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Sample records for wegener granulomatosis computerized

  1. Granulomatosis with Polyangiitis (Wegener's)

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    ... Patient / Caregiver Diseases & Conditions Granulomatosis with Polyangitis (Wegner's) Granulomatosis with Polyangiitis (Wegener's) Fast Facts Granulomatosis with polyangiitis (Wegener's) - GPA - is ...

  2. Wegener's Granulomatosis

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    ... such as lactoferrin. ANCAs may be not only markers for Wegener’s granulomatosis and related disorders, but they may also be actors in pathogenesis. Studies show that when neutrophils are exposed to cytokines such as tumor necrosis factor, small amounts of ...

  3. WEGENER GRANULOMATOSIS- CASE REPORT

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    Ivanka Djordjevic

    2008-10-01

    Full Text Available Wegener granulomatosis is uncommon multisystemic disease, characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tracts and general focal necrotizing vasculitis (Commonly known as „Wegener's triad“. The lungs are involved in 72 per cent of patients and the clinic and radiographic findings indicated bilateral pulmonary nodules of varying size and definition, cavitated in half of the patients, accompanied by the nodular lesion with a rare involvement of the pleura.We described a case of 62-year-old women with pansinusitis, mild azotemia and initial respiratory tract symptoms such as chronic cough and occasional hemoptysis. Due to bilateral nodular infiltrates in lungs on chest radiogram she was initially treated for smear negative pulmonary tuberculosis, but without expected antituberculous response. An additional diagnostic procedure pointed to Morbus Wegener.Two patterns of ANCA positive immunofluorescence are recognized as reliable and valuable diagnostic tools in the absence of histopathology for the diagnosis of Wegener granulomatosis.

  4. Granulomatose de Wegener Wegener's granulomatosis

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    Telma Antunes

    2005-07-01

    Full Text Available A granulomatose de Wegener caracteriza-se por vasculite necrosante granulomatosa que acomete preferencialmente vias aéreas superiores, inferiores e rins. Seu diagnóstico é feito associando-se as manifestações clínicas, radiológicas (multiplos nódulos escavados e os achados anatomopatológicos e o anticorpo anticitoplasma de neutrófilos positivo. O tratamento com corticosteróides e ciclofosfamida leva a 90% de remissão da doença em 1 ano.Wegener's granulomatosis is characterized by granulomatous necrotizing vasculitis that primarily affects the airways (upper and lower and the kidneys. The diagnosis is made by analyzing the clinical and radiological manifestations (multiple pulmonary cavitations, together with the pathological findings and results of the test for cytoplasmic-pattern antineutrophil cytoplasmic antibodies. Treatment consists of corticosteroids and cyclophosphamide and leads to remission of the disease within one year in 90% of cases.

  5. Wegener granulomatosis masquerading as pneumonia.

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    Theodorou, D J; Theodorou, S J; Mpougias, K; Mastora, M; Stefanaki, S; Akritidis, N C

    2010-01-01

    We report a case of an elderly patient with a limited form of Wegener granulomatosis, which simulated the clinical and imaging features of organizing pneumonia. Here we call attention to this atypical case presentation that eloquently illustrates the many faces of Wegener granulomatosis.

  6. Pachymeningitis in Wegener`s granulomatosis

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    Burrell, H.; McConachie, N.S. [City and Univeristy Hospital, Nottingham (United Kingdom). Department of Radiology

    1998-11-01

    A case of Wegener`s granulomatosis is described in which meningeal thickening and enhancement was demonstrated on MR imaging. The diagnosis was suggested by the clinical picture, imaging findings and a positive anti-neutrophil cytoplasmic antibody; and confirmed by biopsy of ethmoidal tissue. Copyright (1998) Blackwell Science Pty Ltd 17 refs., 1 fig.

  7. Orbital Granulomatosis With Polyangiitis (Wegener Granulomatosis)

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    Muller, Karra; Lin, Jonathan H.

    2014-01-01

    The pathology of granulomatosis with polyangiitis (GPA), formerly Wegener granulomatosis, typically features a granulomatous and sometimes necrotizing vasculitis targeting the respiratory tract and kidneys. However, orbital involvement occurs in up to 60% of patients and is frequently the first or only clinical presentation in patients with systemic or limited forms of GPA. Orbital GPA can cause significant morbidity and potentially lead to complete loss of vision and permanent facial deformity. Fortunately, GPA is highly responsive to medical treatment with corticosteroids combined with cyclophosphamide or, more recently, rituximab. Therefore, it is imperative for this disease to be accurately diagnosed on orbital biopsy and distinguished from other histologically similar orbital lesions. Herein, we review the clinical and pathologic findings of orbital GPA, focusing on the differentiation of this disease from other inflammatory orbital lesions. PMID:25076302

  8. Echocardiographic findings in patients with Wegener granulomatosis.

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    Oliveira, Guilherme H M; Seward, James B; Tsang, Teresa S M; Specks, Ulrich

    2005-11-01

    To describe the spectrum and clinical implications of echocardiographic findings associated with Wegener granulomatosis. We retrospectively reviewed the clinical records and echocardiographic data of consecutive patients with confirmed Wegener granulomatosis referred to the echocardiography laboratory during the 21-year period from 1976 through 1997. Of the 85 patients Identified as having confirmed Wegener granulomatosis, 73 (86%) were found to have echocardiographic abnormalities. In 26 (36%) of these 73 patients, lesions appeared directly related to Wegener granulomatosis. We found regional wall motion abnormalities in 17 (65%) of these 26 patients. Left ventricular systolic dysfunction with decreased ejection fraction was found in 13 patients (50%) and pericardial effusion in 5 patients (19%). Other findings Included valvulitis, left ventricular aneurysm, and a large intracardlac mass. A significantly increased mortality rate was observed among patients who had cardiac involvement of Wegener granulomatosis found by echocardiography. We found a high frequency of echocardiographic abnormalities that appear to be related to Wegener granulomatosis and associated with Increased mortality. Because cardiac involvement in Wegener granulomatosis often is silent and associated with Increased morbidity and worse prognosis, echocardlographic screening of patients with active Wegener granulomatosis may be of clinical value.

  9. Supraglottic stenosis in localized Wegener granulomatosis.

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    Belloso, Antonio; Estrach, Cristina; Keith, Andrew O

    2008-07-01

    We present what we believe is the first reported case of a patient with supraglottic stenosis secondary to Wegener granulomatosis. The diagnosis was unclear initially because the biopsy results were nonspecific, but a finding of an elevated cytoplasmic-pattern antineutrophil cytoplasmic antibody (c-ANCA) level established the diagnosis of localized supraglottic Wegener granulomatosis. Wegener granulomatosis is characterized by necrotizing vasculitis that is localized predominantly to the kidneys and the upper and lower airways. In the airways, subglottic involvement is well documented, but to the best of our knowledge, supraglottic stenosis has not previously been described. Localized forms of Wegener granulomatosis are characterized by limited disease that involves only the upper airway. The diagnosis in localized forms is complex because histology is diagnostic in only 50% of cases, and only 60% of patients have a positive c-ANCA level. We discuss the diagnostic criteria and management strategies for these localized forms.

  10. Rituximab for severe refractory pediatric Wegener granulomatosis.

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    Patel, Aarat M; Lehman, Thomas J A

    2008-10-01

    We describe a case of pediatric Wegener granulomatosis initially treated with cyclophosphamide and oral corticosteroids resulting in remission for 5 years. Of note in this case is relapse with severe pulmonary disease treated with multiple regimens, all unsuccessful. Patient achieved remission with rituximab infusion therapy. This demonstrates how rituximab may be beneficial for childhood-onset Wegener granulomatosis unresponsive to conventional therapy. The case is followed by a review of the current treatment options.

  11. Wegener granulomatosis--rare case presentation.

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    Bande, Uday; Baligar, Basavaraj; Terdal, Nandish

    2012-10-01

    Wegener granulomatosis is often misdiagnosed as pneumonia and most common cause for bilateral lung infiltrates' are bacterial, viral, pneumocystis jiroveci infection. We describe a 35 year old female with 15 days history of nasal obstruction, breathlessness and fever. Investigations in this patient revealed chronic necrotizing vasculitic and granulomatous lesion in nasal biopsy, vasculitic pauciimmune glomerulonephritis in renal biopsy, serology test was positive for C-ANCA, nodular with cystic lesion in CT-thorax. All these findings confirmed the diagnosis of Wegener granulomatosis.

