurized 2 psi, thus allowing personnel inside to perform the sniffing operation. Second, the sniffing probe used (Leybold--Heraus ''Quick Test'') could detect a change in helium concentration as small as 100 ppb, which allows for faster scanning of the vessel inferior. Use of this technique to find an elusive 10 -3 Torrxl/s leak in the Doublet III tokamak vacuum vessel will be presented

Concordance of Time-of-Flight MRA and Digital Subtraction Angiography in Adult Primary Central Nervous System Vasculitis.

Science.gov (United States)

de Boysson, H; Boulouis, G; Parienti, J-J; Touzé, E; Zuber, M; Arquizan, C; Dequatre, N; Detante, O; Bienvenu, B; Aouba, A; Guillevin, L; Pagnoux, C; Naggara, O

2017-10-01

3D-TOF-MRA and DSA are 2 available tools to demonstrate neurovascular involvement in primary central nervous system vasculitis. We aimed to compare the diagnostic concordance of vessel imaging using 3D-TOF-MRA and DSA in patients with primary central nervous system vasculitis. We retrospectively identified all patients included in the French primary central nervous system vasculitis cohort of 85 patients who underwent, at baseline, both intracranial 3D-TOF-MRA and DSA in an interval of no more than 2 weeks and before treatment initiation. Two neuroradiologists independently reviewed all 3D-TOF-MRA and DSA imaging. Brain vasculature was divided into 25 arterial segments. Concordance between 3D-TOF-MRA and DSA for the identification of arterial stenosis was assessed by the Cohen κ Index. Thirty-one patients met the inclusion criteria, including 20 imaged with a 1.5T MR unit and 11 with a 3T MR unit. Among the 25 patients (81%) with abnormal DSA findings, 24 demonstrated abnormal 3D-TOF-MRA findings, whereas all 6 remaining patients with normal DSA findings had normal 3D-TOF-MRA findings. In the per-segment analysis, concordance between 1.5T 3D-TOF-MRA and DSA was 0.82 (95% CI, 0.75-0.93), and between 3T 3D-TOF-MRA and DSA, it was 0.87 (95% CI, 0.78-0.91). 3D-TOF-MRA shows a high concordance with DSA in diagnostic performance when analyzing brain vasculature in patients with primary central nervous system vasculitis. In patients with negative 3T 3D-TOF-MRA findings, the added diagnostic value of DSA is limited. © 2017 by American Journal of Neuroradiology.

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.)

Magnetic resonance angiography in suspected cerebral vasculitis

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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.)

Contribution to the pathogenesis of radiation-induced injury to large arteries

International Nuclear Information System (INIS)

Zidar, Nina; Ferluga, Dusan; Hvala, Asta; Popovic, Mara; Soba, Erika

1997-01-01

We report a case of a 35-year-old man who died of a brain infarct 20 months after radiotherapy for carcinoma of the tonsil with metastases to the cervical lymph nodes. Histology revealed mild atherosclerosis, necrotizing vasculitis, and occlusive thrombosis of the internal carotid artery. Significant changes were observed in the vasa vasorum; swelling and detachment of the endothelium, subendothelial oedema, hyaline change, fibrinoid necrosis of the vessel walls with mononuclear cellular infiltration, accompanied by focal haemorrhages and chronic inflammation in the periadventitial soft tissue. We believe that these changes of the vasa vasorum and necrotizing vasculitis are causally related and that vasculitis represents focal ischaemic necroses with inflammatory reaction. Our findings support the hypothesis, based on experimental studies, that injury to the vasa vasorum is an important mechanism in the development of radiation-induced vasculopathy of large arteries. They also suggest an evolution of the injury to the vasa vasorum and periadventitial tissue from the early lesions described in our patient, to late stages resulting in dense periadventitial fibrosis as reported previously. We suggest that injury to the vasa vasorum and the consequent ischaemic lesions of the arterial wall are morphological features distinguishing radiation-induced arterial injury from spontaneous atherosclerosis. (author)

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....

Cocaine-induced vasculitis: is this a new trend?

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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

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.

Stroke in Ehlers-Danlos Syndrome Kyphoscoliotic Type: Dissection or Vasculitis?

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Quade, Annegret; Wiesmann, Martin; Weis, Joachim; Kurth, Ingo; Jalaie, Houman; Rohrbach, Marianne; Häusler, Martin

2017-09-01

Patients with the kyphoscoliotic type of Ehlers-Danlos syndrome have an increased risk of vascular complications such as aortic dissection and perforation. Cerebral ischemia has only rarely been documented. This 13-year-old girl with the kyphoscoliotic type of Ehlers-Danlos syndrome experienced a large right middle cerebral artery distribution infarction. Full intravenous heparinization was started in response to presumed arterial dissection. Magnetic resonance imaging studies including magnetic resonance angiography and digital subtraction angiography, however, did not confirm dissection but suggested with cerebral vasculitis extending from the intradural right internal carotid artery to the M2 branches of the middle cerebral artery. Combined steroid and cyclophosphamide therapy was associated with clinical improvement. Two months later she died from hemorrhagic shock caused by a two-sided spontaneous rupture of the aortic artery. Cerebral vasculitis should be included in the differential diagnosis of vascular complications in kyphoscoliotic type of Ehlers-Danlos syndrome. Copyright © 2017 Elsevier Inc. All rights reserved.

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.

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.

Vasculitis Terms A to Z

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... Our Board Our Team Anniversary Vasculitis Foundation Newsletters Annual Reports Press Page Literature Order Form Join Contact Us Awareness Donate Donate Support the VF Through the Amazon Smile Program United Way Giving Ways to Give ...

Fibrosis pulmonar asociada a vasculitis con anticuerpos anticitoplasmáticos positivos Pulmonary fibrosis associated with anti-neutrophil cytoplasmic antibody-positive vasculitis

Directory of Open Access Journals (Sweden)

Marcelo Fernández Casares

2012-08-01

Full Text Available Las complicaciones pulmonares más conocidas de las vasculitis con anticuerpos anticitoplasmáticos de los neutrófilos (ANCA positivos (VAA, son la hemorragia alveolar, los granulomas y la estenosis de la vía aérea. En los últimos años han aparecido algunos informes aislados que muestran la asociación con fibrosis pulmonar (FP, sugiriendo que ésta sería otra complicación de las VAA. En este trabajo informamos dos casos con dicha asociación describiendo sus características clínicas, tomográficas e inmunológicas. Dado que en la asociación de FP y VAA notificada en los últimos años, la FP puede ser su primera manifestación, podría ser necesaria la búsqueda de ANCA en pacientes con FP, como causa de la misma y por el posible desarrollo posterior de vasculitis.The most frequently observed pulmonary complications of vasculitis (AAV with anti-neutrophil cytoplasmic positive antibodies (ANCA are alveolar hemorrhage, granulomas and airway stenosis. In recent years, some reports have been published that show the association of vasculitis with pulmonary fibrosis (PF, suggesting that it may be another complication of AAV. We report and describe here two cases with such association, and their clinical, tomographic and immunological characteristics. Given that in the association between PF and AAV, as reported in the last years, PF could be the first manifestation of AAV, the search for ANCA in patients with PF may be necessary, as a cause of it and for the possible subsequent development of vasculitis.

[Glomerulonephritis and vasculitis as causes of arterial hypertension].

Science.gov (United States)

Eicken, Sibylle; Gugger, Mathias; Marti, Hans-Peter

2012-05-01

The various types of glomerulonephritis, including many forms of vasculitis, are responsible for about 15% of cases of end-stage renal disease (ESRD). Arterial hypertension represents a frequent finding in patients suffering from glomerulonephritis or vasculitis and hypertension also serves as an indicator for these severe types of diseases. In addition, there are symptoms and signs like hematuria, proteinuria and renal failure. Especially, rapidly progressive glomerulonephritis (RPGN) constitutes a medical emergency and must not be missed by treating physicians. This disease can either occur limited to the kidneys or in the context of a systemic inflammatory disorder, like a vasculitis. If left untreated, RPGN can lead to a necrotizing destruction of glomeruli causing irreversible kidney damage within several months or even weeks. With respect to the immunologically caused vasculitis, there are - depending upon the severity and type of organ involved - many clinical warning signs to be recognized, such as arterial hypertension, hemoptysis, arthalgias, muscle pain, palpable purpura, hematuria, proteinuria and renal failure. In addition, constitutional signs, such as fever and loss of body weight may occur concurrently. Investigations of glomerulonephritis or vasculitis must contain a careful and complete examination of family history and medications used by the respective patient. Thereafter, a thorough clinical examination must follow, including skin, joints and measurement of arterial blood pressure. In addition, a spectrum of laboratory analyses is required in blood, such as full blood screen, erythrocyte sedimentation rate, CRP, creatinine, urea and glucose, and in urine, including urinalysis looking for hematuria, red cell casts and proteinuria. Importantly, proteinuria needs to be quantified by the utilization of a random urine sample. Proteinuria > 3g/d is diagnostic for a glomerular damage. These basic tests are usually followed by more specialized analyses

Large inelastic deformation analysis of steel pressure vessels at high temperature

International Nuclear Information System (INIS)

Ikonen, K.

2001-01-01

This publication describes the calculation methodology developed for a large inelastic deformation analysis of pressure vessels at high temperature. Continuum mechanical formulation related to a large deformation analysis is presented. Application of the constitutive equations is simplified when the evolution of stress and deformation state of an infinitesimal material element is considered in the directions of principal strains determined by the deformation during a finite time increment. A quantitative modelling of time dependent inelastic deformation is applied for reactor pressure vessel steels. Experimental data of uniaxial tensile, relaxation and creep tests performed at different laboratories for reactor pressure vessel steels are investigated and processed. An inelastic deformation rate model of strain hardening type is adopted. The model simulates well the axial tensile, relaxation and creep tests from room temperature to high temperature with only a few fitting parameters. The measurement data refined for the inelastic deformation rate model show useful information about inelastic deformation phenomena of reactor pressure vessel steels over a wide temperature range. The methodology and calculation process are validated by comparing the calculated results with measurements from experiments on small scale pressure vessels. A reasonably good agreement, when taking several uncertainties into account, is obtained between the measured and calculated results concerning deformation rate and failure location. (orig.)

Does vasculitis alone cause AVN? A review of literature.

Science.gov (United States)

Abraham, Rtika R; Meyerhoff, John O

2013-10-01

AVN is caused by a disease, or severe trauma that affects the blood supply to the bone or in many cases may be idiopathic, with no known cause. AVN pathophysiology is most closely linked to SLE literature, and there is a strong cause and effect relationship between corticosteroid intake and AVN development in SLE patients, and AVN is extremely rare in the absence of steroid use. Apart from few anecdotal reports, there is no data on exact pathophysiologic mechanisms responsible for AVN in the setting of vasculitis. We saw a 69-year-old man with femoral AVN and a possibility of vasculitis as the underlying cause was raised by the radiologist, and hence we present this literature search on vasculitis per se causing AVN of the bone.

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.

Storkarsvaskulitis påvist ved PET/CT hos patient med initialt formodet cancer

DEFF Research Database (Denmark)

Andersen, Kim Francis; Skøt, Jens; Bülow, Jens

2012-01-01

Large vessel vasculitis, including giant cell arteritis and Takayasu arteritis, is traditionally diagnosed and classified according to the American College of Rheumatology criteria, which do not include findings on imaging modalities. We present a case in which non-invasive imaging with 18F......-fluorodeoxyglucose positron emission tomography/computed tomography gave essential information in the diagnostic work-up of large vessel vasculitis in a female presenting with non-specific symptoms. We discuss the role of nuclear medicine imaging in early diagnosis and follow-up of this inflammatory disease, characterized...

Acute posterior multifocal placoid pigment epitheliopathy associated with cerebral vasculitis.

Science.gov (United States)

Weinstein, J M; Bresnick, G H; Bell, C L; Roschmann, R A; Brooks, B R; Strother, C M

1988-09-01

Acute multifocal posterior placoid pigment epitheliopathy (APMPPE) is an unusual self-limited retinal disorder that has been associated with various systemic complications. To our knowledge, three prior cases associated with cerebral vasculitis have been described. This article describes a patient with APMPPE and angiographically documented cerebral vasculitis who was notable because of (a) the presence of two different cerebral ischemic events, occurring 1 month apart, and (b) the long latency (3 months) between the onset of ocular symptoms and the second cerebral ischemic event. Recognition of the association between APMPPE and cerebral vasculitis may permit early treatment of CNS involvement and prevention of morbidity.

HIFU procedures at moderate intensities-effect of large blood vessels

International Nuclear Information System (INIS)

Hariharan, P; Myers, M R; Banerjee, R K

2007-01-01

A three-dimensional computational model is presented for studying the efficacy of high-intensity focused ultrasound (HIFU) procedures targeted near large blood vessels. The analysis applies to procedures performed at intensities below the threshold for cavitation, boiling and highly nonlinear propagation, but high enough to increase tissue temperature a few degrees per second. The model is based upon the linearized KZK equation and the bioheat equation in tissue. In the blood vessel the momentum and energy equations are satisfied. The model is first validated in a tissue phantom, to verify the absence of bubble formation and nonlinear effects. Temperature rise and lesion-volume calculations are then shown for different beam locations and orientations relative to a large vessel. Both single and multiple ablations are considered. Results show that when the vessel is located within about a beam width (few mm) of the ultrasound beam, significant reduction in lesion volume is observed due to blood flow. However, for gaps larger than a beam width, blood flow has no major effect on the lesion formation. Under the clinically representative conditions considered, the lesion volume is reduced about 40% (relative to the no-flow case) when the beam is parallel to the blood vessel, compared to about 20% for a perpendicular orientation. Procedures involving multiple ablation sites are affected less by blood flow than single ablations. The model also suggests that optimally focused transducers can generate lesions that are significantly larger (>2 times) than the ones produced by highly focused beams

HIFU procedures at moderate intensities-effect of large blood vessels

Energy Technology Data Exchange (ETDEWEB)

Hariharan, P [Mechanical, Industrial, and Nuclear Engineering Department, University of Cincinnati, Cincinnati, OH (United States); Myers, M R [Division of Solid and Fluid Mechanics, Center for Devices and Radiological Health, US Food and Drug Administration, 10903 New Hampshire Avenue, Building 62, Silver Spring, MD 20993-0002 (United States); Banerjee, R K [Mechanical, Industrial, and Nuclear Engineering Department, University of Cincinnati, Cincinnati, OH (United States)

2007-07-21

A three-dimensional computational model is presented for studying the efficacy of high-intensity focused ultrasound (HIFU) procedures targeted near large blood vessels. The analysis applies to procedures performed at intensities below the threshold for cavitation, boiling and highly nonlinear propagation, but high enough to increase tissue temperature a few degrees per second. The model is based upon the linearized KZK equation and the bioheat equation in tissue. In the blood vessel the momentum and energy equations are satisfied. The model is first validated in a tissue phantom, to verify the absence of bubble formation and nonlinear effects. Temperature rise and lesion-volume calculations are then shown for different beam locations and orientations relative to a large vessel. Both single and multiple ablations are considered. Results show that when the vessel is located within about a beam width (few mm) of the ultrasound beam, significant reduction in lesion volume is observed due to blood flow. However, for gaps larger than a beam width, blood flow has no major effect on the lesion formation. Under the clinically representative conditions considered, the lesion volume is reduced about 40% (relative to the no-flow case) when the beam is parallel to the blood vessel, compared to about 20% for a perpendicular orientation. Procedures involving multiple ablation sites are affected less by blood flow than single ablations. The model also suggests that optimally focused transducers can generate lesions that are significantly larger (>2 times) than the ones produced by highly focused beams.

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.

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.

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.

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.)

CT findings at lupus mesenteric vasculitis

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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.).

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

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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.

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

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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.

Present and future management of anti-neutrophil cytoplasmic antibody associated vasculitis: how therapy changed the prognosis

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Massimo L’Andolina

2013-03-01

Full Text Available Anti-neutrophil cytoplasmic antibody associated vasculitis is part of a multi-systemic idiopathic, small vessel pouci-immune vasculitis. Given the heterogeneous spectrum of the disease, and the need to update therapeutic protocols, the aim of this review was to evaluate clinical-diagnostic approaches. We examined statistical data available in the literature, in particular the 2010 review of St. Hamour et al. Management of Anca-associated Vasculitis, published in Therapeutics and Clinical Risk Management. Acute immunosuppressive therapy and long-term maintenance, with the use of prednisolone, have significantly changed the prognosis of this disease, particularly compared with the 1970s before the introductions of steroids and cyclophosphamide. New drugs such as rituximab, monoclonal antibodies and other modulating immune system molecules are entering clinical use, and experience will confirm whether or not therapeutic guidelines are appropriate. The current diagnostic tools, ranging from laboratory and autoimmune tests, chest X-ray, broncho-alveolar lavage to capillaroscopy, allow prompt diagnosis and early treatment through a first phase of induction-remission, and a second phase of maintenance. There are, however, recurrent and refractory forms of the disease that require long-term immunosuppression and further research into this is merited. These issues have continued to drive the search for safer and more effective modulation of the immune system using targeted immunotherapy. However, the treatment limitations of incomplete efficacy, infection, and cumulative toxicity persist. Modifications to traditional treatment protocols by the use of azathioprine or methotrexate rather than cyclophosphamide, and the introduction of newer agents, such as rituximab, have meant that outcomes have been maintained while toxicity has been reduced.

Prednisone and vardenafil hydrochloride for refractory levamisole-induced vasculitis.

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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.

Eosinophilic leukocytoclastic vasculitis - a spectrum ranging from Wells' syndrome to Churg-Strauss syndrome?

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Ratzinger, Gudrun; Zankl, Julia; Eisendle, Klaus; Zelger, Bernhard

2014-01-01

Wells' syndrome is defined as an inflammatory disorder with the histopathological presence of eosinophilic infiltrates and flame figures in the absence of vasculitis. Eosinophilic leukocytoclastic vasculitis shows eosinophilic infiltrates in combination with vasculitic changes. And Churg Strauss Syndrome comprises all three characteristics - eosinophilic infiltrates, vasculitis and flame figures. To determine whether these three diseases are distinct entities or different manifestations of a similar clinicopathologic process. Histopathological samples and clinical courses of 17 patients with eosinophilic infiltrates, flame figures and clinical features of Wells' syndrome were re-evaluated. Histopathologically, we focused on the presence or absence of vasculitic features. Clinically, we included only patients who were diagnosed with Wells' syndrome at least once in the course of their disease. 4 patients were finally diagnosed with Wells' syndrome, 5 with eosinophilic leukocytoclastic vasculitis and 6 with Churg Strauss syndrome. Further, we had one case of an overlap between Wells' syndrome and eosinophilic vasculitis and one case of Wegener granulomatosis. Vasculitic features were found in the samples of all patients. Histologically, we find vasculitic features in typical presentations of Wells' syndrome. Clinically, we find typical features of Wells' syndrome in patients finally diagnosed with eosinophilic leukocytoclastic vasculitis or Churg Strauss syndrome. Furthermore, we have observed and formerly reported 3 patients with progression from Wells' syndrome to Churg Strauss syndrome. Thus, we assume that eosinophilic leukocytoclastic vasculitis might form a bridge between Wells' syndrome and Churg Strauss syndrome.

Facial ulcerations due to Acinetobacter baumannii: Vessel thrombosis with bacterial mycelia

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Dong Ming Li

2014-01-01

Full Text Available A 14-year-old girl presented with a 2-week history of progressive facial ulcerations that did not respond to cephalexin and topical dexamethasone. Biopsy on the ulcer showed rod-shaped bacteria and actinomycetes-like mycelia in the vessel walls and within thrombi. Tissue culture yielded Acinetobacter baumannii, which was resistant to cephalexin. A favourite outcome was achieved with minocycline treatment. This is the first case report of A. baumannii-related vasculitis.

Educational Needs of Patients With Systemic Vasculitis

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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

Central nervous system vasculitis caused by propylthiouracil therapy: a case report and literature review.

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Vanek, C; Samuels, M H

2005-01-01

Antineutrophil cytoplasmic antibodies (ANCA) are associated with vasculitis, including vasculitis induced by drugs such as the thionamides. The affected organ systems in thionamide-induced vasculitis have been primarily renal, musculoskeletal, and dermatologic. We describe the first case of thionamide-induced central nervous system vasculitis presenting as confusion, with complete resolution after discontinuation of propylthiouracil. We review the literature and summarize 42 additional cases of thionamide-induced ANCA-positive vasculitis since 1992. Propylthiouracil was responsible in 93% of cases and the predominant ANCA pattern on immunofluorescent staining was perinuclear (p-ANCA). Clinical improvement occurred after drug discontinuation in 93%, steroid therapy was used in some cases. The mean duration of treatment with thionamides was 35 months prior to presentation. Long-term medical treatment with thionamides for hyperthyroidism may increase the risk of this severe side effect.

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

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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.

Feasibility of local stress relieving close to main shell of a large vessel

International Nuclear Information System (INIS)

Hancinsky, O.A.

1978-01-01

This work determines the feasibility of local stress relieving for a circumferential pipe-to-nozzle field weld positioned close to the main shell of a large pressure vessel. This is applicable to nuclear as well as conventional vessels. ANSYS computer program is utilized to perform thermal and thermal stress analysis and ASME Pressure Vessels Code is adhered to. Conclusions and recommendations are made with a view on their applicability in practice

Focal cerebral vasculitis associated with circulating immune complexes and brain irradiation

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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.

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

Circulating Markers of Vascular Injury and Angiogenesis in ANCA-Associated Vasculitis

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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

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

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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

Acute kidney injury with granulomatous interstitial nephritis and vasculitis revealing sarcoidosis

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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.

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

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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.

Forging technology for large nuclear pressure vessel parts

International Nuclear Information System (INIS)

Kakimoto, Hideki; Ikegami, Tomonori

2014-01-01

The increasing output of nuclear power generation calls for larger vessels for next-generation nuclear power plants. A vessel with an increased diameter requires increased load for its forging, which can make it difficult to use a conventional solid die. In order to reduce the forging load, a rotary incremental forging method has been applied to hot forging. This method includes pressing and rotating a material in an incremental manner such that a target shape is obtained. This study aimed at improving the accuracy of numerical simulation for the rotary incremental forging to reduce the load when forging large vessels. This has enabled the temperature of the material and flow stress to be precisely predicted; an example of this is reported in the paper. Specifically, the heat transfer coefficient to be used for the numerical simulation had been determined experimentally from a small-scale hot-forging. The reduction of the flow stress associated with incremental forging, had been deduced from a compression test, and the value was applied to the numerical simulation. A preform was designed on the basis of the above simulation to perform a 1/1 size scale experiment. A precision of better than 5% has been confirmed for the shape prediction. (author)

Idiopathic Pulmonary Hemosiderosis in a Young Adult Patient: A Rare Case

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Abhishek Agarwal

2018-01-01

Full Text Available Idiopathic pulmonary hemosiderosis (IPH is often an ignored and rare cause of diffuse alveolar hemorrhage (DAH. It is characterized by triad of hemoptysis, anemia, and alveolar opacity on radiology. It is a diagnosis of exclusion, established after ruling out other causes of DAH such as Goodpasture’s syndrome, large vessel vasculitis, small vessel vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA (Wegener’s granulomatosis, Churg–Strauss syndrome, microscopic polyangiitis, immune complex-related vasculitis (collagen vascular diseases, Henoch–Schönlein purpura, mixed cryoglobulinemia drug reactions, anticoagulation and thrombocytopenia. Though it is a disease primarily affecting children, we hereby report a case of IPH in an adult patient who responded dramatically to oral corticosteroid.