  12. Tracheal Stenosis Because of Wegener Granulomatosis Misdiagnosed as Asthma.

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    O'Hear, Kelley E; Ingrande, Jerry; Brodsky, Jay B; Morton, John M; Sung, Chih-Kwang

    2016-05-15

    We describe a patient with Wegener granulomatosis whose complaint of wheezing was incorrectly attributed to asthma. Anesthesiologists must recognize that tracheal stenosis is extremely common in Wegener granulomatosis and can mimic other causes of wheezing.

  13. Treatment of orbital inflammation with rituximab in Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Baslund, Bo; Wiencke, Anne Katrine; Rasmussen, Niels

    2012-01-01

    To study the efficacy of rituximab therapy for the treatment of orbital inflammation in patients with Wegener's granulomatosis (WG).......To study the efficacy of rituximab therapy for the treatment of orbital inflammation in patients with Wegener's granulomatosis (WG)....

  14. Wegener Granulomatosis: Otologic Manifestation as First Symptom.

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    Costa, Carla Fabiane da; Polanski, Jose Fernando

    2015-07-01

    Introduction Wegener granulomatosis is a systemic vasculitis affecting small and medium-sized vessels of the upper and lower respiratory tract and kidneys. Objective To describe a case of Wegener disease with atypical manifestation. Resumed Report We describe the case of a 50-year-old woman with chronic otitis media and sensorineural hearing loss as the primary symptoms, without other manifestations. Conclusion In cases of acute ear manifestations with or without hearing loss and with poor response to usual treatments, Wegener granulomatosis should be included among the possible etiologies. After adequate diagnoses and treatment of this rare disease, there was favorable evolution.

  15. Wegener Granulomatosis: Otologic Manifestation as First Symptom

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    Costa, Carla Fabiane da

    2014-11-01

    Full Text Available Introduction Wegener granulomatosis is a systemic vasculitis affecting small and medium-sized vessels of the upper and lower respiratory tract and kidneys. Objective To describe a case of Wegener disease with atypical manifestation. Resumed Report We describe the case of a 50-year-old woman with chronic otitis media and sensorineural hearing loss as the primary symptoms, without other manifestations. Conclusion In cases of acute ear manifestations with or without hearing loss and with poor response to usual treatments, Wegener granulomatosis should be included among the possible etiologies. After adequate diagnoses and treatment of this rare disease, there was favorable evolution.

  16. Deoxyspergualin in relapsing and refractory Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Flossmann, O; Baslund, B; Bruchfeld, A

    2008-01-01

    OBJECTIVES: Conventional therapy of Wegener's granulomatosis with cyclophosphamide and corticosteroids is limited by incomplete remissions and a high relapse rate. The efficacy and safety of an alternative immunosuppressive drug, deoxyspergualin, was evaluated in patients with relapsing...... or refractory disease. METHODS: A prospective, international, multicentre, single-limb, open-label study. Entry required active Wegener's granulomatosis with a Birmingham vasculitis activity score (BVAS) > or =4 and previous therapy with cyclophosphamide or methotrexate. Immunosuppressive drugs were withdrawn......-threatening (> or = grade 3) treatment-related adverse events occurred in 24 (53%) patients mostly due to leucopaenias. CONCLUSIONS: Deoxyspergualin achieved a high rate of disease remission and permitted prednisolone reduction in refractory or relapsing Wegener's granulomatosis. Adverse events were common but rarely led...

  17. Sclerosing Wegener's granulomatosis in the orbit

    DEFF Research Database (Denmark)

    Ostri, Christoffer; Heegaard, Steffen; Prause, Jan Ulrik

    2008-01-01

    PURPOSE: To report three cases of sclerosing Wegener's granulomatosis in the orbit and to compare the histopathological morphology with those of other types of sclerosing orbital inflammation. METHODS: We analysed the clinical data along with histopathological specimens from orbital biopsies......, tissue necrosis, vasculitis and widespread dense fibrosis. In certain areas of all specimens the fibrous tissue was arranged in characteristic concentric, onionskin-like whorls of collagen around obliterated small blood vessels. CONCLUSIONS: Wegener's granulomatosis may lead to orbital fibrosis...... and sclerosis. The stromal changes and cellular infiltrate resemble the non-specific orbital inflammation previously termed orbital pseudotumour. We present the first 'onionskin' lesions in association with Wegener's granulomatosis in the orbit. This latter morphology may represent an abnormal fibrotic reaction...

  18. Wegener s granulomatosis with granulomatous liver involvement.

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    Holl-Ulrich, Konstanze; Klass, Monika

    2010-01-01

    We report on a patient with biopsy proven systemic Wegener's granulomatosis (WG) with a granulomatous necrotising manifestation of WG in the liver, lung, parotid gland and skin with subsequent death of liver failure. Liver involvement in WG is an exceedingly rare, though potentially fatal, organ manifestation of WG.

  19. Wegener's granulomatosis in childhood

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    Neumann, G.; Benz-Bohm, G.; Rister, M.

    1984-07-01

    Wegener's granulomatosis (WG) is a rare disease in pediatric patients. The case of a 14-year-old girl with generalized WG is described and the pediatric literature reviewed. Thirty additional cases of WG in childhood have been traced in previous studies. Diagnostic problems are discussed in the context of clinical, radiological and biopsy findings.

  20. Application of computed tomography in Wegener's granulomatosis

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    Nagaoka, S.; Tani, K.; Kato, K. (Yokohama City Univ. (Japan). Faculty of Medicine)

    1982-06-01

    Computed tomographic (CT) findings in four patiemts with Wegener's granulomatosis were investigated. The diagnosis of Wegener's granulomatosis was made by clinical symptoms and the histological examination of biopsied materials from the nasal mucosa, bronchus and kidney. CT findings in three patients were compatible with the clinical and laboratory manifestations of Wegener's granulomatosis. In one case, CT showed a widening of the nasal cavity, bone destruction of the ethmoid and maxillary sinus. These results suggest that CT studies are very effective to evaluate the extent of Wegener's granulomatosis and its therapeutic effect.

  1. Granulomatose de Wegener: relatos de casos Wegener's granulomatosis: cases reports

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    Ivana Cardoso Pereira

    2007-12-01

    Full Text Available A granulomatose de Wegener é descrita como uma tríade de lesões: granuloma necrosante do trato respiratório, vasculite disseminada e glomerulonefrite. Pode ocorrer de maneira sistêmica ou localizada. O envolvimento ocular e orbitário é comum em ambas as formas da doença, estando presente em 50% dos casos. O exame anatomopatológico e o c-ANCA+ foram fundamentais no diagnóstico efetivo da granulomatose de Wegener a despeito do envolvimento sistêmico nos casos apresentados.Wegener granulomatosis (WG is characterized by a classic triad of granulomatous inflammation of the respiratory tract, necrotizing vasculitis and nephritis. The absence of renal disease defines a subset of " limited WG" . Approximately 50% of WG patients develop ophthalmic disease. The histopatological study and +c-ANCA were essential to make a definite diagnosis in these cases.

  2. [Massive alveolar haemorrhage in Wegener's granulomatosis].

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    Valero-Roldán, J; Nuñez-Castillo, D; Fernández-Fígares, C; López-Leiva, I

    2014-01-01

    Wegener's granulomatosis is a systemic vasculitis with involvement of primary granulomatous upper and lower respiratory tract, glomerulonephritis and vasculitis of small vessels. The lung disease ranges from asymptomatic pulmonary nodules to pulmonary infiltrates and fulminant alveolar haemorrhage. The prognosis is poor due to kidney and respiratory failure, although the data are changing due to new treatments with glucocorticoids and cyclophosphamide. We report a case with severe lung disease, which after appropriate anamnesis, multiple tests, and optimal sequential action, the patient was diagnosed with Wegener's granulomatosis. This disease has a low incidence in the Emergency Department, where the patient history supported by the appropriate additional provides a diagnostic suspicion. It is important that the Emergency Department has the skills to manage the stability in these patients in order to resolve their symptoms. Copyright © 2013 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España. All rights reserved.

  3. Paranasal sinus obliteration in Wegener granulomatosis

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    Paling, M.R.; Roberts, R.L.; Fauci, A.S.

    1982-08-01

    The authors report 14 cases of Wegener granulomatosis in which one or more paranasal sinuses were obliterated by bone. The maxillary antra were involved in all cases, with the other sinuses being affected less frequently. These changes are thought to result from chronic bacterial sinusitis superimposed on the granulomatous vasculitic process. Computed tomography dramatically demonstrated the bone changes, consisting of a combination of sinus wall thickening and trabeculated new bone formation within the sinuses.

  4. Kidney involvement in a Wegener granulomatosis case.

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    Cavoli, Gioacchino Li; Ferrantelli, Angelo; Bono, Luisa; Tortorici, Calogera; Passantino, Rita; Rotolo, Ugo

    2012-01-01

    Wegener Granulomatosis is a systemic Anti-Neutrophil Cytoplasmic Autoantibody-associated Vasculitis, affecting small-to-medium vessels. Clinical presentation with simultaneous involvement of kidney and upper and lower respiratory tract is unusual. We report an instructive case of WG, analyzing clinical course, laboratory, and radiological features, kidney, lung, and larynx histological pictures. Besides renal biopsy, nephrology team performed larynx and lung biopsies because of unusual clinical presentation, computed tomography chest examination, and relevant malignancy risk regarding following immunosuppressant therapy.

  5. Serum chitotriosidase activity and Wegener's granulomatosis.

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    Koening, Curry L; Gota, Carmen E; Langford, Carol A; Hoffman, Gary S; Natowicz, Marvin R

    2010-03-01

    We evaluated serum chitotriosidase activity in patients with Wegener's granulomatosis (WG) and compared the values to controls. We used a standard fluorometric assay to measure chitotriosidase enzyme activity. Serum chitotriosidase enzyme activity levels were higher in WG patients. We found no association between clinical disease activity and chitotriosidase enzyme activity. Serum chitotriosidase enzyme activity has limited utility as a biomarker in WG patients. Published by Elsevier Inc.

  6. Diagnostic Consideration for Sinonasal Wegener's Granulomatosis Clinically Mistaken for Carcinoma

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    La Rosa, Cristina; Emmanuele, Carmela; Tranchina, Maria Grazia; Ippolito, Massimo; Cosentino, Sebastiano; Saita, Vincenzo; Fraggetta, Filippo

    2013-01-01

    We report a case of Wegener's granulomatosis clinically mistaken for carcinoma in a 21-year-old girl presenting with an ulcerated mass of the nasopharynx associated with enlarged laterocervical nodes. The lesion was clinically suspected as malignant on the basis of clinical and radiological findings (namely, computed tomography scan and positron emission tomography). However, multiple biopsies were not conclusive for malignancy showing histological change suggestive of Wegener's granulomatosis. A serum determination of cANCA supported the diagnosis of Wegener's granulomatosis. Clinical findings and image studies suggested an erroneous diagnosis of malignancy whereas a definitive diagnosis of Wegener's granulomatosis was achieved only after repeated biopsies thus leading to a correct therapeutic approach. The Wegener granulomatosis must be added to the list of the differential diagnoses of the masses of the nasopharynx associated with or without enlarged laterocervical nodes. PMID:24106630

  7. Wegener granulomatosis (granulomatosis with polyangiitis): evolving concepts in treatment.

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    Lynch, Joseph P; Tazelaar, Henry

    2011-06-01

    Wegener granulomatosis (WG), the most common of the pulmonary granulomatous vasculitides, typically involves the upper respiratory tract, lower respiratory tract (bronchi and lung), and kidney, with varying degrees of disseminated vasculitis. THE TERM GRANULOMATOSIS WITH POLYANGIITIS (WEGENER) WAS RECENTLY PROPOSED TO REPLACE THE OLDER TERM, WG. THE TERM GRANULOMATOSIS WITH POLYANGIITIS CAN BE ABBREVIATED TO GPA, WITH THE IDEA THAT THE EPONYM WEGENER WOULD BE OMITTED OVER TIME. Cardinal histologic features include a necrotizing vasculitis involving small vessels, extensive "geographic" necrosis, and granulomatous inflammation. Clinical manifestations of WG are protean; virtually any organ can be involved. The spectrum and severity of the disease are heterogeneous, ranging from indolent disease involving only one site to fulminant, multiorgan vasculitis. The pathogenesis of WG has not been elucidated, but both cellular and humoral components are involved. Circulating antibodies against cytoplasmic components of neutrophils [anti-neutrophil cytoplasmic antibodies (c-ANCAs)] likely play a role in the pathogenesis, and often correlate with activity of the disease. Treatment strategies are evolving. Cyclophosphamide (CYC) plus corticosteroids (CSs) is the mainstay of therapy for generalized, multisystemic WG. Historically, the combination of CYC plus CS was used for a minimum of 12 months, but concern about late toxicities associated with CYC has led to novel treatment approaches. Currently, short-course (3 to 6 months) induction treatment with CYC plus CS, followed by maintenance therapy with less toxic agents (e.g., methotrexate, azathioprine) is recommended. Further, methotrexate combined with CS may be adequate for limited, non-life-threatening WG. Recent studies suggest that rituximab may be useful for induction therapy or CYC-refractory WG. The role of other immunomodulatory agents (including trimethoprim-sulfamethoxazole) is also explored. © Thieme Medical

  8. Orbital granulomatosis with polyangiitis (Wegener granulomatosis): clinical and pathologic findings.

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    Muller, Karra; Lin, Jonathan H

    2014-08-01

    The pathology of granulomatosis with polyangiitis (GPA), formerly Wegener granulomatosis, typically features a granulomatous and sometimes necrotizing vasculitis targeting the respiratory tract and kidneys. However, orbital involvement occurs in up to 60% of patients and is frequently the first or only clinical presentation in patients with systemic or limited forms of GPA. Orbital GPA can cause significant morbidity and potentially lead to complete loss of vision and permanent facial deformity. Fortunately, GPA is highly responsive to medical treatment with corticosteroids combined with cyclophosphamide or, more recently, rituximab. Therefore, it is imperative for this disease to be accurately diagnosed on orbital biopsy and distinguished from other histologically similar orbital lesions. Herein, we review the clinical and pathologic findings of orbital GPA, focusing on the differentiation of this disease from other inflammatory orbital lesions.

  9. Massive intracerebral hemorrhage associated with Wegener granulomatosis.

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    Ceri, Mevlut; Ortabozkoyun, Levent; Unverdi, Selman; Kirac, Mustafa; Duranay, Murat

    2012-06-01

    Wegener granulomatosis (WG) is a necrotizing granulomatous vasculitis that predominantly affects airways and kidneys. But central nervous system involvement (7-11%) is an uncommon. Massive ICH may occur in the course of WG, and this serious condition is related with high risk of mortality. Therefore, the new treatment strategies may be considered in addition to classical practices in serious organ involvement and recurrent attack. Here, we present an adult patient with WG whose disease was complicated by a massive intracerebral hemorrhage (ICH), which subsequently led to death.

  10. Mucocutaneous leishmaniasis masquerading as Wegener granulomatosis.

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    Brahn, Ernest; Pegues, David A; Yao, Qingping; Craft, Noah

    2010-04-01

    A 43-year-old Brazilian female presented in 2001 with nasal stuffiness and sinusitis. A biopsy was consistent with limited Wegener's granulomatosis although antineutrophil cytoplasmic antibodies were negative. Her nasal inflammation progressed despite trials of prednisone, methotrexate, and azathioprine. A septal perforation developed and a repeat biopsy showed granulomatous inflammation. In 2006 the patient was referred to Division of Rheumatology, University of California, Los Angeles. The nose was grossly erythematous and a magnetic resonance imaging revealed nasal destruction and sinusitis. Palatine biopsies showed chronic inflammation. Cyclophosphamide at 150 mg/d resulted in markedly improved mucocutaneous lesions. The patient developed a leg and arm rash in 2007. A skin biopsy was positive for Leishmania braziliensis. The cyclophosphamide was discontinued and amphotericin B was initiated with transient benefit. Remission was achieved with pentavalent antimony. Despite multiple nasopharyngeal biopsies, for a 6-year span, mucocutaneous leishmaniasis masqueraded as Wegener's granulomatosis. Cyclophosphamide not only resulted in clinical improvement, due to reduced inflammatory response, but also allowed widespread cutaneous dissemination.

  11. Neutrophil activation in vitro and in vivo in Wegener's granulomatosis

    NARCIS (Netherlands)

    BROUWER, E; HUITEMA, MG; Mulder, AHL; HEERINGA, P; VANGOOR, H; TERVAERT, JWC; Weening, Jan; KALLENBERG, CGM

    The mechanisms underlying glomerular capillary wall injury in Wegener's granulomatosis (WG) are not well understood. Anti-neutrophil cytoplasmic antibodies (ANCA), present in sera from patients with WG, are known to stimulate respiratory burst and degranulation of primed polymorphonuclear

  12. A rare presentation of renal Wegener granulomatosis in a child.

    Science.gov (United States)

    D'Hauwe, Riet; Lerut, Evelyne; Breysem, Luc; Smet, Marleen

    2011-09-01

    We present a rare case of Wegener granulomatosis of the kidney in childhood. The diagnosis was suggested on imaging due to a combination of clinical symptoms, the lack of improvement with antibiotic therapy, and the absence of signs of inflammation on renal imaging. It was confirmed by histological examination following biopsy. The features and appearance of renal Wegener granulomatosis are described, and the differential diagnosis for a childhood renal mass is discussed.