LARGE VESSEL INVOLVEMENT IN BEHCET’S DISEASE

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AR. Jamshidi F. Davatchi

2004-08-01

Full Text Available Large vessel involvement is one of the hallmarks of Behcet’s disease (BD but its prevalence varies widely due to ethnic variation or environmental factors. The aim of this study is to find the characteristics of vasculo-Behcet (VB in Iran. In a cohort of 4769 patients with BD, those with vascular involvement were selected. Different manifestations of disease were compared with the remaining group of patients. A confidence interval at 95% (CI was calculated for each item. Vascular involvement was seen in 409 cases (8.6%; CI, 0.8. Venous involvement was seen in 396 cases, deep vein thrombosis in 294 (6.2%; CI, 0.7, superficial phlebitis in 108 (2.3%; CI, 0.4 and large vein thrombosis in 45 (0.9%; CI, 0.3. Arterial involvement was seen in 28 patients (25 aneurysms and 4 thromboses. Thirteen patients showed both arterial and venous involvement. The mean age of the patients with VB was slightly higher (P<0.03, but the disease duration was significantly longer (P<0.0003. VB was more common in men. As the presenting sign, ocular lesions were less frequent in VB (P<0.0006, while skin lesions were over 2 times more common in these cases (P<0.000001. VB was associated with a higher frequency of genital aphthosis, skin involvement, joint manifestations, epididymitis, CNS lesions and GI involvement. The juvenile form was less common in VB (P<0.03. High ESR was more frequent in VB (P=0.000002, but the frequency of false positive VDRL, pathergy phenomenon, HLA-B5 or HLA-B27 showed no significant difference between the two groups. In Iranian patients with BD, vascular involvement is not common and large vessel involvement is rare. It may be sex-related, and is more common in well-established disease with multiple organ involvement and longer disease duration.

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

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

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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.

Theoretical evaluations of magnetic nanoparticle-enhanced heating on tumor embedded with large blood vessels during hyperthermia

International Nuclear Information System (INIS)

Wang, Q.; Deng, Z. S.; Liu, J.

2012-01-01

The large blood vessels surrounding the tumor would significantly result in heat sink, and thus seriously limit the thermal ablative area during tumor hyperthermia. Magnetic nanoparticle (MNP) was recently identified as an important heating enhancer to improve the treatment efficiency. It will not only help to absorb more energy under the irradiation of external magnetic field, but also can block the blood flow and subsequently weaken the heat sink effect of large vessels. In this study, these two critical factors, reserved to be undisclosed before in theory, were comprehensively investigated through three-dimensional numerical simulation. The results suggested that concerning the contribution to temperature increase in the tissues surrounding large vessel, the factor of blood flow blocking is more effective than that of energy absorption. Therefore, selective loading of MNPs to the target sites is expected to serve as a promising method to perform successful hyperthermia treatment for tumor tissues embedded with large blood vessels.

Theoretical evaluations of magnetic nanoparticle-enhanced heating on tumor embedded with large blood vessels during hyperthermia

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Wang, Q. [Tsinghua University, Department of Biomedical Engineering, School of Medicine (China); Deng, Z. S. [Chinese Academy of Sciences, Key Laboratory of Cryogenics, Technical Institute of Physics and Chemistry (China); Liu, J., E-mail: jliubme@tsinghua.edu.cn [Tsinghua University, Department of Biomedical Engineering, School of Medicine (China)

2012-07-15

The large blood vessels surrounding the tumor would significantly result in heat sink, and thus seriously limit the thermal ablative area during tumor hyperthermia. Magnetic nanoparticle (MNP) was recently identified as an important heating enhancer to improve the treatment efficiency. It will not only help to absorb more energy under the irradiation of external magnetic field, but also can block the blood flow and subsequently weaken the heat sink effect of large vessels. In this study, these two critical factors, reserved to be undisclosed before in theory, were comprehensively investigated through three-dimensional numerical simulation. The results suggested that concerning the contribution to temperature increase in the tissues surrounding large vessel, the factor of blood flow blocking is more effective than that of energy absorption. Therefore, selective loading of MNPs to the target sites is expected to serve as a promising method to perform successful hyperthermia treatment for tumor tissues embedded with large blood vessels.

76 FR 27897 - Security and Safety Zone Regulations, Large Passenger Vessel Protection, Captain of the Port...

Science.gov (United States)

2011-05-13

... DEPARTMENT OF HOMELAND SECURITY Coast Guard 33 CFR Part 165 [Docket No. USCG-2011-0342] Security and Safety Zone Regulations, Large Passenger Vessel Protection, Captain of the Port Columbia River... will enforce the security and safety zone in 33 CFR 165.1318 for large passenger vessels operating in...

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

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

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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

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.

[Central nervous system vasculitis and of the peripheral nerves in the elderly].

Science.gov (United States)

Boddaert, Jacques; Verny, Marc

2002-11-01

Vasculitis of the nervous system are rare in the elderly. When present, they may constitute an urgent diagnosis and a therapeutic emergency. Clinical expression is rich and without specificity. Atypical signs (unusual course of dementia, systemic signs) or atypical laboratory results (inflammatory syndrome) may suggest the diagnosis of vasculitis. However, as multiple comorbidity is the rule in elderly subjects, searching for intercurrent factors (e.g. atrial fibrilation due to infectious disease causing embolic stroke) may be more contributive than searching for proof of a rare disease (vasculitis) with invasive procedures in this population. Giant cell (temporal) arteritis is the only vasculitis specifically related with age; the vital prognosis of vision may be compromised. Corticosterid therapy must be instituted without delay. Periartritis nodosa begins in 30% of cases after 60 years of age. The clinical features are the same as in younger subjects. Other vasculidis are rare in the elderly. In absence of specific studies in this population, therapeutic protocols are the same as in younger subjects but may have to be adjusted.

Effects of irradiation on renal vessels of rabbits

International Nuclear Information System (INIS)

Hanayama, Hirotaka

1982-01-01

The male rabbits weighing approximately 3 kg were divided into two groups: one group received irradiation to the left kidney through 6 x 4 cm field size with doses of 5 Gy, while the other group was irradiated with 50 Gy. The vessel casts were observed using the injection replica scanning electron microscopic method. In 50 Gy irradiated group, the surface of blood vessel's lumen became irregular with a decrease in the number of glomeruli at 5 days. Partial narrowing was also seen in the cortex and glomerular vessels. The number of glomeruli decreased by 48% one week later and 90% 4 weeks later. The cortex vessels progressively narrowed, and irregularity of the net-like structure was also observed. Similarly, a decrease in the number of glomeruli and partial narrowing of the blood vessels were seen at 5 days on the nonirradiated side, but the damage was considerably less than that on the irradiated side. In the 5 Gy irradiated group, a decrease in the number of glomeruli was seen 5 days later and it became 71% 5 days later and 81% one week later. It returned to 95% 4 weeks later. No changes whatsoever were seen in the non-irradiated side after 4 weeks and changes in the number of glomeruli were not observed. The vasculation of medulla showed no changes in the 50 Gy or the 5 Gy irradiated group. There were seen no significant changes in the blood vessel diameter. (J.P.N.)

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.

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...

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

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...

Study on external reactor vessel cooling capacity for advanced large size PWR

International Nuclear Information System (INIS)

Jin Di; Liu Xiaojing; Cheng Xu; Li Fei

2014-01-01

External reactor vessel cooling (ERVC) is widely adopted as a part of in- vessel retention (IVR) in severe accident management strategies. In this paper, some flow parameters and boundary conditions, eg., inlet and outlet area, water inlet temperature, heating power of the lower head, the annular gap size at the position of the lower head and flooding water level, were considered to qualitatively study the effect of them on natural circulation capacity of the external reactor vessel cooling for an advanced large size PWR by using RELAP5 code. And the calculation results provide some basis of analysis for the structure design and the following transient response behavior of the system. (authors)

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

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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.

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...

Complete remission of coronary vasculitis in Churg-Strauss Syndrome by prednisone and cyclophosphamide

NARCIS (Netherlands)

Riksen, N.P.; Gehlmann, H.R.; Brouwer, A.E.; Deuren, M. van

2013-01-01

The heart is involved in up to 50% of all patients with Churg-Strauss syndrome, but vasculitis of the coronary arteries has only been rarely documented. We present a young patient with severe coronary aneurysms and stenotic lesions due to a Churg-Strauss vasculitis. Prompt therapy with prednisone

F-18 FDG PET/CT Findings of a Patient with Takayasu Arteritis Before and After Therapy

Directory of Open Access Journals (Sweden)

Sait Sağer

2012-04-01

Full Text Available Vasculitis is defined as inflammation and necrosis with leukocytic infiltration of the blood vessel wall. Takayasu arteritis is a chronic inflammatory arteritis that primarily involves the aorta and its main branches. A 64-year-old female patient with a 2-month history of fever of unknown origin was presented to our clinic for F-18 FDG PET/CT imaging. Baseline PET/CT images demonstrated intense F-18 FDG uptake in the aorta, bilateral subclavian and brachiocephalic arteries consistent with Takayasu arteritis. After 2 months of immunosuppressive therapy, she was asymptomatic and follow-up FDG PET/CT scan showed almost complete disappearance of large vessels’ F-18 FDG uptake. FDG PET/CT is a sensitive technique for assessing presence of large-vessel vasculitis such as Takayasu arteritis, extent of large-vessel inflammation and disease activity after therapy. (MIRT 2012;21:32-34

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

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?

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

Vasculites pulmonares: quando suspeitar e como fazer o diagnóstico Pulmonary vasculitis: when suspicion equal diagnosis

Directory of Open Access Journals (Sweden)

Carmen Sílvia Valente Barbas

2005-07-01

Full Text Available As vasculites pulmonares primárias são caracterizadas por processo inflamatório na parede dos vasos pulmonares que leva a isquemia e hemorragia pulmonar com as conseqüentes expressões clínicas e radiológicas. As vasculites pulmonares primárias são acompanhadas de expressão sistêmicas cutâneas, em nervos periféricos, rins, seios da face, olhos e ouvidos, além do trato gastrintestinal, e sistemas cardíaco e nervoso central. O diagnóstico é feito através da associação das informações clínicas, radiológicas e anatomopatológicas. O tratamento com corticosteróides e imunossupressores deve ser instituído precocemente e apresenta altas taxas de remissão.The primary forms of pulmonary vasculitis are characterized by an inflammatory process in the pulmonary vessel walls, leading to pulmonary ischemia and hemorrhage and the consequent clinical and radiological manifestations. These forms of vasculitis are accompanied by symptoms involving the skin, peripheral nerves, kidneys, sinuses, eyes, ears and gastrointestinal tract, as well as the cardiac and central nervous systems. The diagnosis is made through analysis of the clinical, radiological and pathological data. When treatment with corticosteroids and immunosuppressive therapy is initiated early, remission rates are high.

The design of lifting attachments for the erection of large diameter and heavy wall pressure vessels

International Nuclear Information System (INIS)

Antalffy, Leslie P.; Miller, George A.; Kirkpatrick, Kenneth D.; Rajguru, Anil; Zhu, Yong

2016-01-01

Lifting attachments for the erection of large diameter and heavy wall pressure vessels require special consideration to ensure that their attachment to their vessel shells or heads do not overstress the vessel during the erection process when lifting these from grade onto their respective foundations. Today, in refinery and petrochemical services, large diameter vessels with diameters ranging up to 15 m and reactors with lifting weights in the range of 700–1400 tons are not uncommon. In today's fabrication market, these vessels may be purchased and fabricated in shops dispersed globally and will require unique equipment for their safe handling, transportation and subsequent erection. The challenge is to design the lifting attachments in such a manner that the attachments provide a safe, cost effective and effective solution based upon the limitations of the job site lift equipment available for erection. Such equipment for the transportation and subsequent lifting of large diameter and heavy wall pressure equipment is usually scarce and quite expensive. Planning ahead, well in advance of the lift date is almost a mandatory requirement. Usually, the specific parameters of the vessel to be lifted and the lifting equipment available at the site will dictate the type of lifting attachments to be designed for the vessel. Once the type of vessel attachment has been chosen, careful consideration must be given to the design of attachments to the pressure vessel in consideration to ensure that the vessel and lifting components are not overstressed during the lifting process. The paper also discusses different types of lifting attachments that may be attached to each end of the vessel either by bolting or welding and discusses the pros and cons of each. The paper also provides an example of a finite element analysis (FEA) of a top nozzle, a FEA of a pair of lifting trunnions and a FEA of welded on lifting lugs for buried pipe. The purpose of the paper is to outline the

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

Scoping calculations for design and analysis of large reactor vessels for liquid-metal fast breeder reactor (LMFBR) plants

International Nuclear Information System (INIS)

Fiala, C.; Kulak, R.F.; Ma, D.C.; Pan, Y.C.; Seidensticker, R.W.; Wang, C.Y.; Zeuch, W.R.

1982-01-01

Reactor vessels for commercial-sized LMFBR plants are quite large - ranging 40 to 70 ft in diameter and 50 to 70 ft in overall depth. These stainless steel vessels contain liquid sodium at relatively low pressures, but at high temperatures. The resulting thin-walled vessels present the structural designer and analyst with special problems, particularly in providing a balanced design to accommodate seismic loads, design basis accident loads, and thermal loadings. A comprehensive set of scoping calculations - though preliminary in detail and depth of design - provides substantial guidance to the vessel designer for subsequent design iterations. Emphasis is placed on the analysis of the large-diameter top closure of the vessel - the deck structure

Disease: H01658 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available toimmune disease characterized by systemic vasculitis. MPA predominantly affects small-calibre blood vessels...irected against proteinase 3 (PR3-ANCA). Not all patients, however, have ANCA. MPA is clinically characterized by small-vessel vascul...itis primarily affecting the kidneys and the lungs but o

Complete remission of coronary vasculitis in Churg-Strauss Syndrome by prednisone and cyclophosphamide.

Science.gov (United States)

Riksen, Niels P; Gehlmann, Helmut; Brouwer, Annemarie E; van Deuren, Marcel

2013-03-01

The heart is involved in up to 50% of all patients with Churg-Strauss syndrome, but vasculitis of the coronary arteries has only been rarely documented. We present a young patient with severe coronary aneurysms and stenotic lesions due to a Churg-Strauss vasculitis. Prompt therapy with prednisone and cyclophosphamide resulted in the complete resolution of all lesions.

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

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.

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.

An investigation of the flow dependence of temperature gradients near large vessels during steady state and transient tissue heating

International Nuclear Information System (INIS)

Kolios, M.C.; Worthington, A.E.; Hunt, J.W.; Holdsworth, D.W.; Sherar, M.D.

1999-01-01

Temperature distributions measured during thermal therapy are a major prognostic factor of the efficacy and success of the procedure. Thermal models are used to predict the temperature elevation of tissues during heating. Theoretical work has shown that blood flow through large blood vessels plays an important role in determining temperature profiles of heated tissues. In this paper, an experimental investigation of the effects of large vessels on the temperature distribution of heated tissue is performed. The blood flow dependence of steady state and transient temperature profiles created by a cylindrical conductive heat source and an ultrasound transducer were examined using a fixed porcine kidney as a flow model. In the transient experiments, a 20 s pulse of hot water, 30 deg. C above ambient, heated the tissues. Temperatures were measured at selected locations in steps of 0.1 mm. It was observed that vessels could either heat or cool tissues depending on the orientation of the vascular geometry with respect to the heat source and that these effects are a function of flow rate through the vessels. Temperature gradients of 6 deg. C mm -1 close to large vessels were routinely measured. Furthermore, it was observed that the temperature gradients caused by large vessels depended on whether the heating source was highly localized (i.e. a hot needle) or more distributed (i.e. external ultrasound). The gradients measured near large vessels during localized heating were between two and three times greater than the gradients measured during ultrasound heating at the same location, for comparable flows. Moreover, these gradients were more sensitive to flow variations for the localized needle heating. X-ray computed tomography data of the kidney vasculature were in good spatial agreement with the locations of all of the temperature variations measured. The three-dimensional vessel path observed could account for the complex features of the temperature profiles. The flow

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....

Anti-PD-1 Vasculitis of the central nervous system or radionecrosis?

Science.gov (United States)

Sun, Roger; Danlos, Francois-Xavier; Ammari, Samy; Louvel, Guillaume; Dhermain, Frédéric; Champiat, Stéphane; Lambotte, Olivier; Deutsch, Eric

2017-12-19

Commentary on « Cerebral vasculitis mimicking intracranial metastatic progression of lung cancer during PD-1 blockade » by Läubli H et al., J Immunother Cancer. 2017;5:46.The authors diagnosed a cerebral tumor-like lymphocytic vasculitis associated with anti-endothelial cell auto-antibodies secondary to anti-PD-1 therapy, treated by surgical resection and corticosteroids. We thought that this diagnosis should be discussed for at least two reasons. First, etiological explorations were not sufficient. Second, the diagnostic of radionecrosis should also be discussed.

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...

Meningococal Septicemia with Cutaneous Vasculitis

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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

Improving prediction of recanalization in acute large-vessel occlusive stroke.

Science.gov (United States)

Vanacker, P; Lambrou, D; Eskandari, A; Maeder, P; Meuli, R; Ntaios, G; Michel, P

2014-06-01

Recanalization in acute ischemic stroke with large-vessel occlusion is a potent indicator of good clinical outcome. To identify easily available clinical and radiologic variables predicting recanalization at various occlusion sites. All consecutive, acute stroke patients from the Acute STroke Registry and Analysis of Lausanne (2003-2011) who had a large-vessel occlusion on computed tomographic angiography (CTA) (< 12 h) were included. Recanalization status was assessed at 24 h (range: 12-48 h) with CTA, magnetic resonance angiography, or ultrasonography. Complete and partial recanalization (corresponding to the modified Treatment in Cerebral Ischemia scale 2-3) were grouped together. Patients were categorized according to occlusion site and treatment modality. Among 439 patients, 51% (224) showed complete or partial recanalization. In multivariate analysis, recanalization of any occlusion site was most strongly associated with endovascular treatment, including bridging therapy (odds ratio [OR] 7.1, 95% confidence interval [CI] 2.2-23.2), and less so with intravenous thrombolysis (OR 1.6, 95% CI 1.0-2.6) and recanalization treatments performed beyond guidelines (OR 2.6, 95% CI 1.2-5.7). Clot location (large vs. intermediate) and tandem pathology (the combination of intracranial occlusion and symptomatic extracranial stenosis) were other variables discriminating between recanalizers and non-recanalizers. For patients with intracranial occlusions, the variables significantly associated with recanalization after 24 h were: baseline National Institutes of Health Stroke Scale (NIHSS) (OR 1.04, 95% CI 1.02-1.1), Alberta Stroke Program Early CT Score (ASPECTS) on initial computed tomography (OR 1.2, 95% CI 1.1-1.3), and an altered level of consciousness (OR 0.2, 95% CI 0.1-0.5). Acute endovascular treatment is the single most important factor promoting recanalization in acute ischemic stroke. The presence of extracranial vessel stenosis or occlusion decreases

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.

Large potassium dihydrogen phosphate crystal growth using a three-vessel system for fusion lasers

International Nuclear Information System (INIS)

Sasaki, T.; Yokotani, A.; Yamanaka, T.; Nakai, S.; Yamanaka, C.

1989-01-01

Large scale laser fusion experiments are being performed in the Institute of Laser Engineering, Osaka University, using the glass laser system Gekko-XII. For this laser, very large nonlinear crystals of potassium dihydrogen phosphate (KDP) with a cross section over 40 X 40 cm is needed as a frequency converter to obtain a short wavelength laser. Generally the temperature falling method (TFM) is used to grow such a huge crystal, but the volume of the growing vessel becomes tremendously large. The three-vessel system (TVS), which is a constant temperature and concentration method, allows better control of supersaturation than does the TFM, and the volume of the main growth vessel can be smaller than that in the case of the TFM. The authors have constructed a TVS. The KDP crystal grew in the growth tank that was kept at a constant temperature of 20 +- 0.01 0 C. The authors show the growth history of the KDP crystal of a 40- X 40-cm cross section. This system is now being operated to obtain the KDP of 100-cm height, and a theoretical estimate of the growth rate is under consideration. These results are presented

Aortoarteritis: Could it be a form of catecholamine-induced vasculitis?

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Vijaya Sarathi

2013-01-01

Full Text Available Catecholamine-induced vasculitis is a well known but rarely described entity. However, aortoarteritis as a manifestation of catecholamine-induced vasculitis is not described in the literature. We have reported two patients in whom pheochromocytoma coexisted with aortoarteritis. Both patients were young females with history of bilateral pheochromocytomas in more than one first-degree relative. Both patients also had bilateral adrenal pheochromocytomas (second patient also had paraganglioma at left renal hilum with elevation of plasma free normetanephrine levels. We conclude that there may be an association between pheochromocytoma and aortoarteritis, and that catecholamine excess may have a role in the etiopathogenesis of aortoarteritis in these patients.

Aortoarteritis: Could it be a form of catecholamine-induced vasculitis?

Science.gov (United States)

Sarathi, Vijaya; Lila, Anurag R.; Bandgar, Tushar R.; Shah, Nalini S.

2013-01-01

Catecholamine-induced vasculitis is a well known but rarely described entity. However, aortoarteritis as a manifestation of catecholamine-induced vasculitis is not described in the literature. We have reported two patients in whom pheochromocytoma coexisted with aortoarteritis. Both patients were young females with history of bilateral pheochromocytomas in more than one first-degree relative. Both patients also had bilateral adrenal pheochromocytomas (second patient also had paraganglioma at left renal hilum) with elevation of plasma free normetanephrine levels. We conclude that there may be an association between pheochromocytoma and aortoarteritis, and that catecholamine excess may have a role in the etiopathogenesis of aortoarteritis in these patients. PMID:23776874

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

A three-temperature model of selective photothermolysis for laser treatment of port wine stain containing large malformed blood vessels

International Nuclear Information System (INIS)

Li, D.; Wang, G.X.; He, Y.L.; Wu, W.J.; Chen, B.