  13. Bronchoplastic procedure for an unusual indication--Wegener's granulomatosis.

    LENUS (Irish Health Repository)

    Soo, Alan

    2009-09-01

    Wegener\\'s granulomatosis (WG) is a systemic vasculitic condition that commonly affects the lung and kidneys. With improvement in medical therapy, airway complications are increasingly encountered and are difficult to manage. Here, we present a case whereby a patient presenting with airway complication is successfully treated with surgery.

  14. [Granulomatosis with polyangiitis (previously Wegener's granulomatosis) mimicking malingering].

    Science.gov (United States)

    Ene, N; Bélénotti, P; Benyamine, A; Sovaila, S; Ben Sahla Talet, M H; Kaminsky, P; Serratrice, J; Weiller, P-J

    2014-08-01

    ANCA vasculitis may involve the skin and develop slowly without specific histology, and without autoantibodies. We report a 50-year-old woman who experienced bilateral mastectomy because of ulcero-necrotic, non-specific inflammatory cutaneous lesions of the breasts. First considered by others as a malinger patient, she developed oto-neurological lesions leading to the diagnosis of Wegener's granulomatosis. Five years later, specific antibodies of the disease were present. Cutaneous involvement by ANCA vasculitis can be isolated for a long time. Physicians must have a high degree of suspicion to avoid diagnostic delay of ANCA vasculitis. Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  15. Wegener Granulomatosis Revealed by Pleural Effusion

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    Toffart, Anne-Claire; Arbib, François; Lantuejoul, Sylvie; Roux, Jean-François; Bland, Vincent; Ferretti, Gilbert; Diab, Samia

    2009-01-01

    Pulmonary signs are common in Wegener's granulomatosis (WG). However, an initial presentation including pleural effusion has not been described. We describe a case of WG in which pleural effusion was the first clinical manifestation. A 45-year-old man with dorsal pain presented with pleural thickening and effusion, and a visible nodule on a thoracic scan. A dense chronic inflammatory infiltrate was obtained by pleural biopsy and an open lung biopsy revealed necrotizing granulomatous vasculitis. Serologies were positive for antineutrophil cytoplasmic antibodies and antiproteinase 3 antibodies. A diagnosis of WG was conducted and the patient was started on cyclophosphamide and methylprednisolone as an initial treatment, with a favorable evolution. Although pleural effusion is rarely described in WG, this pathology must be considered in the presence of this clinical manifestation. PMID:20168982

  16. Marathon of eponyms: 23 Wegener granulomatosis.

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    Scully, C; Langdon, J; Evans, J

    2012-03-01

    The use of eponyms has long been contentious, but many remain in common use, as discussed elsewhere (Editorial: Oral Diseases. 2009: 15; 185). The use of eponyms in diseases of the head and neck is found mainly in specialties dealing with medically compromised individuals (paediatric dentistry, special care dentistry, oral and maxillofacial medicine, oral and maxillofacial pathology and oral, oral and maxillofacial radiology and maxillofacial surgery) and particularly by hospital-centred practitioners. This series has selected some of the more recognized relevant eponymous conditions and presents them alphabetically. The information is based largely on data available from MEDLINE and a number of internet websites as noted below: the authors would welcome any corrections. This document summarizes data about Wegener granulomatosis. © 2012 John Wiley & Sons A/S.

  17. Wegener Granulomatosis Revealed by Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Anne-Claire Toffart

    2009-01-01

    Full Text Available Pulmonary signs are common in Wegener's granulomatosis (WG. However, an initial presentation including pleural effusion has not been described. We describe a case of WG in which pleural effusion was the first clinical manifestation. A 45-year-old man with dorsal pain presented with pleural thickening and effusion, and a visible nodule on a thoracic scan. A dense chronic inflammatory infiltrate was obtained by pleural biopsy and an open lung biopsy revealed necrotizing granulomatous vasculitis. Serologies were positive for antineutrophil cytoplasmic antibodies and antiproteinase 3 antibodies. A diagnosis of WG was conducted and the patient was started on cyclophosphamide and methylprednisolone as an initial treatment, with a favorable evolution. Although pleural effusion is rarely described in WG, this pathology must be considered in the presence of this clinical manifestation.

  18. Fulminant Wegener's granulomatosis: a case report.

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    Dinić, Miroslav Z; Sekulović, Lidija Kandolf; Zolotarevski, Lidija; Zecević, Rados D

    2013-09-01

    Granulomatosis Wegener is anti-neutrophil cytoplasmic antibodies (ANCAs)-associated systemic vasculitis of unknown etiology. It is manifested as granulomatous necrotizing inflammation of the upper and lower parts of the respiratory tract, glomerulonephritis and systemic vasculitis involving most frequently the skin and oral mucous membrane. Sera markers of this disease are c-ANCA and pANCA. We presented a female patient aged 52 years with purpuric spots that had appeared on the lower legs ten months before admission to our hospital. The disease ran an aggressive course, and a month before admission hemorrhagic bullae, skin ulcers, hoarseness, dyspnea, generalized arthralgia, fatigue and fever had rapidly developed. Histopathological examination of a skin sample revealed necrotizing vasculitis, so that sera markers concentrations were elevated (c-ANCA, p-ANCA). There was a perforation of the nasal septum found on rhinoscopy. During hospitalization acute abdominal pain occurred, a possible tumor in the small intestine and possible granulomas in the liver were seen by multislice computed tomography (MSCT) examination, with normal findings on the lungs and kidneys. The treatment started with methylprednisolone: 500 mg/d i.v. infusion for consecutive 3 days, then 60 mg/d. On exploratory laparotomy small bowel perforation and diffuse peritonitis were found. Unstable in the postoperative period, the patient died on the day 12 of hospitalization. The reported patient was with fulminant Wegener's granulomatosis, dominantly with skin changes and with gastrointestinal manifestation. This case accents the need for rapid systemic clinical evaluation in a severely ill patient with unclear diagnosis.

  19. Granulomatosis with polyangiitis (Wegener granulomatosis): A proteinase-3 driven disease?

    Science.gov (United States)

    Witko-Sarsat, Véronique; Thieblemont, Nathalie

    2018-03-01

    Granulomatosis with polyangiitis (GPA, Wegener granulomatosis) is a systemic autoimmune vasculitis that affects small arteries, arterioles, and capillaries, most notably in the kidneys and lungs. In this disease, proteinase-3 (PR3), produced by neutrophils, is targeted by antineutrophil cytoplasmic antibodies (ANCA). Recent work by our group has shown how PR3 impairs the resolution of inflammation and deregulates the immune system. Normally, the clearance of activated neutrophils triggers an anti-inflammatory, pro-resolution process. In patients with GPA, however, macrophages phagocytose apoptotic neutrophils then release massive amounts of pro-inflammatory mediators, notably interleukin-1, thereby generating a pro-inflammatory microenvironment conducive to autoimmunity. This deregulation of immune processes is accompanied with activation of plasmacytoid dendritic cells and with polarization of T-helper-2 (Th2), Th9, and Th17 cells. These recent data highlight the dual role of PR3, both auto-antigenic and auto-inflammatory, thus potentially opening up new therapeutic avenues. Copyright © 2017 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  20. Coexistent pulmonary granulomatosis with polyangiitis (Wegener granulomatosis) and Crohn disease.