2014-01-01

As congenital vascular malformations, port wine stain (PWS) is composed of ectatic venular capillary blood vessels buried within healthy dermis. In clinic, pulsed dye laser (PDL) in visible band (e.g. 585 nm) together with cryogen spray cooling (CSC) have become the golden standard for treatment of PWS. However, due to the limited energy deposition of the PDL in blood, large blood vessels are likely to survive from the laser irradiation. As a result, complete clearance of the lesions is rarely achieved. Assuming the local thermal non-equilibrium in skin tissue during the laser surgery, a three-temperature model is proposed to treat the PWS tissue as a porous media composed of a non-absorbing dermal matrix buried with the blood as well as the large malformed blood vessels. Three energy equations are constructed and solved coupling for the temperature of the blood in average-sized PWS vessels, non-absorbing dermal tissues and large malformed blood vessels, respectively. Subsequently, the thermal responses of human skin to visible (585 nm) and near-infrared (1064 nm) laser irradiations with various pulse durations in conjunction with cryogen spray cooling are investigated by the new model, and Arrhenius integral is used to analyze the thermal damage. The simulations show that the short pulse duration of 1.5 ms results in a higher selective heating of blood over epidermis, which will lead to a desired clinic outcome than the longer pulse duration. Due to a much deeper light penetration depth, laser irradiation with 1064 nm in wavelength is superior to that with 585 nm in treating patients with cutaneous hyper-vascular malformation. Complete coagulations are predicted in large-sized and deeply extending blood vessels by 1064 nm laser. - Highlights: •A three-temperature model is proposed for the laser treatment of port wine stain (PWS). •Average sized and large malformed blood vessels in porous medium (tissue) are considered. •Thermal responses of PWS to

The prospect of modern thermomechanics in structural integrity calculations of large-scale pressure vessels

Science.gov (United States)

Fekete, Tamás

2018-05-01

Structural integrity calculations play a crucial role in designing large-scale pressure vessels. Used in the electric power generation industry, these kinds of vessels undergo extensive safety analyses and certification procedures before deemed feasible for future long-term operation. The calculations are nowadays directed and supported by international standards and guides based on state-of-the-art results of applied research and technical development. However, their ability to predict a vessel's behavior under accidental circumstances after long-term operation is largely limited by the strong dependence of the analysis methodology on empirical models that are correlated to the behavior of structural materials and their changes during material aging. Recently a new scientific engineering paradigm, structural integrity has been developing that is essentially a synergistic collaboration between a number of scientific and engineering disciplines, modeling, experiments and numerics. Although the application of the structural integrity paradigm highly contributed to improving the accuracy of safety evaluations of large-scale pressure vessels, the predictive power of the analysis methodology has not yet improved significantly. This is due to the fact that already existing structural integrity calculation methodologies are based on the widespread and commonly accepted 'traditional' engineering thermal stress approach, which is essentially based on the weakly coupled model of thermomechanics and fracture mechanics. Recently, a research has been initiated in MTA EK with the aim to review and evaluate current methodologies and models applied in structural integrity calculations, including their scope of validity. The research intends to come to a better understanding of the physical problems that are inherently present in the pool of structural integrity problems of reactor pressure vessels, and to ultimately find a theoretical framework that could serve as a well

Effect of variable heat transfer coefficient on tissue temperature next to a large vessel during radiofrequency tumor ablation

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Pinheiro Cleber

2008-07-01

Full Text Available Abstract Background One of the current shortcomings of radiofrequency (RF tumor ablation is its limited performance in regions close to large blood vessels, resulting in high recurrence rates at these locations. Computer models have been used to determine tissue temperatures during tumor ablation procedures. To simulate large vessels, either constant wall temperature or constant convective heat transfer coefficient (h have been assumed at the vessel surface to simulate convection. However, the actual distribution of the temperature on the vessel wall is non-uniform and time-varying, and this feature makes the convective coefficient variable. Methods This paper presents a realistic time-varying model in which h is a function of the temperature distribution at the vessel wall. The finite-element method (FEM was employed in order to model RF hepatic ablation. Two geometrical configurations were investigated. The RF electrode was placed at distances of 1 and 5 mm from a large vessel (10 mm diameter. Results When the ablation procedure takes longer than 1–2 min, the attained coagulation zone obtained with both time-varying h and constant h does not differ significantly. However, for short duration ablation (5–10 s and when the electrode is 1 mm away from the vessel, the use of constant h can lead to errors as high as 20% in the estimation of the coagulation zone. Conclusion For tumor ablation procedures typically lasting at least 5 min, this study shows that modeling the heat sink effect of large vessels by applying constant h as a boundary condition will yield precise results while reducing computational complexity. However, for other thermal therapies with shorter treatment using a time-varying h may be necessary.

Etiology of cutaneous vasculitis: utility of a systemic approach

Science.gov (United States)

Chanussot-Deprez, Caroline; Vega-Memije, María Elisa; Flores-Suárez, Luis; Ríos-Romero, Celia; Cabiedes-Contreras, Javier; Reyes, Edgardo; Rangel-Gamboa, Lucia

2018-01-01

Cutaneous vasculities (CV) represents a diagnostic challenge, occurs as primary cutaneous disorder or as a manifestation of other entities. To search the cause of CV. Methods: Patients with CV were prospectively evaluated. In all patients, skin biopsies were drawn, and direct immunofluorescence was done in most of the patients. American College of Rheumatology (ACR) and Chapel Hill Consensus Conference Criteria (CHCC) were used for classification. 32 patients were studied. There was female predominance (71.8%). Children presented drug-associated CV or Schönlein-Henoch púrpura (SHP). Adults presented more frequently SHP, systemic lupus erythematosus or paraneoplastic vasculitis, other diagnosis as polyarteritis nodosa, microscopic polyangiitis, thrombotic vasculitis (post-puerperal), antiphospholipid syndrome, Churg-Strauss syndrome, and drug-associated CV were presented. Using the ACR and CHCC criteria, 50% of cases were classified. In our institution, during this work the etiologic diagnostic of CV increased more than twice. However, in the case of HSV or LA and SHP none of the proposed criteria had high specificity; other parameters were used to discern between both. Six patients remained as not classified. In our view, cryoglobulins and hepatitis serology do not seem useful unless patient’s history supports they need to be done. Unclassified patients were followed-up closely for 2 years. Copyright: © 2018 SecretarÍa de Salud

Bioinspired engineering study of Plantae vascules for self-healing composite structures

Science.gov (United States)

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

2010-01-01

This paper presents the first conceptual study into creating a Plantae-inspired vascular network within a fibre-reinforced polymer composite laminate, which provides an ongoing self-healing functionality without incurring a mass penalty. Through the application of a ‘lost-wax’ technique, orthogonal hollow vascules, inspired by the ‘ray cell’ structures found in ring porous hardwoods, were successfully introduced within a carbon fibre-reinforced epoxy polymer composite laminate. The influence on fibre architecture and mechanical behaviour of single vascules (located on the laminate centreline) when aligned parallel and transverse to the local host ply was characterized experimentally using a compression-after-impact test methodology. Ultrasonic C-scanning and high-resolution micro-CT X-ray was undertaken to identify the influence of and interaction between the internal vasculature and impact damage. The results clearly show that damage morphology is influenced by vascule orientation and that a 10 J low-velocity impact damage event is sufficient to breach the vasculature; a prerequisite for any subsequent self-healing function. The residual compressive strength after a 10 J impact was found to be dependent upon vascule orientation. In general, residual compressive strength decreased to 70 per cent of undamaged strength when vasculature was aligned parallel to the local host ply and a value of 63 per cent when aligned transverse. This bioinspired engineering study has illustrated the potential that a vasculature concept has to offer in terms of providing a self-healing function with minimum mass penalty, without initiating premature failure within a composite structure. PMID:19955122

Evaluation of creep-fatigue crack growth for large-scale FBR reactor vessel and NDE assessment

Energy Technology Data Exchange (ETDEWEB)

Joo, Young Sang; Kim, Jong Bum; Kim, Seok Hun; Yoo, Bong

2001-03-01

Creep fatigue crack growth contributes to the failure of FRB reactor vessels in high temperature condition. In the design stage of reactor vessel, crack growth evaluation is very important to ensure the structural safety and setup the in-service inspection strategy. In this study, creep-fatigue crack growth evaluation has been performed for the semi-elliptical surface cracks subjected to thermal loading. The thermal stress analysis of a large-scale FBR reactor vessel has been carried out for the load conditions. The distributions of axial, radial, hoop, and Von Mises stresses were obtained for the loading conditions. At the maximum point of the axial and hoop stress, the longitudinal and circumferential surface cracks (i.e. PTS crack, NDE short crack and shallow long crack) were postulated. Using the maximum and minimum values of stresses, the creep-fatigue crack growth of the proposed cracks was simulated. The crack growth rate of circumferential cracks becomes greater than that of longitudinal cracks. The total crack growth of the largest PTS crack is very small after 427 cycles. The structural integrity of a large-scale reactor can be maintained for the plant life. The crack depth growth of the shallow long crack is faster than that of the NDE short crack. In the ISI of the large-scale FBR reactor vessel, the ultrasonic inspection is beneficial to detect the shallow circumferential cracks.

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.

Noninvasive diagnosis of thrombus in the heart and large vessels

International Nuclear Information System (INIS)

Masuda, Yoshiaki; Morooka, Nobuhiro; Yoshida, Hideo; Watanabe, Shigeru; Inagaki, Yoshiaki

1984-01-01

The usefulness of two dimensional echocardiography (2-D echocardiography) and x-ray computed tomography (CT) for the diagnosis of thrombi in the cardiac cavity and large vessels was studied by comparing them with the findings of invasive methods. Among 56 subjects with mitral stenosis, left atrial thrombi were noted in 12 cases (16 regions) by CT and 8 cases (9 regions) by 2-D echocardiography. In 16 subjects who underwent operations, one false negative case by CT and 3 false negative and one false positive cases by 2-D echocardiography were found. In 80 subjects with myocardial infarction 2-D echocardiography, CT and left ventriculography (LVG) were performed at approximately the same time. Thrombi were detected in 10 subjects (12.5%) by 2-D echocardiography, in 15 (18.8%) by CT and in 14 (17.5%) by LVG. Although mural thrombi in abdominal aortic aneurysm were detected very easily, thin thrombi surrounding the false lumen of the dissecting aneurysm were not detected ultrasonographically. These thrombi were only detected by the enhanced CT. Our results show the usefulness of both methods for detecting thrombi in the heart and large vessels. CT can distinguish the thrombi more clearly than 2-D echocardiography, but 2-D echocardiography is performed more easily, safely and economically than CT. (author)

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

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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.

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.

Postoperative spinal infection mimicking systemic vasculitis with titanium-spinal implants

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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.

Anti-neutrophil cytoplasmic antibody-associated vasculitis associated with infectious mononucleosis due to primary Epstein-Barr virus infection: report of three cases.

Science.gov (United States)

Yamaguchi, Makoto; Yoshioka, Tomoki; Yamakawa, Taishi; Maeda, Matsuyoshi; Shimizu, Hideaki; Fujita, Yoshiro; Maruyama, Shoichi; Ito, Yasuhiko; Matsuo, Seiichi

2014-02-01

Although the aetiology of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis remains unclear, it is generally believed that environmental factors such as infections contribute to its development of ANCA-associated vasculitis. Prior Epstein-Barr virus (EBV) infection is reported to be a trigger of systemic vasculitis. We herein report three cases of ANCA-associated vasculitis presenting with infectious mononucleosis due to primary EBV infection. The causal link between the two pathologies could not be proved, but primary EBV infection may play a role in the initiation or exacerbation of ANCA-associated vasculitis. Future studies are necessary to determine the interaction between these diseases conditions.

Warfarin-induced leukocytoclastic vasculitis and proteinuria

Directory of Open Access Journals (Sweden)

Khalid Jumean

2016-01-01

Full Text Available Warfarin is typically prescribed for patients with thromboembolic diseases and atrial fibrillation. In addition to the complications of bleeding, allergic skin reaction is one of its rare adverse effects. We herein report a case of a 79 year old male patient with leukocytoclastic vasculitis and proteinuria secondary to warfarin. The warfarin was discontinued and oral prednisone therapy was initiated. The cutaneous lesions and the proteinuria resolved thereafter.

Value of CT angiography in anterior circulation large vessel occlusive stroke: Imaging findings, pearls, and pitfalls

Energy Technology Data Exchange (ETDEWEB)

Power, Sarah, E-mail: drsarahpower@gmail.com [Department of Neuroradiology, Beaumont Hospital, P.O. Box 1297, Beaumont Rd, Dublin 9 (Ireland); McEvoy, Sinead H., E-mail: sineadmcevoy@beaumont.ie [Department of Neuroradiology, Beaumont Hospital, P.O. Box 1297, Beaumont Rd, Dublin 9 (Ireland); Cunningham, Jane, E-mail: janecunningham0708@gmail.com [Department of Radiology, Beaumont Hospital, P.O. Box 1297, Beaumont Rd, Dublin 9 (Ireland); Ti, Joanna P., E-mail: joannapearlyti@gmail.com [Department of Neuroradiology, Beaumont Hospital, P.O. Box 1297, Beaumont Rd, Dublin 9 (Ireland); Looby, Seamus, E-mail: seamuslooby@beaumont.ie [Department of Neuroradiology, Beaumont Hospital, P.O. Box 1297, Beaumont Rd, Dublin 9 (Ireland); O' Hare, Alan, E-mail: alanohare@beaumont.ie [Department of Neuroradiology, Beaumont Hospital, P.O. Box 1297, Beaumont Rd, Dublin 9 (Ireland); Williams, David, E-mail: davidwilliams@rcsi.ie [Department of Geriatrics and Stroke Medicine, Royal College of Surgeons in Ireland (RCSI) and Beaumont Hospital, P.O. Box 1297, Beaumont Rd, Dublin 9 (Ireland); Brennan, Paul, E-mail: paulbrennan@beaumont.ie [Department of Neuroradiology, Beaumont Hospital, P.O. Box 1297, Beaumont Rd, Dublin 9 (Ireland); Thornton, John, E-mail: johnthornton@beaumont.ie [Department of Neuroradiology, Beaumont Hospital, P.O. Box 1297, Beaumont Rd, Dublin 9 (Ireland)

2015-07-15

Highlights: • Site of occlusion determines potential collateralization routes and impacts outcome. • Multifocality of arterial occlusion is common, seen in approximately 20% of cases. • ICA false occlusion sign can be seen in setting of ICA stenosis or carotid T occlusion. • False patency sign: hyperdense thrombus/calcified occlusive plaque misinterpreted as patent vessel. • Additional abnormalities on CTA may infer stroke mechanism or alter decision making. - Abstract: Hyperacute stroke imaging is playing an increasingly important role in determining management decisions in acute stroke patients, particularly patients with large vessel occlusive stroke who may benefit from endovascular intervention. CT angiography (CTA) is an important tool in the work-up of the acute stroke patient. It reliably detects large occlusive thrombi in proximal cerebral arteries and is a quick and highly accurate method in identifying candidates for endovascular stroke treatment. In this article we review the imaging findings on CTA in acute large vessel occlusive stroke using a pictorial case based approach. We retrospectively reviewed CTA studies in 48 patients presenting with acute anterior circulation large vessel occlusive stroke who were brought for intra-arterial acute stroke intervention. We discuss and illustrate patterns of proximal intracranial arterial occlusion, collateralization to the occluded territory, as well as reviewing some important pearls, pitfalls and teaching points in CTA assessment of the acute stroke patient. Performed from the level of the aortic arch CTA also gives valuable information regarding the state of other vessels in the acute stroke patient, identifying additional significant vascular stenoses or occlusions, and as we illustrate, can demonstrate other clinically significant findings which may impact on patient management and outcome.

Value of CT angiography in anterior circulation large vessel occlusive stroke: Imaging findings, pearls, and pitfalls

International Nuclear Information System (INIS)

Power, Sarah; McEvoy, Sinead H.; Cunningham, Jane; Ti, Joanna P.; Looby, Seamus; O'Hare, Alan; Williams, David; Brennan, Paul; Thornton, John

2015-01-01

Highlights: • Site of occlusion determines potential collateralization routes and impacts outcome. • Multifocality of arterial occlusion is common, seen in approximately 20% of cases. • ICA false occlusion sign can be seen in setting of ICA stenosis or carotid T occlusion. • False patency sign: hyperdense thrombus/calcified occlusive plaque misinterpreted as patent vessel. • Additional abnormalities on CTA may infer stroke mechanism or alter decision making. - Abstract: Hyperacute stroke imaging is playing an increasingly important role in determining management decisions in acute stroke patients, particularly patients with large vessel occlusive stroke who may benefit from endovascular intervention. CT angiography (CTA) is an important tool in the work-up of the acute stroke patient. It reliably detects large occlusive thrombi in proximal cerebral arteries and is a quick and highly accurate method in identifying candidates for endovascular stroke treatment. In this article we review the imaging findings on CTA in acute large vessel occlusive stroke using a pictorial case based approach. We retrospectively reviewed CTA studies in 48 patients presenting with acute anterior circulation large vessel occlusive stroke who were brought for intra-arterial acute stroke intervention. We discuss and illustrate patterns of proximal intracranial arterial occlusion, collateralization to the occluded territory, as well as reviewing some important pearls, pitfalls and teaching points in CTA assessment of the acute stroke patient. Performed from the level of the aortic arch CTA also gives valuable information regarding the state of other vessels in the acute stroke patient, identifying additional significant vascular stenoses or occlusions, and as we illustrate, can demonstrate other clinically significant findings which may impact on patient management and outcome

Multifocal Choroiditis with Retinal Vasculitis, Optic Neuropathy, and Keratoconus in a Young Saudi Male.

Science.gov (United States)

Dhafiri, Yousef; Al Rubaie, Khalid; Kirat, Omar; May, William N; Nguyen, Quan D; Kozak, Igor

2017-01-01

The purpose of this study is to describe an association of unilateral multifocal choroiditis (MFC), retinal vasculitis, optic neuropathy, and bilateral keratoconus in a young Saudi male. A 27-year-old male patient with stable bilateral keratoconus presented with a painless vision loss in his left eye. Ophthalmic examinations revealed multiple foci of idiopathic chorioretinitis, retinal vasculitis, and mild optic disc leakage on fluorescein angiography, all of which resolved on systemic therapy with mycophenolate mofetil and prednisone after 3 months. Systemic medication was stopped after 8 months. One year after presentation, patient's visual acuity has improved and remained stable. Systemic immunomodulatory therapy can be effective in managing and leading to resolution of MFC, retinal vasculitis, and optic disc leak in young patients.

Ductile fracture of cylindrical vessels containing a large flaw

Science.gov (United States)

Erdogan, F.; Irwin, G. R.; Ratwani, M.

1976-01-01

The fracture process in pressurized cylindrical vessels containing a relatively large flaw is considered. The flaw is assumed to be a part-through or through meridional crack. The flaw geometry, the yield behavior of the material, and the internal pressure are assumed to be such that in the neighborhood of the flaw the cylinder wall undergoes large-scale plastic deformations. Thus, the problem falls outside the range of applicability of conventional brittle fracture theories. To study the problem, plasticity considerations are introduced into the shell theory through the assumptions of fully-yielded net ligaments using a plastic strip model. Then a ductile fracture criterion is developed which is based on the concept of net ligament plastic instability. A limited verification is attempted by comparing the theoretical predictions with some existing experimental results.

Leukocytoclastic Vasculitis in a Patient with Ankylosing Spondylitis

Directory of Open Access Journals (Sweden)

Senol Kobak

2014-01-01

Full Text Available A 26-year-old male patient presented to our rheumatology clinic with pain, swelling and limitation of movement in his right ankle, and also purpuric skin lesions in the lower extremity pretibial region. He was asked questions, and he said that he had been having chronic low back pain and morning stiffness for the last few years. His physical examination revealed that he had arthritis in his right ankle, purpuric skin lesions in pretibial regions of both legs, and bilateral FABERE/FADIR positivity. The sacroiliac joint imaging and MRI revealed bilateral sacroiliitis findings, and the lateral heel imaging revealed enthesitis. HLA-B27 was positive. Skin biopsy from lower skin lesions was reported to be consistent with leukocytoclastic vasculitis. Based on clinical, laboratory, radiological, and pathological examinations, the patient was diagnosed with ankylosing spondylitis and leukocytoclastic vasculitis. Administration of corticosteroid, salazopyrin, and nonsteroid anti-inflammatory medications was started. Notable clinical and laboratory regression was observed during his checks 3 months later.

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

Ultrasonographic evidence of colonic mesenteric vessels as an indicator of right dorsal displacement of the large colon in 13 horses.

Science.gov (United States)

Grenager, N S; Durham, M G

2011-08-01

This report describes the use of ultrasound to diagnose right dorsal displacement of the large colon (RDDLC) in 13 horses prior to surgery. Horses had ultrasonographic examinations performed of the right lateroventral aspect of the abdomen upon admission to the hospital with a 2-5 MHz broadband curvilinear sector scanning transducer after alcohol was used to wet the hair. First, the caecal vessels were identified in the right flank and followed medially and cranially. Next, each intercostal space, from caudal to cranial, was scanned from dorsal to ventral evaluating for abnormally-located mesenteric vessels associated with the large colon. Abnormally-located mesenteric vessels associated with the large colon, distinct from the caecal vessels, were identified in 13 of 23 horses with a diagnosis of RDDLC obtained at exploratory laparotomy. In horses, ultrasonographic visualisation of mesenteric vessels along the right lateral abdomen, dorsal to the costochondral junction in at least 2 intercostal spaces, distinct from the caecal vessels, is consistent with a surgical diagnosis of RDDLC. © 2011 EVJ Ltd.

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)

Randomized Trial of C5a Receptor Inhibitor Avacopan in ANCA-Associated Vasculitis.

Science.gov (United States)

Jayne, David R W; Bruchfeld, Annette N; Harper, Lorraine; Schaier, Matthias; Venning, Michael C; Hamilton, Patrick; Burst, Volker; Grundmann, Franziska; Jadoul, Michel; Szombati, István; Tesař, Vladimír; Segelmark, Mårten; Potarca, Antonia; Schall, Thomas J; Bekker, Pirow

2017-09-01

Alternative C activation is involved in the pathogenesis of ANCA-associated vasculitis. However, glucocorticoids used as treatment contribute to the morbidity and mortality of vasculitis. We determined whether avacopan (CCX168), an orally administered, selective C5a receptor inhibitor, could replace oral glucocorticoids without compromising efficacy. In this randomized, placebo-controlled trial, adults with newly diagnosed or relapsing vasculitis received placebo plus prednisone starting at 60 mg daily (control group), avacopan (30 mg, twice daily) plus reduced-dose prednisone (20 mg daily), or avacopan (30 mg, twice daily) without prednisone. All patients received cyclophosphamide or rituximab. The primary efficacy measure was the proportion of patients achieving a ≥50% reduction in Birmingham Vasculitis Activity Score by week 12 and no worsening in any body system. We enrolled 67 patients, 23 in the control and 22 in each of the avacopan groups. Clinical response at week 12 was achieved in 14 of 20 (70.0%) control patients, 19 of 22 (86.4%) patients in the avacopan plus reduced-dose prednisone group (difference from control 16.4%; two-sided 90% confidence limit, -4.3% to 37.1%; P =0.002 for noninferiority), and 17 of 21 (81.0%) patients in the avacopan without prednisone group (difference from control 11.0%; two-sided 90% confidence limit, -11.0% to 32.9%; P =0.01 for noninferiority). Adverse events occurred in 21 of 23 (91%) control patients, 19 of 22 (86%) patients in the avacopan plus reduced-dose prednisone group, and 21 of 22 (96%) patients in the avacopan without prednisone group. In conclusion, C5a receptor inhibition with avacopan was effective in replacing high-dose glucocorticoids in treating vasculitis. Copyright © 2017 by the American Society of Nephrology.

Clinical features and outcomes of ANCA-associated renal vasculitis

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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.

Proinflammatory genotype of interleukin-1 and interleukin-1 receptor antagonist is associated with ESRD in proteinase 3-ANCA vasculitis patients.

Science.gov (United States)

Borgmann, Stefan; Endisch, Georg; Hacker, Ulrich T; Song, Bong-Seok; Fricke, Harald

2003-05-01

Small-vessel vasculitides are associated with antineutrophil cytoplasmic antibodies (ANCAs). Cytoplasmic ANCAs are targeted mainly against proteinase 3 (PR3), whereas myeloperoxidase (MPO) is the major antigen of perinuclear ANCAs. These relapsing vasculitides show heterogeneous clinical pictures, and disease severity may vary broadly from mild local organ manifestation to acute organ failure (eg, renal failure). We tested whether two cytokine polymorphisms in the interleukin-1beta (IL-1beta) and IL-1 receptor antagonist (IL-1ra) genes, known to determine cytokine secretion, are associated with clinical manifestations and outcome of ANCA-associated vasculitides. Polymerase chain reaction and restriction fragment length polymorphism analyses were performed to determine polymorphisms in the IL-1beta and IL-1ra genes in 79 patients with PR3-ANCA, 30 patients with MPO-ANCA vasculitis, and 196 healthy controls. The frequency of the so-called proinflammatory genotype, characterized by high secretion of IL-1beta and low secretion of its antagonist IL-1ra, was increased significantly in patients with PR3-ANCA with end-stage renal disease. Patients with a renal manifestation of PR3-ANCA vasculitis have an increased risk for developing end-stage renal disease when carrying the proinflammatory IL-1beta/IL-1ra genotype. Anti-inflammatory therapy specifically antagonizing the proinflammatory effect of IL-1beta may be a promising treatment for patients with Wegener's granulomatosis with renal manifestations.

Vasculitis and antineutrophil cytoplasmic autoantibodies associated with propylthiouracil therapy

NARCIS (Netherlands)

Dolman, K. M.; Gans, R. O.; Vervaat, T. J.; Zevenbergen, G.; Maingay, D.; Nikkels, R. E.; Donker, A. J.; von dem Borne, A. E.; Goldschmeding, R.

1993-01-01

Vasculitis is a rare complication of propylthiouracil therapy. Antineutrophil cytoplasmic antibodies (ANCA) have been described in association with several vasculitic disorders. We report detection of ANCA against human neutrophil elastase, proteinase 3, and myeloperoxidase in serum from six

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.

Propylthiouracil induced leukocytoclastic vasculitis: A rare manifestation

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Semra Ayturk

2013-01-01

Full Text Available Propylthiouracil (PTU is a common drug used in patients with hyperthyroidism. It may cause perinuclearantineutrophil cytoplasmic antibodies (p-ANCA in few patients with Graves′ disease. This antibody has been associated with different forms of vasculitis. We report a patient who presented with cutaneous manifestations of leukocytoclasticvasculitis with simultaneous development of p-ANCAs during PTU therapy for Graves′ disease.

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.

Large-scale testing of in-vessel debris cooling through external flooding of the reactor pressure vessel in the CYBL facility

International Nuclear Information System (INIS)

Chu, T.Y.; Bentz, J.H.; Bergeron, K.D.; Slezak, S.E.; Simpson, R.B.

1994-01-01

The possibility of achieving in-vessel core retention by flooding the reactor cavity, or the ''flooded cavity'', is an accident management concept currently under consideration for advanced light water reactors (ALWR), as well as for existing light water reactors (LWR). The CYBL (CYlindrical BoiLing) facility is a facility specifically designed to perform large-scale confirmatory testing of the flooded cavity concept. CYBL has a tank-within-a-tank design; the inner 3.7 m diameter tank simulates the reactor vessel, and the outer tank simulates the reactor cavity. The energy deposition on the bottom head is simulated with an array of radiant heaters. The array can deliver a tailored heat flux distribution corresponding to that resulting from core melt convection. The present paper provides a detailed description of the capabilities of the facility, as well as results of recent experiments with heat flux in the range of interest to those required for in-vessel retention in typical ALWRs. The paper concludes with a discussion of other experiments for the flooded cavity applications

Large-Scale testing of in-vessel debris cooling through external flooding of the reactor pressure vessel in the CYBL facility

International Nuclear Information System (INIS)

Chu, T.Y.; Bentz, J.H.; Bergeron, K.D.; Slezak, S.E.; Simpson, R.B.

1994-01-01

The possibility of achieving in-vessel core retention by flooding the reactor cavity, or the open-quotes flooded cavityclose quotes, is an accident management concept currently under consideration for advanced light water reactors (ALWR), as well as for existing light water reactors (LWR). The CYBL (CYlindrical BoiLing) facility is a facility specifically designed to perform large-scale confirmatory testing of the flooded cavity concept. CYBL has a tank-within-a-tank design; the inner 3.7 m diameter tank simulates the reactor vessel, and the outer tank simulates the reactor cavity. The energy deposition on the bottom head is simulated with an array of radiant heaters. The array can deliver a tailored heat flux distribution corresponding to that resulting from core melt convection. The present paper provides a detailed description of the capabilities of the facility, as well as results of recent experiments with heat flux in the range of interest to those required for in-vessel retention in typical ALWRs. The paper concludes with a discussion of other experiments for the flooded cavity applications

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

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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.

Mock-up test of remote controlled dismantling apparatus for large-sized vessels (contract research)

Energy Technology Data Exchange (ETDEWEB)

Myodo, Masato; Miyajima, Kazutoshi [Japan Atomic Energy Research Inst., Tokai, Ibaraki (Japan). Tokai Research Establishment; Okane, Shogo [Japan Atomic Energy Research Inst., Oarai, Ibaraki (Japan). Oarai Research Establishment

2001-03-01

The Remote dismantling apparatus, which is equipped with multi-units for functioning of washing, cutting, collection of cut pieces and so on, has been constructed to dismantle the large-sized vessels in the JAERI's Reprocessing Test Facility (JRTF). The apparatus has five-axis movement capability and its operation is performed remotely. The mock-up tests were performed to evaluate the applicability of the apparatus to actual dismantling activities by using the mock-ups of LV-3 and LV-5 in the facility. It was confirmed that each unit was satisfactory functioned by remote operation. Efficient procedures for dismantling the large-sized vessel was studied and various date was obtained in the mock-up tests. This apparatus was found to be applicable for the actual dismantling activity in JRTF. (author)

Mock-up test of remote controlled dismantling apparatus for large-sized vessels (contract research)

International Nuclear Information System (INIS)

Myodo, Masato; Miyajima, Kazutoshi; Okane, Shogo

2001-03-01

The Remote dismantling apparatus, which is equipped with multi-units for functioning of washing, cutting, collection of cut pieces and so on, has been constructed to dismantle the large-sized vessels in the JAERI's Reprocessing Test Facility (JRTF). The apparatus has five-axis movement capability and its operation is performed remotely. The mock-up tests were performed to evaluate the applicability of the apparatus to actual dismantling activities by using the mock-ups of LV-3 and LV-5 in the facility. It was confirmed that each unit was satisfactory functioned by remote operation. Efficient procedures for dismantling the large-sized vessel was studied and various date was obtained in the mock-up tests. This apparatus was found to be applicable for the actual dismantling activity in JRTF. (author)

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...

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.)

Process for producing curved surface of membrane rings for large containers, particulary for prestressed concrete pressure vessels of nuclear reactors

International Nuclear Information System (INIS)

Kumpf, H.

1977-01-01

Membrane rings for large pressure vessels, particularly for prestressed-concrete pressure vessels, often have curved surfaces. The invention describes a process of producing these at site, which is particularly advantageous as the forming and installation of the vessel component coincide. According to the invention, the originally flat membrane ring is set in a predetermined position, is then pressed in sections by a forming tool (with a preformed support ring as opposite tool), and shaped. After this, the shaped parts are welded to the ring-shaped wall parts of the large vessel. The manufacture of single and double membrane rings arrangements is described. (HP) [de

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.

Progressive outer retinal necrosis associated with occlusive vasculitis in acquired immunodeficiency syndrome

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Chien-Chi Tseng

2015-05-01

Full Text Available A 45-year-old man, a case of acquired immunodeficiency syndrome, received a highly active antiretroviral therapy at the outpatient service for 4 years without regular follow-up. He experienced progressively blurred vision for 6 months and a cutaneous zoster on his back 3 months ago. He was diagnosed with progressive outer retinal necrosis by polymerase chain reaction-restriction fragment length polymorphism using an aqueous humor sample, which revealed an existence of varicella zoster virus. He was given a combination of systemic, intravitreal antiviral and a highly active antiretroviral therapy. Occlusive vasculitis, an unusual finding for progressive outer retinal necrosis, developed in both eyes 1 week after the secondary intravitreal injection. Unfortunately, his vision deteriorated to no light perception in both eyes within 2 weeks. Progressive outer retinal necrosis is characterized clinically as showing minimal or no inflammation in the aqueous and vitreous humors, absence of retinal vasculitis, and patches of yellowish spots located deep in the retina. Physicians should pay attention to this rare case of progressive outer retinal necrosis associated occlusive vasculitis with very poor prognosis in spite of aggressive treatment.

Large Pelagic Logbook Set Survey (Vessels)

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set contains catch and effort for fishing trips that are taken by vessels with a Federal permit issued for the swordfish and sharks under the Highly...

Large Pelagic Logbook Trip Survey (Vessels)

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — This data set contains catch and effort for fishing trips that are taken by vessels with a Federal permit issued for the swordfish and sharks under the Highly...

Prediction of Large Vessel Occlusions in Acute Stroke: National Institute of Health Stroke Scale Is Hard to Beat.

Science.gov (United States)

Vanacker, Peter; Heldner, Mirjam R; Amiguet, Michael; Faouzi, Mohamed; Cras, Patrick; Ntaios, George; Arnold, Marcel; Mattle, Heinrich P; Gralla, Jan; Fischer, Urs; Michel, Patrik

2016-06-01

Endovascular treatment for acute ischemic stroke with a large vessel occlusion was recently shown to be effective. We aimed to develop a score capable of predicting large vessel occlusion eligible for endovascular treatment in the early hospital management. Retrospective, cohort study. Two tertiary, Swiss stroke centers. Consecutive acute ischemic stroke patients (1,645 patients; Acute STroke Registry and Analysis of Lausanne registry), who had CT angiography within 6 and 12 hours of symptom onset, were categorized according to the occlusion site. Demographic and clinical information was used in logistic regression analysis to derive predictors of large vessel occlusion (defined as intracranial carotid, basilar, and M1 segment of middle cerebral artery occlusions). Based on logistic regression coefficients, an integer score was created and validated internally and externally (848 patients; Bernese Stroke Registry). None. Large vessel occlusions were present in 316 patients (21%) in the derivation and 566 (28%) in the external validation cohort. Five predictors added significantly to the score: National Institute of Health Stroke Scale at admission, hemineglect, female sex, atrial fibrillation, and no history of stroke and prestroke handicap (modified Rankin Scale score, < 2). Diagnostic accuracy in internal and external validation cohorts was excellent (area under the receiver operating characteristic curve, 0.84 both). The score performed slightly better than National Institute of Health Stroke Scale alone regarding prediction error (Wilcoxon signed rank test, p < 0.001) and regarding discriminatory power in derivation and pooled cohorts (area under the receiver operating characteristic curve, 0.81 vs 0.80; DeLong test, p = 0.02). Our score accurately predicts the presence of emergent large vessel occlusions, which are eligible for endovascular treatment. However, incorporation of additional demographic and historical information available on hospital arrival

[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.

Renal paraneoplastic vasculitis complicating lung adenocarcinoma

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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.

Delayed diagnosis of ocular syphilis that manifested as retinal vasculitis and acute posterior multifocal placoid epitheliopathy

Directory of Open Access Journals (Sweden)

Jong Hoon Park

2013-01-01

Full Text Available A 55-year-old female presented with bilateral progressive retinal vasculitis. She was on systemic and intravitreal steroids on the basis of uveitis work-up result (negative result including rapid plasma reagin, but her visual acuity continued to deteriorate to light perception only. Ocular examination showed retinal vasculitis, multiple yellow placoid lesions and severe macula edema in both eyes. Repeated work-up revealed positivity of fluorescent treponemal antibody-absorption in serum and subsequently in cerebrospinal fluid. Ocular syphilis was diagnosed. And intravenous penicillin G resulted in rapid resolution of vasculitis and macular edema. To avoid delay in the diagnosis of ocular syphilis, high index of suspicion and repeating serological tests (including both treponemal and non-treponemal tests are warranted.

Urticarial vasculitis induced by OTC diet pills: a case report.

Science.gov (United States)

Chérrez Ojeda, Iván; Loayza, Enrique; Greiding, Leonardo; Calderón, Juan Carlos; Cherrez, Annia; Adum, Farid

2015-01-01

Urticarial Vasculitis (UV) is in most of the cases idiopathic; however it has been associated with several conditions and drugs. Over the counter (OTC) diet pills are widely available, even on-line, but they are rarely regulated by pharmaceutical control. We present the case of a 35-year-old female patient suffering of pruriginous and painful wheals more than 1 cm in diameter, with a burning sensation. The eruption lasted more than 24 hours and was accompanied by angioedema, headache and myalgia. No remarkable medical history was found, except for previous intake of OTC diet pills. UV diagnosis was confirmed by the skin biopsy of a lesion. OTC diet pills are widely available worldwide, and due to its widespread use, allergologists and dermatologist should be able to recognize symptoms and lesions of cutaneous vasculitis, which may be under reported.

Intermediate monocytes in ANCA vasculitis: increased surface expression of ANCA autoantigens and IL-1β secretion in response to anti-MPO antibodies.

LENUS (Irish Health Repository)

O'Brien, Eóin C

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 pathogenesis is driven by ANCA-mediated activation of neutrophils and monocytes. We investigated a potential role for distinct monocyte subsets. We found that the relative proportion of intermediate monocytes is increased in patients versus control individuals, and both MPO and PR3 are preferentially expressed on these cells. We demonstrate that MPO and PR3 are expressed independently of each other on monocytes and that PR3 is not associated with CD177. MPO expression correlates with that of Fc receptor CD16 on intermediate monocytes. Monocyte subsets respond differently to antibodies directed against MPO and PR3, with anti-MPO but not anti-PR3 leading to increased IL-1β, IL-6 and IL-8 production. In concordance with the observed higher surface expression of MPO on intermediate monocytes, this subset produces the highest quantity of IL-1β in response to anti-MPO stimulation. These data suggest that monocytes, specifically, the intermediate subset, may play a role in ANCA vasculitis, and also indicate that substantial differences exist between the effect of anti-MPO and anti-PR3 antibodies on these cells.

The Amplatzer vascular plug for large vessel occlusion in the endovascular management of aneurysms

Energy Technology Data Exchange (ETDEWEB)

Ratnam, Lakshmi A.; Walkden, Richard M.; Munneke, Graham J.; Morgan, Robert A.; Belli, Anna-Maria [St.George' s Hospital, Department of Radiology, London (United Kingdom)

2008-09-15

The Amplatzer vascular plug (AVP) is derived from the Amplatzer device used in correction of cardiac septal defects. We present a large series of the use of the AVP in the endovascular management of aneurysms. Three patients with a combination of aortic and peripheral aneurysms underwent embolisation with the AVP. Plugs with 10-16 mm diameter were used and delivered using introducer/guiding sheaths. A total of 16 internal iliac arteries, 2 common iliac arteries (CIA), 4 subclavian (SCA) arteries, 1 superior mesenteric (SMA) and 1 popliteal artery were embolised. Successful occlusion with the AVP was achieved in 21 out of 24 vessels (87.5%), of which 18 (75%) occluded immediately and 3 (12.5%) were delayed occlusions. The three patients who are considered to have failed to occlude with the AVP required the use of additional embolic agents. At the end of the 17-month follow-up all 24 target vessels had occluded. Two patients developed persistent buttock claudication, and one had a left hemiparesis. The AVP is useful as an adjunctive device in the management of aneurysms. It has a particular role in embolisation of large-diameter vessels with a short implantation zone. The device is safe and easy to use. (orig.)

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.

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.

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.

SOSTITUTI BIOLOGICI VASCOLARI A BASE DI ACIDO IALURONICO

OpenAIRE

Pandis, Laura

2014-01-01

The discovery of an adequate tissue substitute for the regeneration of small vessels has become one of the most debated issues in the last years. This is due to recent advances in regenerative medicine, which offers new cellular types and biomaterials, and the large clinical applications that this product may have, ranging from the replacement of the coronary vessels to correction of peripheral vascular malformations in pediatric patients. The biomaterials used so far for large caliber vascul...

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...

Model of large scale man-machine systems with an application to vessel traffic control

NARCIS (Netherlands)

Wewerinke, P.H.; van der Ent, W.I.; ten Hove, D.

1989-01-01

Mathematical models are discussed to deal with complex large-scale man-machine systems such as vessel (air, road) traffic and process control systems. Only interrelationships between subsystems are assumed. Each subsystem is controlled by a corresponding human operator (HO). Because of the

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.

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

exchange did not differ significantly across the range of baseline serum creatinine values (P = 0.7) or number of plasma exchange treatments (P = 0.8). The RR for end-stage renal disease was 0.64 (95% CI, 0.47-0.88; P = 0.006), whereas the RR for death alone was 1.01 (95% CI, 0.71-1.4; P = 0......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...

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

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.

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.

Acquired cutis laxa following urticarial vasculitis associated with IgA myeloma.

Science.gov (United States)

Turner, Ryan B; Haynes, Harley A; Granter, Scott R; Miller, Danielle M

2009-06-01

Cutis laxa (CL) is an inherited or acquired connective tissue disorder characterized clinically by loosely hanging skin folds. There is often preceding cutaneous inflammatory eruption (ie, urticaria, eczema, erythema multiforme), and there is frequently internal organ involvement of the gastrointestinal, urogenital, pulmonary, and cardiovascular systems. Histologically, there are degenerative changes in the dermal elastic fibers. Of the few reports on this rare disorder, authors have speculated about an immune-mediated destruction of elastic fibers, and monoclonal gammopathies, such as multiple myeloma or heavy chain deposition disease, have a recognized association with CL. We report an unusual case of rapidly progressing acquired CL associated with leukocytoclastic vasculitis, IgA myeloma, and an immune complex-mediated glomerulonephritis. Light microscopy of the lax skin revealed complete absence of elastic fibers in areas of vasculitis.

Storkarsvaskulitis påvist ved PET/CT hos patient med initialt formodet cancer

DEFF Research Database (Denmark)

Andersen, Kim Francis; Skøt, Jens; Bülow, Jens

2012-01-01

Large vessel vasculitis, including giant cell arteritis and Takayasu arteritis, is traditionally diagnosed and classified according to the American College of Rheumatology criteria, which do not include findings on imaging modalities. We present a case in which non-invasive imaging with 18F...

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...

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.

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.

Application of electron beam welding to large size pressure vessels made of thick low alloy steel

International Nuclear Information System (INIS)

Kuri, S.; Yamamoto, M.; Aoki, S.; Kimura, M.; Nayama, M.; Takano, G.

1993-01-01

The authors describe the results of studies for application of the electron beam welding to the large size pressure vessels made of thick low alloy steel (ASME A533 Gr.B cl.2 and A533 Gr.A cl.1). Two major problems for applying the EBW, the poor toughness of weld metal and the equipment to weld huge pressure vessels are focused on. For the first problem, the effects of Ni content of weld metal, welding conditions and post weld heat treatment are investigated. For the second problem, an applicability of the local vacuum EBW to a large size pressure vessel made of thick plate is qualified by the construction of a 120 mm thick, 2350 mm outside diameter cylindrical model. The model was electron beam welded using local vacuum chamber and the performance of the weld joint is investigated. Based on these results, the electron beam welding has been applied to the production of a steam generator for a PWR. (author). 3 refs., 10 figs., 4 tabs

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 ...

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.

Autonomous sensor particle for parameter tracking in large vessels

International Nuclear Information System (INIS)

Thiele, Sebastian; Da Silva, Marco Jose; Hampel, Uwe

2010-01-01

A self-powered and neutrally buoyant sensor particle has been developed for the long-term measurement of spatially distributed process parameters in the chemically harsh environments of large vessels. One intended application is the measurement of flow parameters in stirred fermentation biogas reactors. The prototype sensor particle is a robust and neutrally buoyant capsule, which allows free movement with the flow. It contains measurement devices that log the temperature, absolute pressure (immersion depth) and 3D-acceleration data. A careful calibration including an uncertainty analysis has been performed. Furthermore, autonomous operation of the developed prototype was successfully proven in a flow experiment in a stirred reactor model. It showed that the sensor particle is feasible for future application in fermentation reactors and other industrial processes

Ulcerative colitis presenting as leukocytoclastic vasculitis of skin

OpenAIRE

Akbulut, Sabiye; Ozaslan, Ersan; Topal, Firdevs; Albayrak, Levent; Kayhan, Burcak; Efe, Cumali

2008-01-01

A number of cutaneous changes are known to occur in the course of inflammatory bowel diseases (IBD), including pyoderma gangrenosum, erythema nodosum, perianal disease, erythematous eruptions, urticaria, and purpura. However, occurrence of skin manifestations prior to the development of ulcerative colitis is a rare occasion. Here, we report a case of ulcerative colitis associated with leukocytoclastic vasculitis in which the intestinal symptoms became overt 8 mo after the development of skin ...

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.

Unusual infectious mononucleosis complicated by vasculitis

Directory of Open Access Journals (Sweden)

Srimanta Kumar Sahu

2016-01-01

Full Text Available Infectious mononucleosis (IM is a clinical syndrome caused by Epstein-Barr virus (EBV. It manifests as fever, pharyngitis, malaise, adenopathy, and atypical lymphocytosis. Cardiovascular complications are thought to be rare in IM. There are very few case reports of EBV-associated vasculitides, like Kawasaki disease and systemic polyarteritis nodosa, however, involvement of the large caliber arteries like the aorta and its branches have been reported only scarcely. Myocarditis also is rare as an early manifestation of EBV infection. We present here a rare case of IM, presented initially with acute myocarditis and later with large-vessels arteritis.