    Science.gov (United States)

    Vaszar, Laszlo T; Orzechowski, Nicole M; Specks, Ulrich; Ytterberg, Steven R; Loftus, Edward V; Mark, Eugene J; Tazelaar, Henry D

    2014-03-01

    Crohn disease (CD) may be associated with various extraintestinal manifestations, including, rarely, respiratory tract involvement. When necrobiotic pulmonary nodules are present, the differential diagnosis includes granulomatosis with polyangiitis (Wegener granulomatosis) (GPA). The respiratory tract manifestations of CD and GPA may mimic each other, complicating the diagnosis and suggesting the possible coexistence of these 2 conditions. The aim of this study was to describe the clinical, radiographic, and pathologic features of patients in whom CD and GPA coexist. We reviewed the teaching files of the authors and searched the Mayo Clinic medical records for coexistent inflammatory bowel diseases and antineutrophilic cytoplasmic antibodies (ANCA)-associated vasculitides. We reviewed in detail 97 patient charts and excluded cases of ulcerative colitis and those in whom only one of the diagnoses was present. Pulmonary and gastrointestinal biopsies were reviewed when available. We also searched the medical literature for previously published cases. We found 6 cases of coexistent CD and pulmonary GPA and 4 cases with extrapulmonary GPA; 3 cases (all with extrapulmonary GPA) have been published previously. The diagnosis of CD preceded that of GPA in 11 cases. Proteinase 3-ANCA was positive in 6 cases, negative in 2, and not reported in 5 cases. Myeloperoxidase-ANCA was negative in 6 cases and unavailable in the remainder of patients. Pathology revealed features diagnostic of GPA in all cases with necrotizing granulomatous inflammation and segmental vasculitis. Pulmonary findings in patients with CD or the presence of granulomatous colitis in patients with GPA should prompt the inclusion in the differential diagnosis of a possible coexistence of CD and GPA.

  1. Fulminant Wegener's granulomatosis: A case report

    Directory of Open Access Journals (Sweden)

    Dinić Miroslav Ž.

    2013-01-01

    Full Text Available Introduction. Granulomatosis Wegener is anti-neutrophil cytoplasmic antibodies (ANCAs-associated systemic vasculitis of unknown etiology. It is manifested as granulomatous necrotizing inflammation of the upper and lower parts of the respiratory tract, glomerulonephritis and systemic vasculitis involving most frequently the skin and oral mucous membrane. Sera markers of this disease are c-ANCA and p-ANCA. Case report. We presented a female patient aged 52 years with purpuric spots that had appeared on the lower legs ten months before admission to our hospital. The disease ran an aggressive course, and a month before admission hemorrhagic bullae, skin ulcers, hoarseness, dyspnea, generalized arthralgia, fatigue and fever had rapidly developed. Histopathological examination of a skin sample revealed necrotizing vasculitis, so that sera markers concentrations were elevated (c-ANCA, p-ANCA. There was a perforation of the nasal septum found on rhinoscopy. During hospitalization acute abdominal pain occurred, a possible tumor in the small intestine and possible granulomas in the liver were seen by multislice computed tomography (MSCT examination, with normal findings on the lungs and kidneys. The treatment started with methylprednisolone: 500 mg/d i.v. infusion for consecutive 3 days, then 60 mg/d. On exploratory laparotomy small bowel perforation and diffuse peritonitis were found. Unstable in the postoperative period, the patient died on the day 12 of hospitalization. Conclusion. The reported patient was with fulminant Wegener’s granulomatosis, dominantly with skin changes and with gastrointestinal manifestation. This case accents the need for rapid systemic clinical evaluation in a severely ill patient with unclear diagnosis.

  2. MRI of the nasal cavity, the paranasal sinuses and orbits in Wegener`s granulomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Muhle, C. [Kiel Univ. (Germany). Dept. of Diagnostic Radiology; Reinhold-Keller, E. [Rheumaklinik Bad Bramstedt (Germany). Dept. of Medicine]|[Medical Univ. Luebeck (Germany). Dept. of Rheumatology; Richter, C. [Rheumaklinik Bad Bramstedt (Germany). Dept. of Medicine]|[Medical Univ. Luebeck (Germany). Dept. of Rheumatology; Duncker, G. [Kiel Univ. (Germany). Dept. of Ophthalmology; Beigel, A. [Dr. Horst-Schmidt-Kliniken, Wiesbaden (Germany). ENT Clinic; Brinkmann, G. [Kiel Univ. (Germany). Dept. of Diagnostic Radiology; Gross, W.L. [Rheumaklinik Bad Bramstedt (Germany). Dept. of Medicine]|[Medical Univ. Luebeck (Germany). Dept. of Rheumatology; Heller, M. [Kiel Univ. (Germany). Dept. of Diagnostic Radiology

    1997-05-01

    The purpose of this study was to evaluate diagnostic MRI criteria in Wegener`s granulomatosis of the nasal cavity, the paranasal sinuses and orbits. Between March 1991 and January 1996, 62 patients with biopsy-proven Wegener`s granulomatosis were studied with T1- and T2-weighted spin-echo (SE) sequences. In 32 patients coronal postcontrast T1-weighted images were obtained. Mucosal thickening of the nasal cavity and paranasal sinuses was demonstrated as high-intensity lesions on T2-weighted SE sequences in 57 patients (92%). Of this group, inflammatory granulomatous tissue was found on biopsy in 30 patients (48%) in the nasal cavity and in 4 patients (6%) in the paranasal sinuses. In 23 patients (37%) biopsy revealed unspecific inflammatory changes without evidence of granulomatous tissue. In 14 patients (23%) granulomas were depicted as low-signal intensity lesions on T1- and T2-weighted SE sequences in the paranasal sinuses and orbits. In 5 patients (8%) osseous destruction was found. After gadolinium injection, 12 of 14 granulomas showed inhomogeneous signal enhancement. In two granulomas no enhancement was found. The MRI technique is helpful in the diagnosis of patients with Wegener`s granulomatosis. In the initial inflammatory process of Wegener`s granulomatosis, it is not possible to differentiate between mucosal inflammation and granulomatous tissue in MRI. In the later stage of granulomatous transformation, granulomas can be depicted as low-signal-intensity lesions. Therefore, Wegener`s granulomatosis should be included in the differential diagnosis of patients with low-signal-intensity lesions on T1- and T2-weighted SE sequences of the nasal cavity, paranasal sinuses and orbits. (orig.)

  3. Granulomatosis with polyangiitis, a new nomenclature for Wegener's Granulomatosis - Case report*

    Science.gov (United States)

    de Lima, Alexandre Moretti; Torraca, Pedro de Freitas Silva; da Rocha, Sheila Pereira; Santiago, Carmelia Matos Reis; Ferraz, Fabio Humberto Ribeiro Paes

    2015-01-01

    The granulomatosis with polyangiitis, initially known as Wegener's granulomatosis, is a small and medium vessels vasculitis. It's classic form presents a triad: necrotizing granuloma of respiratory tract, necrotizing cutaneous vasculitis and glomerulonephritis. This vasculitis has cytoplasmic antineutrophil antibodies as signal. This work illustrates a case, of multisystemic rare disease, in which the segment and treatment were considered satisfactory for symptoms remission. PMID:26312687

  4. Wegener's granulomatosis in childhood

    Energy Technology Data Exchange (ETDEWEB)

    McHugh, K.; Manson, D. (Hospital for Sick Children, Toronto, ON (Canada). Dept. of Diagnostic Imaging); Eberhard, B.A.; Shore, A.; Laxer, R.M. (Hospital for Sick Children, Toronto, ON (Canada). Div. of Rheumatology)

    1991-12-01

    The authors reviewed the medical histories and radiological examinations of five pediatric patients with a histologic diagnosis of Wegener's granulomatosis (WG) seen over a six year period in whom a total of 22 thoracic CT scans were performed. Involvement of both the upper and lower respiratory tracts was seen in all patients at presentation. One patient had subglottic stenosis necessitating tracheotomy. Pulmonary hemorrhage occurred in three patients at initial diagnosis. Classic cavitary lung nodules were seen in two patients - one at initial presentation, the other at relapse. The plain radiographic lower respiratory tract manifestations of pediatric WG were protean both at initial presentation and during follow up. Similarly, disease expression was highly variable on thoracic CT examinations but, overall multifocal parenchymal infiltrates with or without small peripheral nodules were the commonest thoracic CT manifestations. As a consequence of cytotoxic and corticosteroid therapy the long-term prognosis of WG has improved considerably in recent years. Knowledge of the varied patterns of the primary disease and potential for iatrogenic complications are necessary for successful radiologic assessment of pediatric patients with WG. (orig.).