Ulcerative colitis presenting as leukocytoclastic vasculitis of skin.

Science.gov (United States)

Akbulut, Sabiye; Ozaslan, Ersan; Topal, Firdevs; Albayrak, Levent; Kayhan, Burcak; Efe, Cumali

2008-04-21

A number of cutaneous changes are known to occur in the course of inflammatory bowel diseases (IBD), including pyoderma gangrenosum, erythema nodosum, perianal disease, erythematous eruptions, urticaria, and purpura. However, occurrence of skin manifestations prior to the development of ulcerative colitis is a rare occasion. Here, we report a case of ulcerative colitis associated with leukocytoclastic vasculitis in which the intestinal symptoms became overt 8 mo after the development of skin lesions.

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

Splenic vasculitis, thrombosis, and infarction in a febrile dog infected with Bartonella henselae.

Science.gov (United States)

Friedenberg, Steven G; Balakrishnan, Nandhakumar; Guillaumin, Julien; Cooper, Edward S; Lewis, Kristin; Russell, Duncan S; Breitschwerdt, Edward B

2015-01-01

To describe the clinical course and successful management of a febrile dog with polyarthritis, splenic vasculitis, thrombosis, and infarction that was infected with Bartonella henselae. An 8-year-old female spayed Labrador Retriever was referred to The Ohio State University Veterinary Medical Center Emergency Service for evaluation of limping, fever, vomiting, and malaise of 4 days' duration. Physical examination abnormalities included generalized weakness, diminished conscious proprioception, bilateral temporalis muscle atrophy, and diarrhea. Peripheral lymph nodes were normal, and there were no signs of abdominal organomegaly, joint effusion, or spinal pain. Abdominal ultrasound identified a nonocclusive splenic vein thrombus. Fine-needle aspirates of the spleen revealed pyogranulomatous inflammation, mild reactive lymphoid hyperplasia, and mild extramedullary hematopoiesis. Splenic histopathology found marked, multifocal to coalescing acute coagulation necrosis (splenic infarctions) and fibrinoid necrotizing vasculitis. Bartonella henselae DNA was amplified by polymerase chain reaction and sequenced from the splenic tissue. The dog responded favorably to antimicrobials and was healthy at the time of follow-up evaluation. Bartonella henselae is an incompletely characterized emerging canine pathogen. This case report establishes a potential role for this bacterium as a cause of vasculitis and thromboembolism, which have not been previously reported in association with B. henselae infection in dogs. © Veterinary Emergency and Critical Care Society 2015.

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.

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

ANCA-associated vasculitis and malignancy

DEFF Research Database (Denmark)

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

2013-01-01

of individual therapeutic agents is difficult to dissect, but cyclophosphamide has emerged as a major contributor to cancer development because of its direct carcinogenic properties. Awareness of cancer risk in AAV calls for increased implementation of measures to prevent or screen for cancer and development......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 inflammatory rheumatic diseases, AAV diagnosis and therapy are associated with an increased risk of de novo cancer development, likely as a result of impaired immunosurveillance, direct oncogenicity of immunosuppressive agents and perhaps malignant degeneration of tissues undergoing chronic immune stimulation...

Disease: H01465 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available agement of large vessel vasculitis: giant cell arteritis. ... JOURNAL ... Curr Cardiol Rep 16:498 (2014) DOI:10.1007/s11886-014-0498-z ... ...0 D013625 PMID:24893935 (description) ... AUTHORS ... Chatterjee S, Flamm SD, Tan CD, Rodriguez ER ... TITLE ... Clinical diagnosis and man

Calibrating a large slab vessel: A battle of the bulge

International Nuclear Information System (INIS)

Thomas, I.R.

1993-01-01

Slab tanks (critically-safe-by-geometry vessels) were proposed for the storage of concentrated, highly-enriched uranium solution in the design of the Fuel Processing Restoration (FPR) Facility at the Idaho Chemical Processing Plant (ICPP). Currently, measurements of bulk mass in ICPP annular vessels have standard deviations on the order of 0.2%, or less. ICPP personnel felt that their inexperience with the aforementioned expansions would prevent them from attaining comparable precision with slab tanks. To help assess the measurement accuracy of slab vessels, a full-scale mockup of those proposed for the FPR Facility was installed for test calibrations. These calibrations were designed to detect vessel expansion under differing conditions. This paper will compare the base-line, water calibrations with those of the higher-density aluminum nitrate, and any observed deflection will be described using vessel calibration techniques. The calibration using water at an elevated temperature was not performed due to the difficulty of maintaining the elevated temperature. This calibration probably will not be conducted because the construction of the FPR Facility has been halted

Hemorrhagic Ischemic Retinal Vasculitis and Alopecia Areata as a Manifestation of HLA-B27.

Science.gov (United States)

Sharma, Ravi; Randhawa, Sandeep

2018-01-01

A 12-year-old Indian boy presented with acute and severe vision loss in his right eye. He was being treated for scalp alopecia areata and rashes behind the ears and above the brow. The eye examination revealed unilateral hemorrhagic retinal vasculitis. The lab work was normal except for a positive HLA-B27 result. The patient was treated with intravitreal bevacizumab (Avastin; Genentech, South San Francisco, CA) and systemic immunosuppression. The retinal vasculitis improved with treatment, but visual acuity only mildly improved. The alopecia areata also improved with systemic immunosuppression. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:60-63.]. Copyright 2018, SLACK Incorporated.

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....

Usefulness of antineutrophil cytoplasmic autoantibodies in diagnosing and managing systemic vasculitis

NARCIS (Netherlands)

Kallenberg, Cees G. M.

Purpose of reviewAntineutrophil cytoplasmic autoantibodies (ANCAs) are considered important diagnostic tests in the work-up of patients suspected of vasculitis. Here we discuss new developments in the methodology of testing, the pitfalls in using these tests as diagnostic tools, and the value of

Vascular-Rheological Properties of Blood in Hemorrhagic Vasculitis Occurring in Childhood and Adulthood

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V.V. Gerasymenko

2016-11-01

Full Text Available Background. As a result of the immune-inflammatory necrotic changes in the walls of arterioles and capillaries in patients with hemorrhagic vasculitis (HV Henoch — Schönlein endothelial dysfunction of vessels occurs, contributing to violations of blood rheological properties and microcirculation. These processes depend on the age of patients, and in cases of onset of the disease in childhood and adulthood are unknown. Objective: to study vascular and rheological properties of blood serum in HV and to compare the indices with different age of the debut of the pathological process in the groups of patients. Material and methods. The study included 174 patients with HV (83 % men and 17 % women. In 92 patients, the disease made its debut in childhood (on average in 12 years old, and in 82 — in the adult (on average in 25 years old. I, II and III degree of activity of pathological process are set at a ratio of 1 : 2 : 2. Indicators of vascular endothelial function were investigated by immune-enzyme analysis and the adsorption-rheological pro­perties of blood were assessed by computer tensiometry. Results. HV is accompanied by severe disorders of the blood vascular and rheological properties which are involved in the pathogenesis of lesions of skin (endothelin-1, surface tension, joints (only surface activity, kidney (prostacyclin, cyclic guanosine monophosphate and heart (endothelin-1, viscoelastic modulus. At that the integrated indicators of vascular endothelial function, viscoelastic, surface-active and relaxation characteristics of serum depend on the age of the patients in the beginning of the disease, the degree of activity of the pathological process, the clinical form of the disease course, necrotic-ulcerative and polymorphic variants of cutaneous vasculitis, and HV, transforming from juvenile, occurs with lower blood levels of endothelin-1, but with a higher concentration of thromboxane A2, cyclic guanosine monophosphate and

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.)

A wave propagation model of blood flow in large vessels using an approximate velocity profile function

NARCIS (Netherlands)

Bessems, D.; Rutten, M.C.M.; Vosse, van de F.N.

2007-01-01

Lumped-parameter models (zero-dimensional) and wave-propagation models (one-dimensional) for pressure and flow in large vessels, as well as fully three-dimensional fluid–structure interaction models for pressure and velocity, can contribute valuably to answering physiological and patho-physiological

Fibrosis pulmonar asociada a vasculitis con anticuerpos anticitoplasmáticos positivos

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Marcelo Fernández Casares

2012-08-01

Full Text Available Las complicaciones pulmonares más conocidas de las vasculitis con anticuerpos anticitoplasmáticos de los neutrófilos (ANCA positivos (VAA, son la hemorragia alveolar, los granulomas y la estenosis de la vía aérea. En los últimos años han aparecido algunos informes aislados que muestran la asociación con fibrosis pulmonar (FP, sugiriendo que ésta sería otra complicación de las VAA. En este trabajo informamos dos casos con dicha asociación describiendo sus características clínicas, tomográficas e inmunológicas. Dado que en la asociación de FP y VAA notificada en los últimos años, la FP puede ser su primera manifestación, podría ser necesaria la búsqueda de ANCA en pacientes con FP, como causa de la misma y por el posible desarrollo posterior de vasculitis.

Therapeutic plasma exchange in patients with pauciimmune vasculitis: Hospital Universitario San Ignacio experience; Bogotá, Colombia

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Juan P. Córdoba

2014-05-01

Full Text Available Introduction: The vasculitis ANCAS positive, are a group of diseases with different clinical manifestations. Therapeutic plasma exchange has become an excellent tool for the treatment of these patients in specific conditions. Objective: Describe the demographic and clinical characteristics of patients and the final outcomes in patients with positive ANCAS vasculitis that required therapeutic plasma exchange (TPE. Materials and methods: All patients under 18 years old with positive ANCAS vascultiis that required TPE where included during the period of May 2010 and December 2013. Results: 13 patients where treated, with a total of 73 TPE sessions. The average age was 52,3 years (Range 17 to 70. The principal diagnosis for interventions was rapidly progressive glomerulonephritis (RPGN plus alveolar hemorrhage (63%. The average number of sessions per patient was 5,6 (range 1 a 10 with an average of plasma volume exchange per session of 1,26 (range 0.72 a 1,56. Of the 13 patients, 11 (84,6% required renal replacement therapy (RRT during hospitalization. At discharge, 36.3% recover the renal function, 27% continue on RRT and 36,3% died. There was at least one complication in 6,8% of all sessions. Conclusions: TPE is an excellent tool to treat patients with positive ANCAS vasculitis that present with RPGN, high levels of serum creatinine or dialysis need, or alveolar hemorrhage. This is a safe procedure with comparable results according to International literature.Key words: Apheresis, plasmapheresis, therapeutic plasma exchange, vasculitis, ANCA.

A Case Report Describing a Rare Presentation of Simultaneous Occurrence of MPO-ANCA-Associated Vasculitis and Rheumatoid Arthritis

OpenAIRE

Foray, Nathalie; Hudali, Tamer; Papireddy, Muralidhar; Gao, John

2016-01-01

Background. Renal-limited myeloperoxidase vasculitis with simultaneous rheumatoid arthritis is reported as a rare occurrence. Review of literature suggests that most patients had a diagnosis of rheumatoid arthritis for several years prior to presenting with renal failure from myeloperoxidase vasculitis. Case Presentation. A 58-year-old Caucasian male presented to the hospital experiencing malaise, fevers, decreased oral intake, nausea, and vomiting for one week duration. His past medical hist...

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.

Disease: H01698 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available cell arteritis and large vessel vasculitis. ... JOURNAL ... RMD Open 2:e000137 (2016) DOI:10.1136/rmdopen-2015-000137 ...s of C-reactive protein (CRP) Glucocorticoids Tocilizumab [DR:D02596] See also H01465 Large-vessel vasculi...culitis in individuals over the age of 50 in Western countries. It is characterized...nown as temporal arteritis, is a chronic and polygenic immune-mediated disease of unknown etiology. It is the most common form of vas...tis (LVV). ICD-10: M31.6 MeSH: D013700 OMIM: 187360 PMID:27708947 (marker) ... AUTHOR

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.

A Case Report Describing a Rare Presentation of Simultaneous Occurrence of MPO-ANCA-Associated Vasculitis and Rheumatoid Arthritis.

Science.gov (United States)

Foray, Nathalie; Hudali, Tamer; Papireddy, Muralidhar; Gao, John

2016-01-01

Background . Renal-limited myeloperoxidase vasculitis with simultaneous rheumatoid arthritis is reported as a rare occurrence. Review of literature suggests that most patients had a diagnosis of rheumatoid arthritis for several years prior to presenting with renal failure from myeloperoxidase vasculitis. Case Presentation . A 58-year-old Caucasian male presented to the hospital experiencing malaise, fevers, decreased oral intake, nausea, and vomiting for one week duration. His past medical history consisted of newly diagnosed but untreated rheumatoid arthritis, hypertension, and non-insulin-dependent diabetes mellitus. He was found to have acute renal failure, proteinuria, and hypoglycemia. Standard therapy, including intravenous fluids, did not improve his acute renal failure. A vasculitis workup resulted in a positive myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA). Renal biopsy revealed crescentic glomerulonephritis (GN) pauci-immune type, suggestive of MPO-ANCA-associated vasculitis (MPO-AAV). Treatment consisted of prednisone, cyclophosphamide, and seven cycles of plasmapheresis, in addition to hemodialysis for uremia. Upon discharge, he received hemodialysis for another week and continued treatment with cyclophosphamide and prednisone. Conclusion . Patients with longstanding rheumatoid arthritis may develop renal failure due to nonsteroidal anti-inflammatory medication use and AA type amyloidosis; however, necrotizing glomerulonephritis with crescent formation has been rarely reported. This stresses the importance of early recognition and swift initiation of treatment.

A Case Report Describing a Rare Presentation of Simultaneous Occurrence of MPO-ANCA-Associated Vasculitis and Rheumatoid Arthritis

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Nathalie Foray

2016-01-01

Full Text Available Background. Renal-limited myeloperoxidase vasculitis with simultaneous rheumatoid arthritis is reported as a rare occurrence. Review of literature suggests that most patients had a diagnosis of rheumatoid arthritis for several years prior to presenting with renal failure from myeloperoxidase vasculitis. Case Presentation. A 58-year-old Caucasian male presented to the hospital experiencing malaise, fevers, decreased oral intake, nausea, and vomiting for one week duration. His past medical history consisted of newly diagnosed but untreated rheumatoid arthritis, hypertension, and non-insulin-dependent diabetes mellitus. He was found to have acute renal failure, proteinuria, and hypoglycemia. Standard therapy, including intravenous fluids, did not improve his acute renal failure. A vasculitis workup resulted in a positive myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA. Renal biopsy revealed crescentic glomerulonephritis (GN pauci-immune type, suggestive of MPO-ANCA-associated vasculitis (MPO-AAV. Treatment consisted of prednisone, cyclophosphamide, and seven cycles of plasmapheresis, in addition to hemodialysis for uremia. Upon discharge, he received hemodialysis for another week and continued treatment with cyclophosphamide and prednisone. Conclusion. Patients with longstanding rheumatoid arthritis may develop renal failure due to nonsteroidal anti-inflammatory medication use and AA type amyloidosis; however, necrotizing glomerulonephritis with crescent formation has been rarely reported. This stresses the importance of early recognition and swift initiation of treatment.

Pulmonary Vasculitis and a Horseshoe Kidney in Noonan Syndrome

OpenAIRE

Surasak Puvabanditsin; Rosanna Abellar; Adaora Madubuko; Rajeev Mehta; Lauren Walzer

2018-01-01

We report a term male neonate with congenital myeloproliferative disorder, thrombocytopenia, a horseshoe kidney, feeding difficulty secondary to dysphagia/foregut dysmotility, and respiratory failure. Prenatal molecular genetic analysis revealed a fetus carrying c.184T>G (p.Tyr62Asp) pathogenic variant in PTPN11. The infant eventually succumbed to respiratory failure. Bacterial and viral cultures/studies were all no growth/negative. Pulmonary capillaritis and vasculitis were noted at autopsy....

Vasculitis inducida por metimazol: Reporte de caso

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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.

Endovascular treatment for acute ischaemic stroke with large vessel occlusion: the experience of a regional stroke service

International Nuclear Information System (INIS)

McCusker, M.W.; Robinson, S.; Looby, S.; Power, S.; Ti, J.P.; Grech, R.; Galvin, L.; O'Hare, A.; Brennan, P.; O'Kelly, P.; O'Brien, P.; Collins, R.; Dolan, E.; Williams, D.J.; Thornton, J.

2015-01-01

Aim: To report the experience of a regional stroke referral service with endovascular treatment for patients with acute ischaemic stroke (AIS) and large vessel occlusion. Materials and methods: A prospective review was undertaken of 93 consecutive cases receiving endovascular treatment for AIS over a 42-month period (January 2010 to June 2013). The National Institutes of Health Stroke Scale (NIHSS), location of large vessel occlusion, details of endovascular procedure, and degree of reperfusion achieved (Thrombolysis In Cerebral Infarction [TICI] score) were recorded. Mortality and functional outcome (modified Rankin Scale [mRS]) were measured at 90 days. Results: The mean patient age was 62 years (range 26–87 years). The mean NIHSS at presentation was 16 (range 6–29). All patients had confirmed proximal large-artery occlusion on computed tomography (CT) angiography: 87 in the anterior circulation, six in the posterior circulation. Of the 93 patients treated, 64 (69%) received intravenous thrombolysis. Successful reperfusion (TICI grade 2a to 3) was achieved in 80 (86%) cases. There were 13 (14%) cases of failed vessel recanalisation (TICI grade 0). Good functional outcome (mRS ≤2) was achieved in 51 (55%) cases. The 90-day mortality was 20 (22%) cases. Fifty-seven (61%) cases were transferred from outside centres. There was no significant increase in morbidity or mortality for transferred patients. Conclusion: Successful endovascular recanalisation can result in good functional outcomes for patients with AIS and large vessel occlusion. Our interventional neuroradiology service provides endovascular treatment as part of a regional stroke service without increase in morbidity or mortality for patients transferred from outside institutions. - Highlights: • Acute stoke patients may benefit from transfer to a specialist centre for endovascular treatment. • The authors offer endovascular treatment for suitable patients as part of a regional stroke service. �

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)

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...

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

Multiple evanescent white dot syndrome associated with retinal vasculitis

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Takahashi A

2015-09-01

Full Text Available Akihiro Takahashi, Wataru Saito, Yuki Hashimoto, Susumu Ishida Department of Ophthalmology, Hokkaido University Graduate School of Medicine, Sapporo, Japan Purpose: A recent study revealed thickening of the inner retinal layers in acute stage of multiple evanescent white dot syndrome (MEWDS; however, the pathogenesis is still unknown. We report two cases with MEWDS whose funduscopy showed obvious retinal vasculitis. Methods: Case reports. Results: Healthy myopic 16- and 27-year-old women were the cases under study. In both cases, funduscopic examination revealed multiple, faint, small, subretinal white dots at the posterior pole to the midperiphery and macular granularity oculus dexter. Retinal vascular sheathing was also observed at midperiphery. Late-phase fluorescein angiography revealed leakages corresponding to the vascular sheathing. Enhanced depth imaging optical coherence tomography revealed the discontinuity of the ellipsoid zone corresponding to the white dots and increased macular choroidal thickness. One month later, these white dots and retinal sheathing spontaneously resolved in both cases. Three months later, impairments of the outer retinal morphology and the visual acuity were restored. Conclusion: These results suggest that retinal vasculitis possibly plays a role in the pathogenesis of thickened inner retinal layers in acute stage of MEWDS. Keywords: enhanced depth imaging optical coherence tomography, choroidal thickness, inner retinal layer, retinal vascular sheathing

Building foundations for transcatheter intervascular anastomoses: 3D anatomy of the great vessels in large experimental animals

NARCIS (Netherlands)

Sizarov, Aleksander; de Bakker, Bernadette S.; Klein, Karina; Ohlerth, Stefanie

2014-01-01

To provide comprehensive illustrations of anatomy of the relevant vessels in large experimental animals in an interactive format as preparation for developing an effective and safe transcatheter technique of aortopulmonary and bidirectional cavopulmonary intervascular anastomoses. Computed

Disease: H01584 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available H01584 IgA vasculitis; Henoch-Schonlein purpura Henoch-Schonlein purpura (HSP) is ...the most common systemic small vessel vasculitis in childhood with clinical characteristics of non-thrombocy

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...

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

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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.

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

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

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

Outcomes of early carotid stenting and angioplasty in large-vessel anterior circulation strokes treated with mechanical thrombectomy and intravenous thrombolytics.

Science.gov (United States)

Mehta, T; Desai, N; Mehta, K; Parikh, R; Male, S; Hussain, M; Ollenschleger, M; Spiegel, G; Grande, A; Ezzeddine, M; Jagadeesan, B; Tummala, R; McCullough, L

2018-01-01

Introduction Proximal cervical internal carotid artery stenosis greater than 50% merits revascularization to mitigate the risk of stroke recurrence among large-vessel anterior circulation strokes undergoing mechanical thrombectomy. Carotid artery stenting necessitates the use of antiplatelets, and there is a theoretical increased risk of hemorrhagic transformation given that such patients may already have received intravenous thrombolytics and have a significant infarct burden. We investigate the outcomes of large-vessel anterior circulation stroke patients treated with intravenous thrombolytics receiving same-day carotid stenting or selective angioplasty compared to no carotid intervention. Materials and methods The study cohort was obtained from the National (Nationwide) Inpatient Sample database between 2006 and 2014, using International Statistical Classification of Diseases, ninth revision discharge diagnosis and procedure codes. A total of 11,825 patients with large-vessel anterior circulation stroke treated with intravenous thrombolytic and mechanical thrombectomy on the same day were identified. The study population was subdivided into three subgroups: no carotid intervention, same-day carotid angioplasty without carotid stenting, and same-day carotid stenting. Outcomes were assessed with respect to mortality, significant disability at discharge, hemorrhagic transformation, and requirement of percutaneous endoscopic gastronomy tube placement, prolonged mechanical ventilation, or craniotomy. Results This study found no statistically significant difference in patient outcomes in those treated with concurrent carotid stenting compared to no carotid intervention in terms of morbidity or mortality. Conclusions If indicated, it is reasonable to consider concurrent carotid stenting and/or angioplasty for large-vessel anterior circulation stroke patients treated with mechanical thrombectomy who also receive intravenous thrombolytics.

Pathogenesis and diagnosis of otitis media with ANCA-associated vasculitis.