  5. Imaging diagnosis of orbital Wegener granulomatosis

    Science.gov (United States)

    Yang, Bin; Yin, Zhijian; Chen, Shuai; Yuan, Feng; Zhao, Wei; Yang, Yaying

    2017-01-01

    Abstract Introduction: Wegener granulomatosis (WG) is a rare idiopathic autoimmune disease causing necrotizing granulomatous vasculitis. Whether as the first symptom or as part of systemic changes, ocular manifestations in WG patients are not specific. Any part of the eyes can be affected, with the anterior segment and orbit most commonly involved. So, early diagnosis and treatment are essential for controlling the progression of the disease and improving the quality of life for patients. Clinical findings/Patient concerns: Here we present a rare case of orbital WG of a 22-year-old woman was admitted to the hospital because of intense pain associated with decreased visual acuity in her right eye since 1 day. She had been previously diagnosed with WG at our hospital. Imaging diagnosis: Orbital computed tomography imaging showed diffuse swelling of intraorbital muscles, and space-occupying lesions were present in both eyes. Most postnasal anatomical structures were absent, appearing as a massive cavity shadow. Orbital magnetic resonance imaging showed a shadow of orbital soft tissues. Conclusion: WG is a serious, fatal disease. Early diagnosis and treatment are essential for controlling the progression of the disease and improving the quality of life for patients. PMID:28591026

  6. Wegener granulomatosis causing compressive optic neuropathy in a child.

    Science.gov (United States)

    Aakalu, Vinay K; Ahmad, Amjad Z

    2009-01-01

    Wegener granulomatosis is an uncommon illness in children that is known to cause myriad ophthalmic complications, but it is rarely a cause of compressive optic neuropathy. A 17-year-old Hispanic boy with Wegener granulomatosis developed unilateral loss of vision, pain, and proptosis of the left eye. CT findings revealed enlargement of bilateral lacrimal glands with compression of the left optic nerve. The patient was admitted for high-dose intravenous corticosteroids and daily oral cyclophosphamide treatment. The patient's vision, pain, and proptosis improved dramatically, and he is now stable on mycophenolate mofetil and prednisone.

  7. [Wegener granulomatosis - otologic, nasal, tracheobronchial and pulmonary involvement].

    Science.gov (United States)

    Figueiredo, Sandra; Leal, Laurentino Mendes; Morais, António; Magalhães, Adriana; Oliveira, Teresa; Hespanhol, Venceslau; Dias, Carlos; Fernandes, Gabriela

    2009-01-01

    Wegener granulomatosis is a rare systemic idiopathic disease characterized by involvement of small vessels - medium and small arteries, venules, arterioles and ocasionally large arteries. This disease has predilection for the upper and lower respiratory tract and the kidney, with granulomatous inflamation and necrosis. Clinical manifestations and organ involvement of the disease vary widely. Early diagnosis and treatment may lead to a full recovery. Without treatment, Wegener's granulomatosis can be fatal. The authors present a case of a 33 year-old female, with severe disease, but with good outcome, after adequate diagnosis and treatment.

  8. Sinonasal Wegener's granulomatosis: CT characteristics

    Energy Technology Data Exchange (ETDEWEB)

    Benoudiba, F.; Marsot-Dupuch, K.; Rabia, Hadj M.; Lasjaunias, P. [Neuroradiology Department, Bicetre Hospital, 78 rue du General Leclerc, 94275 Le Kremlin Bicetre (France); Cabanne, J. [Department of Internal Medecine, Saint-Antoine Hospital, 184 rue du Faubourg Saint-Antoine, 75012 Paris (France); Bobin, S. [Head and Neck Department, Bicetre Hospital, 78, rue du General Leclerc, 94275 Le Kremlin Bicetre (France)

    2003-02-01

    Wegener's granulomatosis (WG) is a severe and potentially lethal granulomatosis. Even though no specific radiological criteria exist, CT may suggest the correct diagnosis at an early stage. Recent improvement in the prognosis is related to earlier diagnosis, allowing the initiation of efficient and specific treatment before any severe complications occur. We reviewed a series of WG cases in order to establish the CT diagnostic criteria. (orig.)

  9. MRI appearance of pulmonary Wegener`s granulomatosis with concomitant splenic infarction

    Energy Technology Data Exchange (ETDEWEB)

    Kalaitzoglou, I.; Drevelengas, A.; Palladas, P. [Department of Radiology, `G. Papanikolaou` General Hospital, Thessaloniki (Greece); Asimaki, A. [Department of Pathology, `G. Papanikolaou` General Hospital, Thessaloniki (Greece)

    1998-03-27

    The MRI appearance of pulmonary lesions in a patient with Wegener`s granulomatosis is presented. Pulmonary involvement was characterized by multiple solid and cavitated nodules connected to vessels. Transbronchial biopsy demonstrated typical findings of granuloma. The spleen presented diffuse low signal intensity and an enhancing capsule on T1-weighted images, and inhomogeneously high signal intensity on T2-weighted images. This appearance of diffuse infarction could result from the vasculitis caused by Wegener`s disease. (orig.) With 5 figs., 14 refs.

  10. Nomenclature and classification of vasculitis: lessons learned from granulomatosis with polyangiitis (Wegener's granulomatosis)

    Science.gov (United States)

    Jennette, J C

    2011-01-01

    Names influence how something is perceived. Diagnostic terms (diagnoses) are the names of diseases that are usually derived either from some distinctive characteristic of the disease or include an eponym recognizing someone who elucidated the disease. No matter how logical and appropriate a name may be, if it is not usable and used it is of no lasting value. This brief commentary focuses on the nomenclature of systemic vasculitides, and uses as a prime example Wegener's granulomatosis, which has been renamed recently ‘granulomatosis with polyangiitis’, in part because of concerns about the suitability of Friedrich Wegener as the source of an eponym. The most distinctive pathological feature of Wegener's granulomatosis is multi-focal necrotizing inflammation that has long been called granulomatosis. The systemic variant of Wegener's granulomatosis also is characterized by inflammation in many different vessels or different types, i.e. polyangiitis. Thus, granulomatosis with polyangiitis is a very appropriate alternative term for Wegener's granulomatosis. This term also is in accord with the name for a closely related vasculitis, i.e. microscopic polyangiitis. Terms that indicate aetiology and pathogenesis, when known, are useful to include in names for diseases (diagnoses). Anti-neutrophil cytoplasmic autoantibodies specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA) are implicated in the cause of granulomatosis with polyangiitis and thus also should be specified in the diagnosis (e.g. PR3-ANCA-positive granulomatosis with polyangiitis or MPO-ANCA-positive microscopic polyangiitis). As our understanding of the clinical manifestations, pathogenesis and aetiology of vasculitides change over time, the names and approaches for diagnosing these diseases will change accordingly. PMID:21447122

  11. Wegener granulomatosis-associated optic perineuritis.

    Science.gov (United States)

    Takazawa, Takanori; Ikeda, Ken; Nagaoka, Tetsuro; Hirayama, Takehisa; Yamamoto, Tatsuhiro; Yanagihashi, Masaru; Tochikubo, Tetsuo; Iwasaki, Yasuo

    2014-02-01

    INTRODUNCTION: We report two patients with optic perineuritis (OPN) and hypertrophic pachymeningitis in Wegener granulomatosis (WG). Patient 1: a 74-year-old man developed blurred vision in each eye, sequentially, over a year. In the first episode, visual acuity in the right eye was reduced to no light perception, and in the second episode, the vision in the left eye fell to 20/100. Brain and orbital magnetic resonance imaging (MRI) revealed abnormal enhancement in the meninges and the ipsilateral optic nerve sheath. T2-hyperintense lesions were found along the outer rim of the ipsilateral optic nerve. Seropositive proteinase-3-antineutrophil cytoplasmic antibody (PR3-ANCA), microhematuria and multiple pulmonary nodules suggested the diagnosis of WG. Steroid therapy was initiated 3 months after the first onset, but with no clinical response. At the 2nd episode, rapid administration of steroid ameliorated visual disturbance and MRI lesions markedly. Patient 2: a 72-year-old man developed blurred vision in each eye. Visual acuity measured no light perception in OD and 6/12 in OS. Gadolinium-enhanced MRI disclosed enhancement in the meninges and both optic nerve sheaths. T2-weighted imaging displayed hyperintense lesions along the outer rims of optic nerves. Otolaryngologic examination, seropositive PR3-ANCA and pulmonary nodules supported the diagnosis of WG. Steroid and cyclophosphamide treatment improved visual dysfunction and MRI lesions in the meninges and the optic nerve sheaths. The morphological similarity and the anatomical continuity between the meningeal and the perioptic tissues suggest that extension of granulomatous inflammation along such tissue planes accounted for visual loss in these two patients with WG.