Science.gov (United States)

Yoshida, Naohiro; Iino, Yukiko

2014-12-01

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is histologically characterized by systemic necrotizing vasculitis and is clinically classified into two phases, systemic or localized. Recently, otological symptoms such as otitis media and hearing loss, not previously often associated with AAV, have been reported in AAV cases. In these cases we propose a diagnosis of otitis media with AAV (OMAAV). The ANCA titer is important for the diagnosis of OMAAV, and in most cases rapid progressive hearing loss is observed as localized AAV. Peripheral facial nerve palsy or hypertrophic pachymeningitis are coupled with 25% of cases and 18% of cases respectively. Proteinase 3-ANCA (PR3-ANCA) positive otitis media causes granulomatous formation or middle ear effusion in the middle ear, on the other hand myeloperoxidase-ANCA (MPO-ANCA) positive otitis media predominantly presents as otitis media with effusion. The early diagnosed case and the sensorineural hearing loss not progressed deaf could be recovered by the immunosuppressive therapy. Delayed diagnosis of AAV occasionally leads to progression to the irreversible phase; therefore, diagnosis at the early-localized stage is important for treating AAV. In this review, we discuss the current understanding of this newly proposed concept of OMAAV.

Isolated (localized) idiopathic granulomatous (giant cell) vasculitis in an intramuscular lipoma.

Science.gov (United States)

Fernando Val-Bernal, J; Val, Daniel; Calvo, Ignacio; Francisca Garijo, M

2006-01-01

Isolated (localized) idiopathic granulomatous vasculitis (IGV) is an uncommon, heterogeneous, and poorly defined group of disorders characterized by infiltration of the arterial wall caused by compactly grouped mononuclear phagocytes, with or without giant cells, in segmental distribution. We report on a 55-year-old woman with IGV limited to an intramuscular lipoma of the left thigh. The vasculitis was identified incidentally upon microscopic examination of the removed tumor. The IGV was centered on two medium-sized arteries, accompanied by narrowing of the lumens, and not associated with secondary changes such as infart or postinfart fibrosis. The inflammatory infiltrate was rich in T-lymphocytes and macrophages, with the presence of giant cells. The patient was asymptomatic and well in a follow-up period of 2 months, during which she was not treated. To our knowledge, this is the first report of lipoma involvement in localized IGV. It is important to distinguish cases of isolated intratumorous IGV from systemic disease, because the latter implies a poor prognosis and requires an aggressive treatment.

Giant-cell arteritis. Concordance study between aortic CT angiography and FDG-PET/CT in detection of large-vessel involvement

International Nuclear Information System (INIS)

Boysson, Hubert de; Dumont, Anael; Boutemy, Jonathan; Maigne, Gwenola; Martin Silva, Nicolas; Sultan, Audrey; Bienvenu, Boris; Aouba, Achille; Liozon, Eric; Ly, Kim Heang; Lambert, Marc; Aide, Nicolas; Manrique, Alain

2017-01-01

The purpose of our study was to assess the concordance of aortic CT angiography (CTA) and FDG-PET/CT in the detection of large-vessel involvement at diagnosis in patients with giant-cell arteritis (GCA). We created a multicenter cohort of patients with GCA diagnosed between 2010 and 2015, and who underwent both FDG-PET/CT and aortic CTA before or in the first ten days following treatment introduction. Eight vascular segments were studied on each procedure. We calculated concordance between both imaging techniques in a per-patient and a per-segment analysis, using Cohen's kappa concordance index. We included 28 patients (21/7 women/men, median age 67 [56-82]). Nineteen patients had large-vessel involvement on PET/CT and 18 of these patients also presented positive findings on CTA. In a per-segment analysis, a median of 5 [1-7] and 3 [1-6] vascular territories were involved on positive PET/CT and CTA, respectively (p = 0.03). In qualitative analysis, i.e., positivity of the procedure suggesting a large-vessel involvement, the concordance rate between both procedures was 0.85 [0.64-1]. In quantitative analysis, i.e., per-segment analysis in both procedures, the global concordance rate was 0.64 [0.54-0.75]. Using FDG-PET/CT as a reference, CTA showed excellent sensitivity (95%) and specificity (100%) in a per-patient analysis. In a per-segment analysis, sensitivity and specificity were 61% and 97.9%, respectively. CTA and FDG-PET/CT were both able to detect large-vessel involvement in GCA with comparable results in a per-patient analysis. However, PET/CT showed higher performance in a per-segment analysis, especially in the detection of inflammation of the aorta's branches. (orig.)

Giant-cell arteritis. Concordance study between aortic CT angiography and FDG-PET/CT in detection of large-vessel involvement

Energy Technology Data Exchange (ETDEWEB)

Boysson, Hubert de; Dumont, Anael; Boutemy, Jonathan; Maigne, Gwenola; Martin Silva, Nicolas; Sultan, Audrey; Bienvenu, Boris; Aouba, Achille [Caen University Hospital, Department of Internal Medicine, Caen (France); Liozon, Eric; Ly, Kim Heang [Limoges University Hospital, Department of Internal Medicine, Limoges (France); Lambert, Marc [Lille University Hospital, Department of Internal Medicine, Lille (France); Aide, Nicolas [Caen University Hospital, Department of Nuclear Medicine, Caen (France); INSERM U1086 ' ' ANTICIPE' ' , Francois Baclesse Cancer Centre, Caen (France); Manrique, Alain [Caen University Hospital, Department of Nuclear Medicine, Caen (France); Normandy University, Caen (France)

2017-12-15

The purpose of our study was to assess the concordance of aortic CT angiography (CTA) and FDG-PET/CT in the detection of large-vessel involvement at diagnosis in patients with giant-cell arteritis (GCA). We created a multicenter cohort of patients with GCA diagnosed between 2010 and 2015, and who underwent both FDG-PET/CT and aortic CTA before or in the first ten days following treatment introduction. Eight vascular segments were studied on each procedure. We calculated concordance between both imaging techniques in a per-patient and a per-segment analysis, using Cohen's kappa concordance index. We included 28 patients (21/7 women/men, median age 67 [56-82]). Nineteen patients had large-vessel involvement on PET/CT and 18 of these patients also presented positive findings on CTA. In a per-segment analysis, a median of 5 [1-7] and 3 [1-6] vascular territories were involved on positive PET/CT and CTA, respectively (p = 0.03). In qualitative analysis, i.e., positivity of the procedure suggesting a large-vessel involvement, the concordance rate between both procedures was 0.85 [0.64-1]. In quantitative analysis, i.e., per-segment analysis in both procedures, the global concordance rate was 0.64 [0.54-0.75]. Using FDG-PET/CT as a reference, CTA showed excellent sensitivity (95%) and specificity (100%) in a per-patient analysis. In a per-segment analysis, sensitivity and specificity were 61% and 97.9%, respectively. CTA and FDG-PET/CT were both able to detect large-vessel involvement in GCA with comparable results in a per-patient analysis. However, PET/CT showed higher performance in a per-segment analysis, especially in the detection of inflammation of the aorta's branches. (orig.)

Hydralazine-induced anti-neutrophil cytoplasmic antibody-positive renal vasculitis presenting with a vasculitic syndrome, acute nephritis and a puzzling skin rash: a case report

Directory of Open Access Journals (Sweden)

Keasberry Justin

2013-01-01

Full Text Available Abstract Introduction Anti-neutrophil cytoplasmic antibody-associated vasculitis has been associated with many drugs and it is a relatively rare side effect of the antihypertensive drug hydralazine. The diagnosis and management of patients who have anti-neutrophil cytoplasmic antibody-associated vasculitis may be challenging because of its relative infrequency, variability of clinical expression and changing nomenclature. The spectrum of anti-neutrophil cytoplasmic antibody-associated vasculitis is wide and can be fatal. This case documents a 62-year-old woman who presented with hydralazine-induced anti-neutrophil cytoplasmic antibody-positive renal vasculitis with a puzzling cutaneous rash. Case presentation We report a rare case of hydralazine-induced anti-neutrophil cytoplasmic antibody-associated vasculitis in a 62-year-old Caucasian woman who presented with a vasculitic syndrome with a sore throat, mouth ulcers and otalgia after several months of constitutional symptoms. She then proceeded to develop a rash over her right lower limb. Clinically, the rash had features to suggest Sweet’s syndrome, but also had some appearances consistent with embolic phenomena and did not have the appearance of palpable purpure usually associated with cutaneous vasculitis. Differential diagnoses were hydralazine-associated Sweet’s syndrome, streptococcal-induced cutaneous eruption or an unrelated contact dermatitis. A midstream urine sample detected glomerular blood cells in the setting of anti-neutrophil cytoplasmic antibody-positive renal vasculitis and Streptococcus pyogenes bacteremia. A renal biopsy revealed a pauci-immune, focally necrotizing glomerulonephritis with small crescents. Her skin biopsy revealed a heavy neutrophil infiltrate involving the full thickness of the dermis with no evidence of a leucocytoclastic vasculitis, but was non-specific. She was initially commenced on intravenous lincomycin for her bloodstream infection and subsequently

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.

Small ships don't shine: classification of ocean vessels from low resolution, large swath area SAR acquisitions

CSIR Research Space (South Africa)

Meyer, Rory GV

2016-07-01

Full Text Available the Understanding of Our Living Planet, 10-15 July 2016, Beijing, China Small ships don't shine: Classification of ocean vessels from low resolution, large swath area SAR acquisitions R. G. V. Meyer ; W. Kleynhans ; C. P. Schwegmann Abstract: Monitoring...

Vasculopathy in HIV-infected children – a case series

African Journals Online (AJOL)

%).1 A wide range of vascular disease can be encountered, from vasculitis caused by specific infective agents to nonspecific vasculitis. Cytomegalovirus and tuberculosis are leading infective causes,2 with small and medium-sized vessels ...

Early treatment for IgG4-related disease may prevent cognitive impairment caused by cerebral vasculitis: A case report and review of the literature

Directory of Open Access Journals (Sweden)

Toshihiko Usami

2018-03-01

Full Text Available IgG4-related disease (IgG4-RD is a recently recognized disease entity. A 74-year-old male presented with transient headache. He was diagnosed IgG4-RD by pancreatic biopsy at the age of 72. Magnetic Resonance Imaging (MRI showed disseminated cerebral microbleeds and microinfarctions in time and space. It suggested cerebral vasculitis, however any causative factor were not confirmed. IgG4-RD rarely causes cerebral vasculitis. This might be a first case of an asymptomatic cerebral vasculitis due to IgG4-RD. Patient was started on oral prednisolone, and no neurological or neuropsychological symptom was clinically observed. The MRI findings improved after treatment, and revealed no indication of newly lesions at 6-months follow-up. Early treatment for IgG4-RD may be recommended to prevent irreversible cognitive dysfunction. Keywords: IgG4-related disease, Treatment, Cerebral vasculitis

Estimation of turbulence dissipation rate by Large eddy PIV method in an agitated vessel

Directory of Open Access Journals (Sweden)

Kysela Bohuš

2015-01-01

Full Text Available The distribution of turbulent kinetic energy dissipation rate is important for design of mixing apparatuses in chemical industry. Generally used experimental methods of velocity measurements for measurement in complex geometries of an agitated vessel disallow measurement in resolution of small scales close to turbulence dissipation ones. Therefore, Particle image velocity (PIV measurement method improved by large eddy Ply approach was used. Large eddy PIV method is based on modeling of smallest eddies by a sub grid scale (SGS model. This method is similar to numerical calculations using Large Eddy Simulation (LES and the same SGS models are used. In this work the basic Smagorinsky model was employed and compared with power law approximation. Time resolved PIV data were processed by Large Eddy PIV approach and the obtained results of turbulent kinetic dissipation rate were compared in selected points for several operating conditions (impeller speed, operating liquid viscosity.

Catheter-Directed Thrombolysis of Deep Vein Thrombosis in a Patient with Churg-Strauss Syndrome: A Case Report

Energy Technology Data Exchange (ETDEWEB)

Park, Jun Beom; Kim, See Hyung; Choi, Jin Soo; Kim, Young Hwan [Dongsan Hospital, Keimyung University, Daegu (Korea, Republic of)

2010-10-15

Vasculitis by Churg-Strauss Syndrome (CSS) is an uncommon disease characterized by the inflammation of blood vessel walls and can involve many organs. The clinical manifestations and courses of vasculitis are highly variable. Deep vein thrombosis has rarely been reported in vasculitis by CSS. We report a case of deep vein thrombosis associated with CSS that was successfully treated by catheter-directed thrombolysis.

Catheter-Directed Thrombolysis of Deep Vein Thrombosis in a Patient with Churg-Strauss Syndrome: A Case Report

International Nuclear Information System (INIS)

Park, Jun Beom; Kim, See Hyung; Choi, Jin Soo; Kim, Young Hwan

2010-01-01

Vasculitis by Churg-Strauss Syndrome (CSS) is an uncommon disease characterized by the inflammation of blood vessel walls and can involve many organs. The clinical manifestations and courses of vasculitis are highly variable. Deep vein thrombosis has rarely been reported in vasculitis by CSS. We report a case of deep vein thrombosis associated with CSS that was successfully treated by catheter-directed thrombolysis

33 CFR 165.1317 - Security and Safety Zone; Large Passenger Vessel Protection, Puget Sound and adjacent waters...

Science.gov (United States)

2010-07-01

... 33 Navigation and Navigable Waters 2 2010-07-01 2010-07-01 false Security and Safety Zone; Large Passenger Vessel Protection, Puget Sound and adjacent waters, Washington. 165.1317 Section 165.1317 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF HOMELAND SECURITY (CONTINUED) PORTS AND WATERWAYS...

33 CFR 165.1318 - Security and Safety Zone Regulations, Large Passenger Vessel Protection, Portland, OR Captain of...

Science.gov (United States)

2010-07-01

... 33 Navigation and Navigable Waters 2 2010-07-01 2010-07-01 false Security and Safety Zone Regulations, Large Passenger Vessel Protection, Portland, OR Captain of the Port Zone 165.1318 Section 165.1318 Navigation and Navigable Waters COAST GUARD, DEPARTMENT OF HOMELAND SECURITY (CONTINUED) PORTS AND...

A general rehabilitation inpatient with exercise-induced vasculitis.

Science.gov (United States)

Cushman, Dan; Rydberg, Leslie

2013-10-01

While on our general inpatient rehabilitation floor, a 58-year-old man with no hematologic or dermatologic history developed an erythematous patch on his medial ankle that turned more purpuric, with a slight orange tint, and was associated with mild pruritus. The diagnosis of exercise-induced vasculitis was made after initially being mistaken for cellulitis. This common exanthem is often misdiagnosed. Due to its association with exercise, the physiatrist should be aware of its presence in both the inpatient and outpatient settings. Copyright © 2013 American Academy of Physical Medicine and Rehabilitation. Published by Elsevier Inc. All rights reserved.

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.

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.

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

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)

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.

Helium leak testing of large pressure vessels or subassemblies

International Nuclear Information System (INIS)

Hopkins, J.S.; Valania, J.J.

1977-01-01

Specifications for pressure-vessel components [such as the intermediate heat exchangers (IHX)] for service in the liquid metal fast breeder reactor facilities require helium leak testing of pressure boundaries to very exacting standards. The experience of Foster Wheeler Energy Corporation (FWEC) in successfully leak-testing the IHX shells and bundle assemblies now installed in the Fast Flux Test Facility at Richland, WA is described. Vessels of a somewhat smaller size for the closed loop heat exchanger system in the Fast Flux Test Facility have also been fabricated and helium leak tested for integrity of the pressure boundary by FWEC. Specifications on future components call for helium leak testing of the tube to tubesheet welds of the intermediate heat exchangers

Possible research program on a large scale nuclear pressure vessel

International Nuclear Information System (INIS)

1983-01-01

The nuclear pressure vessel structural integrity is actually one of the main items in the nuclear plants safety field. An international study group aimed at investigating the feasibility of a ''possible research program'' on a scale 1:1 LWR pressure vessel. This report presents the study group's work. The different research programs carried out or being carried out in various countries of the European Community are presented (phase I of the study). The main characteristics of the vessel considered for the program and an evaluation of activities required for making them available are listed. Research topic priorities from the different interested countries are summarized in tables (phase 2); a critical review by the study group of the topic is presented. Then, proposals for possible experimental programs and combination of these programs are presented, only as examples of possible useful research activities. The documents pertaining to the results of phase I inquiry performed by the study group are reported in the appendix

Extravasal occlusion of large vessels with titanic clips: efficiency, indications, and contraindications.

Science.gov (United States)

Vasilenko, Yu V; Kim, A I; Kotov, S A

2002-11-01

The mechanism of extravasal occlusion of blood vessels with titanic clips "Atrauclip" and "Ligaclip extra" was studied in order to reveal indications and contraindications to their use. Occlusion with the clips of both types was ineffective in vessels with a diameter of >7.0 mm. Arteritis or the presence of an intravascular occlusion facility in the vessel were also the contraindications for clip occlusion. In overcases the procedure of occlusion with titanic clips was efficient and atraumatic.

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

Hull Girder Fatigue Damage Estimations of a Large Container Vessel by Spectral Analysis

DEFF Research Database (Denmark)

Andersen, Ingrid Marie Vincent; Jensen, Jørgen Juncher

2013-01-01

This paper deals with fatigue damage estimation from the analysis of full-scale stress measurements in the hull of a large container vessel (9,400 TEU) covering several months of operation. For onboard decision support and hull monitoring sys-tems, there is a need for a fast reliable method...... for esti-mation of fatigue damage in the ship hull. The objective of the study is to investigate whether the higher frequency contributions from the hydroelastic responses (springing and whipping) can satisfactory be included in the fatigue damage estimation by only a few parameters derived from the stress...

OMERACT Endorsement of Patient-reported Outcome Instruments in Antineutrophil Cytoplasmic Antibody–associated Vasculitis

Science.gov (United States)

Robson, Joanna C.; Tomasson, Gunnar; Milman, Nataliya; Ashdown, Sue; Boonen, Annelies; Casey, George C.; Cronholm, Peter F.; Cuthbertson, David; Dawson, Jill; Direskeneli, Haner; Easley, Ebony; Kermani, Tanaz A.; Farrar, John T.; Gebhart, Don; Lanier, Georgia; Luqmani, Raashid A.; Mahr, Alfred; McAlear, Carol A.; Peck, Jacqueline; Shea, Beverley; Shea, Judy A.; Sreih, Antoine G.; Tugwell, Peter S.; Merkel, Peter A.

2018-01-01

Objective The antineutrophil cytoplasmic antibody–associated vasculitides (AAV) are multiorgan diseases. Patients with AAV report impairment in their health-related quality of life (HRQOL) and have different priorities regarding disease assessment compared with physicians. The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group previously received endorsement for a core set of domains in AAV. Two approaches to measure patient-reported outcomes (PRO) were presented at OMERACT 2016. Methods A novel 5-step tool was used to facilitate assessment of the instruments by delegates: the OMERACT Filter 2.0 Instrument Selection Algorithm, with a red-amber-green checklist of questions, including (1) good match with domain (face and content validity), (2) feasibility, (3) do numeric scores make sense (construct validity)?, (4) overall ratings of discrimination, and (5) can individual thresholds of meaning be defined? Delegates gave an overall endorsement. Three generic Patient-Reported Outcomes Measurement Information System (PROMIS) instruments (fatigue, physical functioning, and pain interference) and a disease-specific PRO, the AAV-PRO (6 domains related to symptoms and HRQOL), were presented. Results OMERACT delegates endorsed the use of the PROMIS instruments for fatigue, physical functioning, and pain interference (87.6% overall endorsement) and the disease-specific AAV-PRO instrument (89.4% overall endorsement). Conclusion The OMERACT Vasculitis Working Group gained endorsement by OMERACT for use of the PROMIS and the AAV-PRO in clinical trials of vasculitis. These instruments are complementary to each other. The PROMIS and the AAV-PRO need further work to assess their utility in longitudinal settings, including their ability to discriminate between treatments of varying efficacy in the setting of a randomized controlled trial. PMID:28864650

Pulmonary Vasculitis and a Horseshoe Kidney in Noonan Syndrome

Directory of Open Access Journals (Sweden)

Surasak Puvabanditsin

2018-01-01

Full Text Available We report a term male neonate with congenital myeloproliferative disorder, thrombocytopenia, a horseshoe kidney, feeding difficulty secondary to dysphagia/foregut dysmotility, and respiratory failure. Prenatal molecular genetic analysis revealed a fetus carrying c.184T>G (p.Tyr62Asp pathogenic variant in PTPN11. The infant eventually succumbed to respiratory failure. Bacterial and viral cultures/studies were all no growth/negative. Pulmonary capillaritis and vasculitis were noted at autopsy. This report presents a new case of Noonan syndrome with unusual associated disorders and a review of the literature.

Pulmonary Vasculitis and a Horseshoe Kidney in Noonan Syndrome.

Science.gov (United States)

Puvabanditsin, Surasak; Abellar, Rosanna; Madubuko, Adaora; Mehta, Rajeev; Walzer, Lauren

2018-01-01

We report a term male neonate with congenital myeloproliferative disorder, thrombocytopenia, a horseshoe kidney, feeding difficulty secondary to dysphagia/foregut dysmotility, and respiratory failure. Prenatal molecular genetic analysis revealed a fetus carrying c.184T>G (p.Tyr62Asp) pathogenic variant in PTPN11 . The infant eventually succumbed to respiratory failure. Bacterial and viral cultures/studies were all no growth/negative. Pulmonary capillaritis and vasculitis were noted at autopsy. This report presents a new case of Noonan syndrome with unusual associated disorders and a review of the literature.

Offshore wind transport and installation vessel

Energy Technology Data Exchange (ETDEWEB)

NONE

2001-07-01

The initial objective of the project was to complete a feasibility study to determine the viability of an innovative transportation vessel to be deployed in the installation of offshore wind farms. This included the feasibility of providing a stable-working platform that can be used in harsh offshore environments. A study of current installation contractors and their installation equipment was used to provide a preliminary specification for the installation vessel. A typical barge was selected and a number of hydrodynamic analyses were carried out in order to establish it's on course and operational stability. The analysis proved the stability of the vessel during operation was critical and that in order to utilise the crane's full potential a stabilisation system must be employed. The main aim of the work to date was to establish whether it was feasible to use a stabilisation system on the installation vessel. The spud leg FEED study established that it was feasible to use spud legs to stabilise the vessel. In order to achieve the degree of stability required it is necessary to lift the vessel completely out of the water. This was not the original aim of the study but due to the external loads on the hull it was the only viable option. Lifting the vessel out of the water results in the legs and leg casings becoming very large. This has a number of consequences for the final design. Due to large loads on the legs spud cans must be used to avoid bottom penetration, the spud cans increase the draft of the vessel by 2m. The large loads require larger winches and more reeving to be used, this results in larger pumps and motors, all of which have to be housed. The stabilisation system has been proved to be feasible for a large installation vessel, the cost and physical size are however more excessive than first anticipated. (Author)

Sulfated glycosaminoglycans in cultured endothelial cells from capillaries and large vessels of human and bovine origin

International Nuclear Information System (INIS)

Bar, R.S.; Dake, B.L.; Spanheimer, R.G.