  12. Tracheobronchial Stenoses in Granulomatosis With Polyangiitis (Wegener's)

    Science.gov (United States)

    Girard, Charlotte; Charles, Pierre; Terrier, Benjamin; Bussonne, Guillaume; Cohen, Pascal; Pagnoux, Christian; Cottin, Vincent; Cordier, Jean-François; Guillevin, Loïc

    2015-01-01

    Abstract Tracheobronchial stenoses (TBSs) are potentially severe manifestations of granulomatosis with polyangiitis (Wegener's) (GPA) that usually respond poorly to corticosteroids and immunosuppressive agents. We describe 26 GPA patients with ≥1 tracheal (mainly subglottic, SGS) and/or bronchial stenosis(ses) (BS(s)). Sixteen patients had solitary SGS and 10 had ≥1 BS(s). The male/female sex ratio was 9:17, and the median age at GPA diagnosis was 32 years (3:13 and 28 years, respectively, for SGS patients). Antineutrophil cytoplasm antibodies were proteinase 3-positive in 65.5% of the patients (50% of those with SGS). Despite conventional GPA therapy, 62% patients experienced ≥1 stenosis relapse(s) (81% of SGS patients, for a total of 1–8 relapses per patient). None of the several systemic or endoscopic treatments prevented future relapses. Cyclophosphamide induction therapy was effective in 4/6 patients with BS(s) and in 1 patient with SGS among the 7 treated. After many relapses, rituximab achieved remission in 3/4 SGS patients. Endoscopic treatments (dilation, laser, corticosteroid injection, etc.) had only transient efficacy. Other GPA manifestations relapsed independently of TBSs. One SGS patient died of acute respiratory distress syndrome. Our findings confirmed that TBSs are severe GPA manifestations that evolve independently of other organ involvements and do not respond to conventional systemic regimens. As previously described, our population was younger and comprised more females than usual GPA patients, especially those with SGS. The small number of patients and the wide variety of local and systemic treatments prevent us from drawing definitive conclusions about the contribution of each procedure. However, cyclophosphamide seemed to effectively treat BSs, but not SGS, and rituximab may be of interest for SGS management. PMID:26266344

  13. Treatment of orbital inflammation with rituximab in Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Baslund, Bo; Wiencke, Anne Katrine; Rasmussen, Niels

    2012-01-01

    OBJECTIVES: To study the efficacy of rituximab therapy for the treatment of orbital inflammation in patients with Wegener's granulomatosis (WG). METHODS: Ten WG patients with orbital inflammation were included in this case-series. None had symptoms suggestive of extra-orbital disease activity...

  14. Hepatic involvement in Wegener's granulomatosis: a case report

    Directory of Open Access Journals (Sweden)

    Trigidou Rodoula

    2010-01-01

    Full Text Available Abstract Introduction We report the case of a 58-year-old Caucasian Greek man who presented with dry cough, fever, bilateral alveolar infiltrates and acute hepatitis. Case presentation After a lung biopsy, the patient was diagnosed with Wegener's granulomatosis. The diagnosis was supported by the presence of anti-proteinase-3 anti-neutrophil cytoplasmic antibodies. A liver biopsy demonstrated the presence of mild non-specific lobular hepatitis and periodic acid-Schiff positive Lafora-like inclusions in a large number of his liver cells. The patient was treated with prednisone and cyclophosphamide, which was followed by subsequent remissions of chest X-ray findings and liver function studies. Conclusion What makes this case worth reporting is the coexistence of liver inflammation with a biochemical profile of severe anicteric non-viral, non-drug induced hepatitis coinciding with the diagnosis of Wegener's granulomatosis. Our paper may be the first report of hepatic involvement in a patient diagnosed with Wegener's granulomatosis. The aetiological link between the two diseases is supported by the reversion of hepatitis after the immunosuppression of Wegener's granulomatosis. We favor the hypothesis that hepatic vasculitis may be the cause of acute hepatocellular necrosis.

  15. Pulmonary Wegener`s granulomatosis: changes at follow-up CT

    Energy Technology Data Exchange (ETDEWEB)

    Attali, P.; Begum, R.; Ben Romdhane, H.; Brauner, M.W. [Department of Radiology, Univ. Paris XIII (France); Valeyre, D. [Department of Pneumology, Univ. Paris XIII (France); Guillevin, L. [Department of Internal Medicine, Univ. Paris XIII (France)

    1998-07-01

    The objective of this study was to assess the reversibility of pulmonary lesions in Wegener`s granulomatosis using serial CT. We reviewed the follow-up CT scans of ten treated patients with confirmed Wegener`s granulomatosis. The delay between the first evaluation before treatment and the second, on patients in clinical and biological remission, ranged from 6 to 54 months (mean 20.5 months). Follow-up CT showed a decrease in the extent of disease in all cases. Lesions disappeared completely, without scarring, in 4 of 4 ground-glass opacities, 25 of 36 nodules, and 4 of 9 pulmonary consolidations; they disappeared with residual scarring in 8 of 8 masses, 3 of 9 pulmonary consolidations, and 2 of 36 nodules. The majority of lesions disappear without scarring. Residual fibrosis may follow the occurence of masses and pulmonary consolidation. Computed tomography permits assessment of cicatricial lesions. (orig.) With 4 figs., 1 tab., 11 refs.

  16. Analysis of a cluster of cases of Wegener granulomatosis.

    Science.gov (United States)

    Albert, Daniel A; Albert, Alexis N; Vernace, Melchiore; Sebastian, Jodi K; Hsia, Elizabeth C

    2005-08-01

    Wegener granulomatosis is a chronic inflammatory autoimmune disease of unknown etiology. The sporadic occurrence, lack of familial or genetic associations, and rising incidence suggest possible exposure to environmental agents as causative for this disease. The objective of this study was to examine possible environmental triggers of Wegener granulomatosis. While conducting an environmental survey of potential precipitants of Wegener granulomatosis on a cohort of patients seen at Doylestown Hospital and at the University of Pennsylvania, we identified a cluster of cases in the Dublin, Pennsylvania, region. Through hospital records and patient contacts, we located 7 cases diagnosed in a 3-year period within a 10-mile radius of an Environmental Protection Agency (EPA) Superfund toxic waste site. The radius of inclusion represents a population of approximately 50,000 individuals. Assuming complete ascertainment of cases--which is unlikely given the methods used to acquire patients--the prevalence is 2- to 4-fold greater than the expected rate of 3 per 100,000. We identified toxins at or above "action level" within the demarcated geographic region using published data from the EPA. Furthermore, we queried patients regarding their particular chemical exposures. These patients with Wegener granulomatosis were possibly exposed to high levels of trichloroethylene (TCE), vinyl chloride, methyl tertiary-butyl ether (MTBE), dichloroethene (DCE), and chromic acid from several industrial waste sites within the area. Additionally, these patients reported a total of greater than 30 possible exposures, including the aforesaid chemical contaminants. Three of 5 patients whose water source is known had well water that exposed them to industrial runoff and necessitated EPA intervention. This data, along with other epidemiologic studies, suggest possible toxic exposures as potentially correctable risk factors for Wegener granulomatosis. We encourage clinicians to seek data that

  17. An atypical case of Wegener`s granulomatosis complicated by sepsis and coxitis; Ziarniniakowatosc Wegenera o nietypowej lokalizacji powiklana posocznica i ropnym zapaleniem stawu biodrowego

    Energy Technology Data Exchange (ETDEWEB)

    Makowski, A.; Faflik, J. [Centrum Zdrowia Matki Polki, Lodz (Poland)

    1993-12-31

    There is presented an atypical case Wegener`s granulomatosis of maxillo-nasal region without bony destruction. The case is unusual because of sepsis and purulent coxitis. The patient responded well to treatment with vincristine and cyclophosphamide. ANCA (anti-neutrophil cytoplasmic antibodies) assays have very good sensitivity and specificity for Wegener`s granulomatosis. (author). 17 refs, 2 figs.

  18. Diabetes insipidus as a complication of Wegener's granulomatosis and its treatment with biologic agents.

    Science.gov (United States)

    Cunnington, Joanna Rosalind; Jois, Ramesh; Zammit, Ivan; Scott, David; Isaacs, John

    2009-01-01

    Wegener's granulomatosis of the pituitary gland resulting in diabetes insipidus is a rare complication of the disease. Standard treatment for Wegener's granulomatosis involves a combination of prednisolone and cylophosphamide, however biologic agents are now being used in refractory cases. We report three cases of patients with diabetes insipidus as a complication of Wegener's granulomatosis who were treated with biologic agents. All three cases showed clinical response to treatment with biologic agents including rituximab and alemtuzumab and two cases demonstrated improvement in pituitary gland abnormalities by MRI. Clinicians should be aware that diabetes insipidus can present as a complication of Wegener's granulomatosis and that biologic therapies may be effective in refractory cases.