1985-01-01

The ( 35 S)glycosaminoglycans (( 35 S)GAG) synthesized by capillary endothelial cells were analyzed and compared to GAG synthesized by endothelial cells cultured from 4 larger vessels. Two separate cultures of endothelial cells were established from bovine fat capillaries and from 4 larger vessels of human origin (umbilical vein) and bovine origin (pulmonary artery, pulmonary vein and aorta). After incubation with 35 SO 4 for 72 h, the ( 35 S)glycosaminoglycans (GAG) composition of the media, pericellular and cellular fractions of each culture were determined by selective degradation with nitrous acid, chondroitinase ABC and chondroitinase AC. All endothelial cells produced large amounts of ( 35 S)GAG with increased proportions of heparinoids (heparan sulfate and heparin) in the cellular and pericellular fractions. Each culture showed a distinct distribution of ( 35 S)GAG in the media, pericellular and cellular fractions with several specific differences found among the 5 cultures. The differences in GAG content were confirmed in a second group of separate cultures from each of the 5 vessels indicating that, although having several features of GAG metabolism in common, each endothelial cell culture demonstrated a characteristic complement of synthesized, secreted and cell surface-sulfated glycosaminoglycans. (author)

Prestressed cast iron pressure vessels as burst-proof pressure vessels for innovative nuclear applications

International Nuclear Information System (INIS)

Froehling, W.; Boettcher, A.; Bounin, D.; Steinwarz, W.; Geiss, M.; Trauth, M.

2000-01-01

The amendment to the German Atomic Energy Act from July 28, 1994 requires that events 'whose occurrence is practically excluded by the measures against damages', i.e. events of the category residual risk, must not necessitate far reaching protective measures outside the plant. For a conventional reactor pressure vessel, the residual risk consists in the very small probability of a catastrophic failure (formation of a large fracture opening, bursting of the vessel). With a prestressed cast iron vessel (PCIV), the formation of a large fracture opening or bursting of the vessel, respectively, is impossible due to its design properties. Against this background the possibility of the use of this type of pressure vessel for lightwater reactors has been studied in the frame of a 'Working Group for Innovative Nuclear Technology', founded by different research institutes and industrial companies. Furthermore, it has been studied whether the use of the PCIV support the realization of a corecatcher system. The results are presented in this report. Already many years earlier, Siempelkamp has performed industrial development and Forschungszentrum Juelich related experimental and theoretical safety research for the PCIV as an innovative, bust-proof pressure vessel concept. This development of the PCIV as well as its safety properties are also presented in a conclusive manner. (orig.) [de

Recurrent symptomatic ischemic stroke in a 46-year-old African male revealing Angio-Behçet with severe cardiovascular involvement

OpenAIRE

Ba Djibril Marie; Diack Aminata; Mboup Mouhamed Cherif; Fall Moussa Daouda

2017-01-01

Behçet’sdisease (BD) is a chronic, multisystem vasculitis. It is categorized under variable vessel vasculitis in the new Chapel Hill nomenclature as it involves blood vessels of any type and size. It is characterized by relapsing aphthous ulcers commonly occurring in the oral mucosa and genitalia with ocular involvement. Other organ systems may be involved any time throughout the course of the disease. The exact cause is unknown. However, combination of genetic and environmental factors is li...

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.)

MR-angiography in vasculitis and benign angiopathy of the central nervous system; MR-Angiographie in der Diagnostik von Vaskulitiden und benignen Angiopathien des Zentralnervensystems

Energy Technology Data Exchange (ETDEWEB)

Schlueter, A.; Hirsch, W.; Jassoy, A.; Behrmann, C.; Spielmann, R.P. [Klinik fuer Diagnostische Radiologie, Martin-Luther-Univ. Halle-Wittenberg (Germany); Kornhuber, M. [Klinik fuer Neurologie, Martin-Luther-Univ. Halle-Wittenberg (Germany); Keysser, G. [Klinik fuer Innere Medizin I, Martin-Luther-Univ. Halle-Wittenberg (Germany)

2001-06-01

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{sub 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.) [German] Ziel der Studie war es, den Wert der TOF 3D Magnetresonanzangiographie (MRA) bei Patienten mit Vaskulitis oder vaskulitisaehnlichen, benignen Angiopathien des Zentralnervensystems (ZNS) zu erfassen. Methoden: Die MRA-Aufnahmen von 20 Patienten mit klinisch und bildgebend nachgewiesener Vaskulitis (17/20) oder vaskulitisaehnlicher, benigner Angiopathie (3/20) des ZNS wurden retrospektiv ausgewertet. Eingeschlossen wurden Patienten mit Laesionen des Hirnparenchymsgroessen 3 mm in den T{sub 2}-gewichteten Sequenzen der MRT, wobei diese Veraenderungen aufgrund umfangreicher klinischer Untersuchungen nicht auf das Vorliegen neurodegenerativer, anderweitig entzuendlicher, metastatischer oder embolischer Ursachen zurueckzufuehren waren. Fuer die MRA wurde eine TOF 3D FISP-Sequenz auf einem 1,5T MR-Geraet verwendet. Ergebnisse: MR-angiographisch wurden bei 15/20 Patienten (75

Primær vaskulitis i barnealderen - nye klassifikationskriterier

DEFF Research Database (Denmark)

Herlin, Troels; 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...

Recommendations of the Brazilian Society of Rheumatology for the induction therapy of ANCA-associated vasculitis

Directory of Open Access Journals (Sweden)

Alexandre Wagner Silva de Souza

Full Text Available Abstract The purpose of these recommendations is to guide the appropriate induction treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV patients with active disease. The recommendations proposed by the Vasculopathies Committee of the Brazilian Society Rheumatology for induction therapy of AAV, including granulomatosis with polyangiitis, microscopic polyangiitis and renal-limited vasculitis, were based on systematic literature review and expert opinion. Literature review was performed using Medline (PubMed, EMBASE and Cochrane database to retrieve articles until October 2016. PRISMA guidelines were used for the systematic review and articles were assessed according to the Oxford levels of evidence. Sixteen recommendations were made regarding different aspects of induction therapy for AAV. The purpose of these recommendations is to serve as a guide for therapeutic decisions by health care professionals in the management of AAV patients presenting active disease.

Characterizing the relative contributions of large vessels to total ocean noise fields: a case study using the Gerry E. Studds Stellwagen Bank National Marine Sanctuary.

Science.gov (United States)

Hatch, Leila; Clark, Christopher; Merrick, Richard; Van Parijs, Sofie; Ponirakis, Dimitri; Schwehr, Kurt; Thompson, Michael; Wiley, David

2008-11-01

In 2006, we used the U.S. Coast Guard's Automatic Identification System (AIS) to describe patterns of large commercial ship traffic within a U.S. National Marine Sanctuary located off the coast of Massachusetts. We found that 541 large commercial vessels transited the greater sanctuary 3413 times during the year. Cargo ships, tankers, and tug/tows constituted 78% of the vessels and 82% of the total transits. Cargo ships, tankers, and cruise ships predominantly used the designated Boston Traffic Separation Scheme, while tug/tow traffic was concentrated in the western and northern portions of the sanctuary. We combined AIS data with low-frequency acoustic data from an array of nine autonomous recording units analyzed for 2 months in 2006. Analysis of received sound levels (10-1000 Hz, root-mean-square pressure re 1 microPa +/- SE) averaged 119.5 +/- 0.3 dB at high-traffic locations. High-traffic locations experienced double the acoustic power of less trafficked locations for the majority of the time period analyzed. Average source level estimates (71-141 Hz, root-mean-square pressure re 1 microPa +/- SE) for individual vessels ranged from 158 +/- 2 dB (research vessel) to 186 +/- 2 dB (oil tanker). Tankers were estimated to contribute 2 times more acoustic power to the region than cargo ships, and more than 100 times more than research vessels. Our results indicate that noise produced by large commercial vessels was at levels and within frequencies that warrant concern among managers regarding the ability of endangered whales to maintain acoustic contact within greater sanctuary waters.

Antibodies against linear epitopes on Goodpasture autoantigen in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis.

Science.gov (United States)

Jia, Xiao-Yu; Yu, Jun-Tao; Hu, Shui-Yi; Li, Jian-Nan; Wang, Miao; Wang, Chen; Chen, Min; Cui, Zhao; Zhao, Ming-Hui

2017-09-01

In a substantial number of patients with crescentic glomerulonephritis, both anti-glomerular basement membrane (GBM) antibodies and anti-neutrophil cytoplasmic antibodies (ANCA) are detected simultaneously. ANCA is presumed to be the initial event but the mechanism is unknown. In the present study, we investigated the antibodies against linear epitopes on Goodpasture autoantigen in sera from patients with ANCA-associated vasculitis, aiming to reveal the mechanisms of the coexistence of the two kinds of autoantibodies. Thirty-one patients with ANCA-associated vasculitis were enrolled in this study. Twenty-four overlapping linear peptides were synthesized across the whole sequence of Goodpasture autoantigen. Serum antibodies against linear peptides were detected by ELISA and their associations with clinical features were further analyzed. Twenty-five out of the thirty-one (80.6%) sera from patients with ANCA-associated vasculitis possessed antibodies against linear peptides on Goodpasture autoantigen. These antibodies could be detected in 50% of patients with normal renal function (Scr ≤ 133 μmol/L), 70% of patients with moderate renal dysfunction (133 μmol/L  600 μmol/L) (P = 0.032). The highest recognition frequencies were found for peptides P4 (51.6%), P14 (54.8%), and P24 (54.8%), which contained the sequences that constitute the conformational epitopes of E A (P4) and E B (P14) recognized by anti-GBM antibodies. The level of anti-P4 antibodies was positively correlated with the percentage of crescents in glomeruli (r = 0.764, P = 0.027). Patients with anti-P24 antibodies had a significantly higher prevalence of renal dysfunction on diagnosis (88.2 vs. 42.9%, P = 0.018). Antibodies against linear epitopes on Goodpasture autoantigen could be detected in sera of patients with ANCA-associated vasculitis, which might mediate the production of antibodies towards the conformational epitopes on Goodpasture autoantigen, namely, the anti-GBM antibodies.

50 CFR 216.92 - Dolphin-safe requirements for tuna harvested in the ETP by large purse seine vessels.

Science.gov (United States)

2010-10-01

... 50 Wildlife and Fisheries 7 2010-10-01 2010-10-01 false Dolphin-safe requirements for tuna... MAMMALS REGULATIONS GOVERNING THE TAKING AND IMPORTING OF MARINE MAMMALS Dolphin Safe Tuna Labeling § 216.92 Dolphin-safe requirements for tuna harvested in the ETP by large purse seine vessels. (a) U.S...

Sulfated glycosaminoglycans in cultured endothelial cells from capillaries and large vessels of human and bovine origin

Energy Technology Data Exchange (ETDEWEB)

Bar, R.S.; Dake, B.L.; Spanheimer, R.G.

1985-07-01

The (/sup 35/S)glycosaminoglycans ((/sup 35/S)GAG) synthesized by capillary endothelial cells were analyzed and compared to GAG synthesized by endothelial cells cultured from 4 larger vessels. Two separate cultures of endothelial cells were established from bovine fat capillaries and from 4 larger vessels of human origin (umbilical vein) and bovine origin (pulmonary artery, pulmonary vein and aorta). After incubation with /sup 35/SO/sub 4/ for 72 h, the (/sup 35/S)glycosaminoglycans (GAG) composition of the media, pericellular and cellular fractions of each culture were determined by selective degradation with nitrous acid, chondroitinase ABC and chondroitinase AC. All endothelial cells produced large amounts of (/sup 35/S)GAG with increased proportions of heparinoids (heparan sulfate and heparin) in the cellular and pericellular fractions. Each culture showed a distinct distribution of (/sup 35/S)GAG in the media, pericellular and cellular fractions with several specific differences found among the 5 cultures. The differences in GAG content were confirmed in a second group of separate cultures from each of the 5 vessels indicating that, although having several features of GAG metabolism in common, each endothelial cell culture demonstrated a characteristic complement of synthesized, secreted and cell surface-sulfated glycosaminoglycans. (author). 16 refs.

Simultaneous central retinal artery occlusion and optic nerve vasculitis in Crohn disease

Directory of Open Access Journals (Sweden)

Razek Georges Coussa

2017-04-01

Conclusions and importance: To our knowledge, this is the first case of unilateral CRAO and bilateral optic nerve occlusive vasculitis in Crohn disease, which should be considered as an etiology of retinal vascular occlusive disorders especially in young patients. It is important for ophthalmologists to be aware of the ophthalmic risks associated with Crohn disease as aggressive treatment with systemic steroids and immunosuppressive agents is often needed.

Henoch–Schönlein purpura: a clinical case with dramatic presentation

Science.gov (United States)

Bento, João; Magalhães, Adriana; Moura, Conceição Souto; Hespanhol, Venceslau

2010-01-01

A case study involving a 55-year-old Caucasian male with end-stage glomerulosclerosis is presented here. Kidney biopsies showed no deposits on imunofluorescent microscopy. Relapsing massive haemoptysis and suspected bronchovascular malformation required lung lobectomy which revealed malformative and tortuous small blood vessels, with no vasculitis. Blood antinuclear antibodies, antineutrophil cytoplasmic antibodies and antiglomerular basement membrane antibodies were undetectable. Seric immunoglobulins and complement levels were normal. Three months later, arthralgia and joint oedema first appeared. Skin biopsy revealed vasculitis immune-reactive to immunoglobulin A. Systemic corticotherapy was then started. Two weeks later, the patient presented with abdominal pain melena and rectal bleeding (haematoquesia). Endoscopic study showed diffuse gastrointestinal haemorrhage. Angiographic study revealed diffuse lesions compatible with vasculitis and haemorrhage from multiple spots. Cyclophosphamide and then intravenous immunoglobulin were associated to treatment without response. Increasing blood loss occurred, with massive gastrointestinal haemorrhage and haemorrhagic ascitis. Death occurred due to uncontrolled diffuse bleeding. Necropsy findings showed generalised small vessels vasculitis compatible with Henoch–Schönlein purpura. PMID:22778211

Pediatric vasculitides: a generalists approach.

Science.gov (United States)

Khubchandani, Raju P; Viswanathan, V

2010-10-01

Vasculitis is defined as the presence of inflammation in a blood vessel that may occur as a primary process or secondary to an underlying disease. Primary vasculitides are rare in children. These are defined by both the size of vessels involved and the type of inflammatory response. Clinical features consist of multi-organ involvement on a background of constitutional features reflecting the size and location of the blood vessels involved. Whilst some vasculitides are best diagnosed clinically, many forms require sophisticated imaging and other investigations (auto antibodies) to reveal the correct diagnosis. Prompt recognition and treatment is crucial as many of the vasculitides cause significant morbidity or mortality. Treatment options range from symptomatic therapy, immunosuppresive agents, intravenous immunoglobulin (IVIG) or biologic agents and are determined by the type of vasculitis, the severity of the inflammation, and the organ systems affected. Early detection and aggressive treatment is crucial for the best outcomes in the most severe forms of childhood vasculitis.

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.)

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

The challenge of treating hepatitis C virus-associated cryoglobulinemic vasculitis in the era of anti-CD20 monoclonal antibodies and direct antiviral agents.

Science.gov (United States)

Roccatello, Dario; Sciascia, Savino; Rossi, Daniela; Solfietti, Laura; Fenoglio, Roberta; Menegatti, Elisa; Baldovino, Simone

2017-06-20

Mixed cryoglobulinemia syndrome (MC) is a systemic vasculitis involving kidneys, joints, skin, and peripheral nerves. While many autoimmune, lymphoproliferative, and neoplastic disorders have been associated with this disorder, hepatitis C virus (HCV) is known to be the etiologic agent in the majority of patients. Therefore, clinical research has focused on anti-viral drugs and, more recently, on the new, highly potent Direct-acting Antiviral Agents (DAAs). These drugs assure sustained virologic response (SVR) rates >90%. Nevertheless, data on their efficacy in patients with HCV-associated cryoglobulinemic vasculitis are disappointing, possibly due to the inability of the drugs to suppress the immune-mediated process once it has been triggered.Despite the potential risk of exacerbation of the infection, immunosuppression has traditionally been regarded as the first-line intervention in cryoglobulinemic vasculitis, especially if renal involvement is severe. Biologic agents have raised hopes for more manageable therapeutic approaches, and Rituximab (RTX), an anti CD20 monoclonal antibody, is the most widely used biologic drug. It has proved to be safer than conventional immunosuppressants, thus substantially changing the natural history of HCV-associated cryoglobulinemic vasculitis by providing long-term remission, especially with intensive regimens.The present review focuses on the new therapeutic opportunities offered by the combination of biological drugs, mainly Rituximab, with DAAs.

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...... outcome); change in renal function, adverse events, and cumulative dose of cyclophosphamide (secondary outcomes). RESULTS: Groups did not differ in time to remission (hazard ratio, 1.098 [95% CI, 0.78 to 1.55]; P = 0.59) or proportion of patients who achieved remission at 9 months (88.1% vs. 87...... 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....

Vessel calibration for accurate material accountancy at RRP

International Nuclear Information System (INIS)

Yanagisawa, Yuu; Ono, Sawako; Iwamoto, Tomonori

2004-01-01

RRP has a 800t·Upr capacity a year to re-process, where would be handled a large amount of nuclear materials as solution. A large scale plant like RRP will require accurate materials accountancy system, so that the vessel calibration with high-precision is very important as initial vessel calibration before operation. In order to obtain the calibration curve, it is needed well-known each the increment volume related with liquid height. Then we performed at least 2 or 3 times run with water for vessel calibration and careful evaluation for the calibration data should be needed. We performed vessel calibration overall 210 vessels, and the calibration of 81 vessels including IAT and OAT were held under presence of JSGO and IAEA inspectors taking into account importance on the material accountancy. This paper describes outline of the initial vessel calibration and calibration results based on back pressure measurement with dip tubes. (author)

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.

High Prevalence of Autoantibodies to hLAMP-2 in Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

NARCIS (Netherlands)

Kain, Renate; Tadema, Henko; McKinney, Eoin F.; Benharkou, Alexandra; Brandes, Ricarda; Peschel, Andrea; Hubert, Virginie; Feenstra, Tjerk; Sengoelge, Guerkan; Stegeman, Coen; Heeringa, Peter; Lyons, Paul A.; Smith, Kenneth G. C.; Kallenberg, Cees; Rees, Andrew J.

The involvement of autoantibodies to human lysosome-associated membrane protein-2 (hLAMP-2) in anti neutrophil cytoplasmic antibody (ANCA) associated vasculitis is controversial because of the absence of confirmatory data subsequent to the initial reports of their high prevalence in this disease. We

A method of decontaminating large vessels

International Nuclear Information System (INIS)

Williams, John.

1989-01-01

A method of removing contamination from the surfaces of nuclear reactors, heat exchangers and pressure vessels which requires the use of only small volumes of liquid and no abrasives involves contacting the contaminated surface with a moisture laden gas and effecting condensation of the moisture in the gas on the surface by lowering the temperature of the surface to be cleaned before introducing the moisture laden gas. A reactive cleaning agent is concurrently or subsequently introduced and allowed to react with the contaminated surface to produce a water soluble reaction product which is washed away. To ensure the retention of a wetted surface for reactant contact and to avoid loss of reactant contact by evaporation or drainage at the surface a modification of the method provides for the production of the gelatinous layer on the surface to be cleaned. (author)

Large-scale boiling experiments of the flooded cavity concept for in-vessel core retention

International Nuclear Information System (INIS)

Chu, T.Y.; Slezak, S.E.; Bentz, J.H.; Pasedag, W.F.

1994-01-01

This paper presents results of ex-vessel boiling experiments performed in the CYBL (CYlindrical BoiLing) facility. CYBL is a reactor-scale facility for confirmatory research of the flooded cavity concept for accident management. CYBL has a tank-within-a-tank design; the inner tank simulates the reactor vessel and the outer tank simulates the reactor cavity. Experiments with uniform and edge-peaked heat flux distributions up to 20 W/cm 2 across the vessel bottom were performed. Boiling outside the reactor vessel was found to be subcooled nucleate boiling. The subcooling is mainly due to the gravity head which results from flooding the sides of the reactor vessel. The boiling process exhibits a cyclic pattern with four distinct phases: direct liquid/solid contact, bubble nucleation and growth, coalescence, and vapor mass dispersion (ejection). The results suggest that under prototypic heat load and heat flux distributions, the flooded cavity in a passive pressurized water reactor like the AP-600 should be capable of cooling the reactor pressure vessel in the central region of the lower head that is addressed by these tests

Foundamental characteristics of layered pressure vessel

International Nuclear Information System (INIS)

Moriwaki, Yoshikazu; Fugino, Masayuki; Shimizu, Yasuhiro; Nakamura, Takeshi

1978-01-01

Pressure vessels become larger and the working pressure become higher with the remarkable development of petroleum, chemical, thermal power generation and atomic energy industries. Multi-layered pressure vessels can be manufactured cheaply without large installations, and large wall thickness can be made, therefore they are suitable for large pressure vessels. The stress and deformation behaviors of such vessels are very complex because of the effect of frictional force working between layers. In this study, the phenomena arising in multiple layers and the difference as compared with single wall were studied fundamentally as one step for analyzing multi-layered pressure vessels as a whole. Finite element technique was employed as the analyzing method, and the behavior of multiple layers was analyzed, regarding it as multiple contact problem. The behavior of multiple layers seems to appear conspicuously in case of bending load, therefore the basic characteristics regarding bending were examined. The evaluation of interfacial stiffness was carried out by experiment. The computer program for analyzing multiple contact problem was developed. In order to examine the validity of the program, comparison with the analytical solution heretofore and the result of calculation by finite element technique was carried out. Moreover, the experimental proof with multi-layered models was made. The frictional force between layers hardly contributes to the stiffness. (Kako, I.)

The numerical simulation of the WWER-440/V-213 reactor pressure vessel internals response to maximum hypothetical large break loss of coolant accident

International Nuclear Information System (INIS)

Hermansky, P.; Krajcovic, M.

2012-01-01

The reactor internals are designed to ensure cooling of the fuel, to ensure the movement of emergency control assemblies under all operating conditions including accidents and facilitate removal of the fuel and of the internals following an accident This paper presents results of the numerical simulation of the WWER-440/V213 reactor vessel internals dynamic response to maximum hypothetical Large-Break Loss of Coolant Accident. The purpose of this analysis is to determine the reactor vessel internals response due to rapid depressurization and to prove no such deformations occur in the reactor vessel internals which would prevent timely and proper activation of the emergency control assemblies. (Authors)

Plasmapheresis Rescue Therapy in Progressive Systemic ANCA-Associated Vasculitis : Single-Center Results of Stepwise Escalation of Immunosuppression

NARCIS (Netherlands)

de Joode, Anoek A. E.; Sanders, Jan Stephan; Smid, W. Martin; Stegeman, Coen A.

2014-01-01

Objective: We evaluated 26 patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) with progressive disease despite treatment with cyclophosphamide and steroids treated with additional plasmapheresis and compared outcome with 50 matched-disease controls. Methods:

Tumor Blood Vessel Dynamics

Science.gov (United States)

Munn, Lance

2009-11-01

``Normalization'' of tumor blood vessels has shown promise to improve the efficacy of chemotherapeutics. In theory, anti-angiogenic drugs targeting endothelial VEGF signaling can improve vessel network structure and function, enhancing the transport of subsequent cytotoxic drugs to cancer cells. In practice, the effects are unpredictable, with varying levels of success. The predominant effects of anti-VEGF therapies are decreased vessel leakiness (hydraulic conductivity), decreased vessel diameters and pruning of the immature vessel network. It is thought that each of these can influence perfusion of the vessel network, inducing flow in regions that were previously sluggish or stagnant. Unfortunately, when anti-VEGF therapies affect vessel structure and function, the changes are dynamic and overlapping in time, and it has been difficult to identify a consistent and predictable normalization ``window'' during which perfusion and subsequent drug delivery is optimal. This is largely due to the non-linearity in the system, and the inability to distinguish the effects of decreased vessel leakiness from those due to network structural changes in clinical trials or animal studies. We have developed a mathematical model to calculate blood flow in complex tumor networks imaged by two-photon microscopy. The model incorporates the necessary and sufficient components for addressing the problem of normalization of tumor vasculature: i) lattice-Boltzmann calculations of the full flow field within the vasculature and within the tissue, ii) diffusion and convection of soluble species such as oxygen or drugs within vessels and the tissue domain, iii) distinct and spatially-resolved vessel hydraulic conductivities and permeabilities for each species, iv) erythrocyte particles advecting in the flow and delivering oxygen with real oxygen release kinetics, v) shear stress-mediated vascular remodeling. This model, guided by multi-parameter intravital imaging of tumor vessel structure

Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss).

Science.gov (United States)

Cottin, Vincent; Bel, Elisabeth; Bottero, Paolo; Dalhoff, Klaus; Humbert, Marc; Lazor, Romain; Sinico, Renato A; Sivasothy, Pasupathy; Wechsler, Michael E; Groh, Matthieu; Marchand-Adam, Sylvain; Khouatra, Chahéra; Wallaert, Benoit; Taillé, Camille; Delaval, Philippe; Cadranel, Jacques; Bonniaud, Philippe; Prévot, Grégoire; Hirschi, Sandrine; Gondouin, Anne; Dunogué, Bertrand; Chatté, Gérard; Briault, Christophe; Pagnoux, Christian; Jayne, David; Guillevin, Loïc; Cordier, Jean-François

2017-01-01

To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA. Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease. The study population included 157 patients (mean age 49.4±14.1), with a follow-up of 7.4±6.4years. Patients with ANCA (31%) more frequently had weight loss, myalgias, arthralgias, biopsy-proven vasculitis, glomerulonephritis on biopsy, hematuria, leukocytoclastic capillaritis and/or eosinophilic infiltration of arterial wall on biopsy, and other renal disease. A total of 41% of patients had definite vasculitis manifestations (37%) or strong surrogates of vasculitis (4%), of whom only 53% had ANCA. Mononeuritis multiplex was associated with systemic vasculitis (p=0.005) and with the presence of ANCA (p<0.001). Overall, 59% of patients had polyangiitis as defined by definite vasculitis, strong surrogate of vasculitis, mononeuritis multiplex, and/or ANCA with at least one systemic manifestation other than ENT or respiratory. Patients with polyangiitis had more systemic manifestations including arthralgias (p=0.02) and renal disease (p=0.024), had higher peripheral eosinophilia (p=0.027), and a trend towards less myocarditis (p=0.057). Using predefined criteria of vasculitis and surrogates of vasculitis, ANCA alone were found to be insufficient to categorise patients with vasculitis features. We suggest a revised nomenclature and definition for EGPA and a new proposed entity referred to as hypereosinophilic asthma with systemic (non vasculitic) manifestations. Copyright © 2016 Elsevier B.V. All rights reserved.

Cutaneous collagenous vasculopathy: A rare case report

Directory of Open Access Journals (Sweden)

Kinjal Deepak Rambhia

2016-01-01

Full Text Available Cutaneous collagenous vasculopathy (CCV is a distinct, rare, and underdiagnosed condition. We report a case of CCV in a 50-year-old woman presenting as asymptomatic, erythematous to hyperpigmented nonblanchable macules over both the lower extremities. The clinical differential diagnosis of the lesions was pigmented purpuric dermatoses (Schamberg's purpura and cutaneous small vessel vasculitis. Histology of the lesions revealed dilated superficial dermal vessels with abundant pink hyaline material in the vessel wall, which stained with periodic acid Schiff stain. The patient was diagnosed as CCV. This condition remains largely underdiagnosed and is commonly mistaken for pigmented purpuric dermatosis or generalized essential telangiectasia. Emphasis on the differentiation of CCV from its clinical and histological mimicks is made.

VASCULITIS DE CHURG-STRAUSS: PRESENTACIÓN CLÍNICA COMO GLOMERULONEFRITIS EXTRACAPILAR NECROTIZANTE PAUCI-INMUNE CON NEFRITIS TUBULO-INTERSTICIAL EOSINOFÍLICA.

Directory of Open Access Journals (Sweden)

Fernanda da Cunha

2004-01-01

Full Text Available RESUMEN: Se presenta el caso clínico de una mujer de 81 años con antecedentes patología pulmonar obstructiva crónica "idiopática" que desarrolló un cuadro de insuficiencia renal aguda aparentemente prerrenal, con posterior oliguria y eosinofilia simulando una nefropatía túbulo-intersticial alérgica. La evolución atípica y la presencia de p-ANCA, sugerían una vasculitis, patología que se confirmó con la biopsia renal. Los resultados anatomopatológicos revelaron la existencia de una glomerulonefritis necrotizante con semilunas e infiltrado eosinófilo. Estos datos y el historial de la paciente llevaron al diagnóstico de vasculitis de Churg-Strauss. El tratamiento inicial con corticoides y ciclofosfamida y posteriormente con Azatioprina mostró excelentes resultados con mejoría de la función renal, de los parámetros inflamatorios y de la clínica sistémica, que la paciente mantuvo posteriormente. ABSTRACT: We report a case of a 81 year-old woman with idiopathic chronic obstructive pulmonary disease, who developed a functional acute renal failure with delayed oliguria and eosinophily, simulating an acute interstitial nephropathy. The unusual clinical course and the presence of antimyeloperoxidase antibodies (p-ANCA suggested a vasculitis; the renal biopsy confirmed this diagnosis. The histology revealed a crescentic glomerulonephritis with eosinophilic infiltration. With these data and the pulmonary history of the patient, a diagnosis of Churg-Struss Vasculitis was made. The initial treatment with steroids and ciclophosphamide switched later to azatioprine, was succeeded with a sustained improvement in renal function, inflammatory markers and clinical course.

Design of the ITER vacuum vessel

International Nuclear Information System (INIS)

Ioki, K.; Johnson, G.; Shimizu, K.; Williamson, D.

1995-01-01

The ITER vacuum vessel is a major safety barrier and must support electromagnetic loads during plasma disruptions and vertical displacement events (VDE) and withstand plausible accidents without losing confinement.The vacuum vessel has a double wall structure to provide structural and electrical continuity in the toroidal direction. The inner and outer shells and poloidal stiffening ribs between them are joined by welding, which gives the vessel the required mechanical strength. The space between the shells will be filled with steel balls and plate inserts to provide additional nuclear shielding. Water flowing in this space is required to remove nuclear heat deposition, which is 0.2-2.5% of the total fusion power. The minor and major radii of the tokamak are 3.9 m and 13 m respectively, and the overall height is 15 m. The total thickness of the vessel wall structure is 0.4-0.7 m.The inboard and outboard blanket segments are supported from the vacuum vessel. The support structure is required to withstand a large total vertical force of 200-300 MN due to VDE and to allow for differential thermal expansion.The first candidate for the vacuum vessel material is Inconel 625, due to its higher electric resistivity and higher yield strength, even at high temperatures. Type 316 stainless steel is also considered a vacuum vessel material candidate, owing to its large database and because it is supported by more conventional fabrication technology. (orig.)

Treatment of hepatocellular carcinoma adjacent to large blood vessels using 1.5T MRI-guided percutaneous radiofrequency ablation combined with iodine-125 radioactive seed implantation

Energy Technology Data Exchange (ETDEWEB)

Lin, Zheng-Yu, E-mail: linsinlan@yahoo.com.cn [The Department of Radiology, First Affiliated Hospital of Fujian Medical University, 20 Chazhong Road, Fuzhou 350005 (China); Chen, Jin, E-mail: snow8968851@163.com [The Department of Radiology, First Affiliated Hospital of Fujian Medical University, 20 Chazhong Road, Fuzhou 350005 (China); Deng, Xiu-Fen, E-mail: dxf197286@yahoo.com.cn [The Department of Radiology, First Affiliated Hospital of Fujian Medical University, 20 Chazhong Road, Fuzhou 350005 (China)

2012-11-15

Objective: The objective is to study the technology associated with and feasibility of the treatment of hepatocellular carcinoma (HCC) adjacent to large blood vessels using 1.5T MRI-guided radiofrequency ablation combined with iodine-125 (I-125) radioactive seed implantation. Methods: Sixteen patients with a total of 24 HCC lesions (average maximum diameter: 2.35 {+-} 1.03 cm) were pathologically confirmed by biopsy or clinically diagnosed received 1.5T MRI-guided percutaneous radiofrequency ablation (RFA) treatment. Each patient had one lesion adjacent to large blood vessels ({>=}3 mm); after the ablation, I-125 radioactive seeds were implanted in the portions of the lesions that were adjacent to the blood vessels. Results: All the ablations and I-125 radioactive seed implantations were successful; a total of 118 seeds were implanted. The ablated lesions exhibited hypointense signals on the T2WI sequence with a thin rim of hyperintense signals; they also exhibited significant hyperintense signals on the T1WI sequence with clear boundaries. The average follow-up period was 11.1 {+-} 6.2 months. There were 23 complete responses and one partial response in the 24 lesions. The alpha-fetoprotein (AFP) levels of the patients significantly decreased. Conclusion: The 1.5T MRI-guided RFA combined with I-125 radioactive seed implantation for the treatment of HCC adjacent to large blood vessels is an effective technology.

A genetic study of Factor V Leiden (G1691A) mutation in young ischemic strokes with large vessel disease in a South Indian population.

Science.gov (United States)

Anadure, Ravi; Christopher, Rita; Nagaraja, Dindagur; Narayanan, Coimbatore

2017-10-01

Factor V Leiden (FVL) has been, by far, the most investigated gene mutation, with 26 studies to date, on its role in arterial strokes. Overall, a meta-analysis of all these studies taken together showed that carriers of the Factor V Leiden allele were 1.33times more likely to develop arterial strokes when compared to controls. We subjected a highly select subset of young strokes, with large vessel infarcts, to genetic analysis for FVL mutation and compared them with matched healthy controls to look for a statistically significant association. In this prospective study, 6/120 cases (5%) and 2/120 controls (1.6%) were positive for heterozygous FVL (G1691A) mutation. The higher prevalence of FVL mutation in cases (5%) compared to controls (1.6%) did not show statistical significance with a Pearson's Chi square P value of 0.15. The Odds Ratio (OR) for risk of large vessel disease in FVL positive cases was 3.10 (95% CI of 0.61-15.7). FVL mutation (G1691A) in young Indian subjects with ischemic strokes does not seem to be significantly associated with large vessel disease. Copyright © 2017 Elsevier Ltd. All rights reserved.

Analyzing Vessel Behavior Using Process Mining

NARCIS (Netherlands)

Maggi, F.M.; Mooij, A.J.; Aalst, W.M.P. van der

2013-01-01

In the maritime domain, electronic sensors such as AIS receivers and radars collect large amounts of data about the vessels in a certain geographical area. We investigate the use of process mining techniques for analyzing the behavior of the vessels based on these data. In the context of maritime

Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study.

Science.gov (United States)

Sada, Ken-ei; Yamamura, Masahiro; Harigai, Masayoshi; Fujii, Takao; Dobashi, Hiroaki; Takasaki, Yoshinari; Ito, Satoshi; Yamada, Hidehiro; Wada, Takashi; Hirahashi, Junichi; Arimura, Yoshihiro; Makino, Hirofumi

2014-04-23

We investigated the clinical and serological features of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan using data from a nationwide, prospective, inception cohort study. In total, 156 Japanese patients with newly diagnosed AAV were classified according to the European Medicines Agency (EMEA) algorithm with exploratory surrogate markers for AAV-related non-granulomatous pulmonary lesions, predefined as alveolar haemorrhage and interstitial lung disease (ILD), and their clinical and serological features were evaluated. Using the EMEA algorithm, we identified 14 patients (9.0%) with eosinophilic granulomatosis with polyangiitis (EGPA), 33 (21.2%) with granulomatosis with polyangiitis (GPA), 78 (50.0%) with microscopic polyangiitis and renal-limited vasculitis (MPA/RLV), and 31 (19.9%) with unclassifiable vasculitis. The average ages of patients with EGPA (male/female, 5/9), GPA (12/21), and MPA/RLV (35/43) and unclassifiable (9/22) were 58.0, 63.6, 71.1, and 70.6 years, respectively. Myeloperoxidase (MPO)-ANCA and proteinase-3 ANCA positivity was 50.0% and 0% for EGPA, 54.6% and 45.5% for GPA, 97.4% and 2.6% for MPA/RLV, and 93.5% and 3.2% for unclassifiable, respectively. According to the Birmingham Vasculitis Activity Score (BVAS), cutaneous (71.4%) and nervous system (92.9%) manifestations were prominent in EGPA and ear, nose, and throat manifestations (84.9%) and chest manifestations (66.7%) in GPA. Renal manifestations developed frequently in MPA/RLV (91.0%) and GPA (63.6%). The average serum creatinine levels were 0.71 mg/dL for EGPA, 1.51 mg/dL for GPA, 2.46 mg/dL for MPA/RLV, and 0.69 mg/dL for unclassifiable. The percentages of patients with ILD were 14.3% for EGPA, 9.0% for GPA, 47.4% for MPA/RLV, and 61.3% for unclassifiable. Patients with ILD (n = 61) had significantly lower BVAS (P = 0.019) with fewer ear, nose, and throat and cardiovascular manifestations than patients without ILD (n = 95). MPO

Bilateral ocular ischemia-induced blindness as a presenting manifestation of Takayasu arteritis: a case report

OpenAIRE

Pallangyo, Pedro; Epafra, Emmanuel; Nicholaus, Paulina; Lyimo, Frederick; Kazahura, Parvina; Janabi, Mohamed

2017-01-01

Background Takayasu arteritis is a granulomatous panarteritis that predominantly affects the aorta and its major branches. The initial manifestations of this large-vessel vasculitis are usually nonspecific; however, as the disease progresses, typical symptoms of arterial occlusion, aneurysmal formation, and vascular pain become evident. Ischemic ocular complications of Takayasu arteritis which could lead to complete loss of vision are not uncommon and depend on the obliterated portion(s) of c...

Urinary CD4+ Effector Memory T Cells Reflect Renal Disease Activity in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

NARCIS (Netherlands)

Abdulahad, Wayel H.; Kallenberg, Cees G. M.; Limburg, Pieter C.; Stegeman, Coen A.

Objective. Numbers of circulating CD4+ effector memory T cells are proportionally increased in patients with proteinase 3 antineutrophil cytoplasmic antibody-associated vasculitis (AAV) whose disease is in remission and are decreased during active disease, which presumably reflects their migration

PWR vessel inspection performance improvements

International Nuclear Information System (INIS)

Blair Fairbrother, D.; Bodson, Francis

1998-01-01

A compact robot for ultrasonic inspection of reactor vessels has been developed that reduces setup logistics and schedule time for mandatory code inspections. Rather than installing a large structure to access the entire weld inspection area from its flange attachment, the compact robot examines welds in overlapping patches from a suction cup anchor to the shell wall. The compact robot size allows two robots to be operated in the vessel simultaneously. This significantly reduces the time required to complete the inspection. Experience to date indicates that time for vessel examinations can be reduced to fewer than four days. (author)

The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of fifty-seven patients.

Science.gov (United States)

Jachiet, Marie; Flageul, Béatrice; Deroux, Alban; Le Quellec, Alain; Maurier, François; Cordoliani, Florence; Godmer, Pascal; Abasq, Claire; Astudillo, Leonardo; Belenotti, Pauline; Bessis, Didier; Bigot, Adrien; Doutre, Marie-Sylvie; Ebbo, Mikaël; Guichard, Isabelle; Hachulla, Eric; Héron, Emmanuel; Jeudy, Géraldine; Jourde-Chiche, Noémie; Jullien, Denis; Lavigne, Christian; Machet, Laurent; Macher, Marie-Alice; Martel, Clotilde; Melboucy-Belkhir, Sara; Morice, Cécile; Petit, Antoine; Simorre, Bernard; Zenone, Thierry; Bouillet, Laurence; Bagot, Martine; Frémeaux-Bacchi, Véronique; Guillevin, Loïc; Mouthon, Luc; Dupin, Nicolas; Aractingi, Selim; Terrier, Benjamin

2015-02-01

Hypocomplementemic urticarial vasculitis (HUV) is an uncommon vasculitis of unknown etiology that is rarely described in the literature. We undertook this study to analyze the clinical spectrum and the therapeutic management of patients with HUV. We conducted a French nationwide retrospective study that included 57 patients with chronic urticaria, histologic leukocytoclastic vasculitis, and hypocomplementemia. We assessed clinical and laboratory data and evaluated the patients' cutaneous and immunologic responses to therapy. We evaluated treatment efficacy by measuring the time to treatment failure. Urticarial lesions were typically more pruritic than painful and were associated with angioedema in 51% of patients, purpura in 35%, and livedo reticularis in 14%. Extracutaneous manifestations included constitutional symptoms (in 56% of patients) as well as musculoskeletal involvement (in 82%), ocular involvement (in 56%), pulmonary involvement (in 19%), gastrointestinal involvement (in 18%), and kidney involvement (in 14%). Patients with HUV typically presented with low C1q levels and normal C1 inhibitor levels, in association with anti-C1q antibodies in 55% of patients. Hydroxychloroquine or colchicine seemed to be as effective as corticosteroids as first-line therapy. In patients with relapsing and/or refractory disease, rates of cutaneous and immunologic response to therapy seemed to be higher with conventional immunosuppressive agents, in particular, azathioprine, mycophenolate mofetil, or cyclophosphamide, while a rituximab-based regimen tended to have higher efficacy. Finally, a cutaneous response to therapy was strongly associated with an immunologic response to therapy. HUV represents an uncommon systemic and relapsing vasculitis with various manifestations, mainly, musculoskeletal and ocular involvement associated with anti-C1q antibodies, which were found in approximately half of the patients. The best strategy for treating HUV has yet to be defined

Numerical modeling of in-vessel melt water interaction in large scale PWR`s

Energy Technology Data Exchange (ETDEWEB)

Kolev, N.I. [Siemens AG, KWU NA-M, Erlangen (Germany)

1998-01-01

This paper presents a comparison between IVA4 simulations and FARO L14, L20 experiments. Both experiments were performed with the same geometry but under different initial pressures, 51 and 20 bar respectively. A pretest prediction for test L21 which is intended to be performed under an initial pressure of 5 bar is also presented. The strong effect of the volume expansion of the evaporating water at low pressure is demonstrated. An in-vessel simulation for a 1500 MW el. PWR is presented. The insight gained from this study is: that at no time are conditions for the feared large scale melt-water intermixing at low pressure in force, with this due to the limiting effect of the expansion process which accelerates the melt and the water into all available flow paths. (author)

Vasculitis asociadas a anticuerpos anti-citoplasma de neutrófilos: Clínica y tratamiento

Directory of Open Access Journals (Sweden)

María Virginia Paolini

2013-04-01

Full Text Available Las vasculitis asociadas a anticuerpos anti-citoplasma de neutrófilos (ANCA comprenden a un grupo de enfermedades caracterizadas por la inflamación de la pared de pequeños vasos. Analizamos las características epidemiológicas y clínicas en una serie de 47 pacientes: 23 (49% granulomatosis de Wegener (GW, 15 (32% poliangeítis microscópica (PAM y nueve (19% vasculitis limitada al riñón (VLR. La edad media al inicio de los síntomas fue de 50.7 ± 14.9 años. La manifestación clínica más frecuente fue el compromiso renal en 41 (87% pacientes, seguido por el pulmonar en 26 (55% y el otorrinolaringológico en 17 (36%. En 26 (55% se asoció compromiso renal y pulmonar. La forma clínica más frecuente fue la generalizada en 23 (49%, seguida por la grave en 18 (38%. El 89% presentaron determinaciones de ANCA positivas. Cuatro (8% no recibieron tratamiento inmunosupresor de inicio. De los 43 que recibieron tratamiento de inicio, 29 (67% tuvieron remisión completa, con un tiempo de remisión promedio de 35.3 meses. Once (26% presentaron recaídas, diez (91% recaídas mayores y uno (9% menor. Doce (28% fallecieron, siete en forma temprana y cinco durante la evolución de la enfermedad. Quince (31% evolucionaron a insuficiencia renal crónica. Los 26 pacientes en seguimiento tuvieron respuesta al tratamiento y 20 (77% de ellos estaban en remisión al finalizar el estudio. Las vasculitis asociadas a ANCA continúan siendo enfermedades de alta morbilidad y mortalidad, a pesar de las mejorías logradas con los tratamientos inmunosupresores.

Deformation behavior of large, high-pressure vessel flanges

International Nuclear Information System (INIS)

Spaas, H.A.C.M.; Latzko, D.G.H.

1975-01-01

The analysis of the deformation behavior of large high-pressure vessel flanges poses a much more difficult problem than for low-pressure flanges due to their particular geometry. For a particularly narrow flange geometry (typical of PWR flanges) a finite-element analysis (MARC-IBM-program, eight-node, isoparametric ring elements) was used to predict the behavior of the flange rings. The nonlinear elastic problem resulting from the local closing and/or opening of the partial gap between the gasket faces was solved by an incremental technique using gap elements. The resulting deformation behavior of the flange system has been compared to that obtained from an analysis using the refined rigid ring concept for both bolt-tightening and hydro-testing conditions. The elasto-plastic analysis was solved by the same finite element program system as mentioned above. The incremental steps describing the nonlinear material behavior are allowed to be larger than those for the gap-closure mechanism. Besides a comparison with the former elastic analyses an interpretation will be given of the local plasticity effects, which result in a shift in location of the gasket reaction. Experimental data on local gasket face deformation was obtained by a specially developed laser beam apparatus, with the leak detection channel of the flange serving as a beam hole. Additionally strain gauges were used on flanges and bolts, in combination with special sensing pins for the determination of relative flange rotations. Results obtained so far indicate that for high-pressure flanges of the narrow design investigated here the deformation behavior is best described by an elasto-plastic finite element analysis