  19. Granulomatose de Wegener: relato de caso Wegener's granulomatosis: a case report

    Directory of Open Access Journals (Sweden)

    Carlos E. B. Rezende

    2003-03-01

    Full Text Available A Granulomatose de Wegener é uma doença sistêmica, idiopática e rara, com acometimento das pequenas artérias dos tratos respiratórios superior e inferior e rins, levando à necrose e granuloma estes órgãos. Neste trabalho relatamos e discutimos o caso de uma adolescente, do sexo feminino, de apresentação inicial atípica e com evolução favorável após o diagnóstico precoce e tratamento adequados.Wegener's Granulomatosis is a rare systemic idiophatic disease. This disease has predilection for both the upper and lower respiratory tract and the kidney. In this paper we describe and discuss a case of Wegener's Granulomatosis, with atypical manifestations and favourable evolution after adequate diagnoses and treatment.

  20. Development of granulomatosis with polyangiitis (Wegener): listen to the patient.

    Science.gov (United States)

    Kandiah, David A

    2011-08-01

    Patients with rare or complex conditions may sometimes come up with the right diagnosis because of their previous exposure to the condition. This may be due to their knowledge of their family history or even concerns based on their exposure to information from electronic or paper repositories. In this case report, a 40-year-old woman who already had 1 rare condition of lymphangioleiomyomatosis developed breast "abscesses" that did not respond to traditional surgical debridement and antibiotics. She wondered if the lesions could be related to granulomatosis with polyangiitis (Wegener) as she had a strong family history of this condition. Subsequent investigations confirmed the diagnosis. Her response to treatment was complete. Breast lesions in granulomatosis with polyangiitis (Wegener) have been infrequently reported in the literature but should be considered in poorly healing lesions, despite the absence of other more typical symptoms at the time of presentation.

  1. [Recurrent pericarditis as an initial manifestation of Wegener's granulomatosis].

    Science.gov (United States)

    Leroy, S; Gaudebout, N; Lanteme, P; Seve, P

    2014-02-01

    Recurrent pericarditis occur in around a quarter of patients after a first episode of acute pericarditis. Most of the cases are idiopathic or viral pericarditis or post-pericardial injury syndromes. Recurrent pericarditis are most likely to occur in patients with known systemic lupus erythematosus or rheumatoid arthritis but are rare in other systemic auto-immune diseases. We report here an unusual case of a patient with a 5-year history of four acute myopericarditis revealing Wegener's granulomatosis. Clinicians should consider the possibility of Wegener's granulomatosis in case of recurrent pericarditis and look for features suggestive of granulomatous disease affecting the upper and lower respiratory tract. In this setting, antineutrophil cytoplasmic autoantibodies (ANCA) testing and/or biopsy of involved organs appear of particular interest to confirm the diagnosis. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

  2. Etanercept-induced Wegener granulomatosis in a patient with rheumatoid arthritis.

    Science.gov (United States)

    Broshtilova, Valentina; Iliev, Emil; Gantcheva, Mary

    2013-01-01

    A very rare case of etanercept-induced Wegener's granulomatosis in a patient with long-standing rheumatoid arthritis is reported. A thorough critical analysis on Wegener's granulomatosis pathogenetic mechanisms is done. The peculiarities of etanercept pharmacodynamic features are also presented together with some suggestions of possible induction pathways. © 2013 Wiley Periodicals, Inc.

  3. Malignancies in Wegener's granulomatosis: incidence and relation to cyclophosphamide therapy in a cohort of 293 patients

    DEFF Research Database (Denmark)

    Faurschou, M.; Sorensen, I.J.; Mellemkjaer, L.

    2008-01-01

    To describe the incidence of malignancies in a cohort of Danish patients with Wegener's granulomatosis (WG) and to investigate the cancer risk associated with cyclophosphamide (CYC) -therapy in WG.......To describe the incidence of malignancies in a cohort of Danish patients with Wegener's granulomatosis (WG) and to investigate the cancer risk associated with cyclophosphamide (CYC) -therapy in WG....

  4. An unusual orbital localization of wegener granulomatosis detected by 18F-FDG PET/CT.

    Science.gov (United States)

    Bertagna, Francesco; Treglia, Giorgio; Rossini, Pierluigi; Giubbini, Raffaele

    2014-08-01

    We report a case of a 61-year-old male patient affected by Wegener granulomatosis and left exophthalmos who underwent F-FDG PET/CT during follow-up for rectal cancer. The study was negative for secondary lesions but revealed high uptake at the left orbital mass consistent with Wegener granulomatosis.

  5. Granulomatosis with Polyangiitis (Wegener Granulomatosis) with Unusual Presentation.

    Science.gov (United States)

    Mannan, M A; Pasha, K; Nahar, S; Begum, K

    2017-01-01

    We came across a 32 years old male admitted in our hospital with prolonged low-grade fever, haemoptysis, leg swelling, weight loss, purpuric rashes and malaena. He received anti-TB treatment at another hospital without any improvement. He was pale with bullous and purpuric lesions over legs and feet. He also had features of consolidation over both lung fields. His CRP was 312mg/L, Urine R/M/E showed 40-50 RBC/HPF, Chest X-ray showed features of bilateral consolidation, c-ANCA-10U/L (positive); Tracheal aspirate for AFB was found to be negative. We diagnosed the case as Granulomatosis with Polyangitis (WG) and started treatment with steroid and cyclophosphamide. But the patient could not be saved probably due to delay in starting management.

  6. Pulmonary manifestations of wegener granulomatosis: CT findings in 57 patients and a review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Lohrmann, Christian; Uhl, Markus; Kotter, Elmar; Burger, Dieter; Ghanem, Nadir; Langer, Mathias

    2005-03-01

    Wegener granulomatosis is a multisystem disease of unknown cause characterized by a necrotizing granulomatous vasculitis. In comparison to other vasculitides, the lung is the most common organ involved in wegener granulomatosis presenting with a very aggressive airways pathology and chronic relapsing course. Chest radiographs fail to describe the pattern and distribution of thoracic pathology sufficiently, and CT has shown to be more sensitive for detecting lung involvement. We present the CT findings of 57 patients with wegener granulomatosis and a review of the literature.

  7. Granulomatosis with polyangiitis (Wegener's granulomatosis) causing atlantoaxial instability: a case report.

    Science.gov (United States)

    Mohapatra, Anand; Khan, Taleef; Diaz, Jason; Brasington, Richard; Zebala, Lukas P

    2016-10-01

    No previous cases of atlantoaxial instability due to granulomatosis with polyangiitis have been reported. The aim of this study was to report a case of granulomatosis with polyangiitis causing atlantoaxial instability. This is a case report. A 45-year-old woman participated in this study. The patient's pain and atlantoaxial instability were resolved. A 45-year-old Caucasian woman with a large ulcerative lesion in her oropharynx initially presented with chronic sinusitis, pharyngitis, and severe odynophagia. Years after her original symptoms began, she developed neck pain radiating into her upper trapezial region and shoulders. Atlantoaxial fusion was performed on the patient, resolving her neck, upper trapezial, and shoulder pain. She was diagnosed with granulomatosis with polyangiitis (formerly Wegener's granulomatosis) and treated with cyclophosphamide. Granulomatosis with polyangiitis should be part of the working differential diagnosis for non-traumatic cervical spine injury. The atlantoaxial instability can be managed with stabilization, and the disease process itself can be treated with cyclophosphamide. Copyright © 2016 Elsevier Inc. All rights reserved.

  8. Cardiopatía valvular en un paciente con granulomatosis de Wegener Valvular cardiopathy in a patient with Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    Luis E Silva

    2012-01-01

    Full Text Available La granulomatosis de Wegener es una vasculitis necrotizante autoinmune cuya prevalencia reportada es de 3/100.000 habitantes. Tiene compromiso multisistémico, principalmente el tracto respiratorio superior e inferior, y el sistema nervioso central y renal. La frecuencia del compromiso cardiaco varía según las series estudiadas, pero oscila alrededor de 6%; sin embargo, las manifestaciones clínicas son poco frecuentes. El compromiso valvular se reporta como insuficiencia aórtica o mitral, secundaria a infiltración de las valvas o dilatación de la raíz aórtica. El tratamiento se basa en el control de la enfermedad, el manejo de la falla cardiaca y la intervención quirúrgica de la válvula comprometida según la indicación.Wegener's granulomatosis is an autoimmune necrotizing vasculitis with a reported prevalence of 3/100.000 inhabitants. It is a multisystemic disease, involving mainly the upper and lower respiratory tract, the central nervous system and the kidneys. The frequency of cardiac involvement varies depending on the series studied, but oscillates around 6%; however, the clinical manifestations are rare. The valvular involvement is reported as aortic or mitral insufficiency secondary to infiltration of the leaflets, or as aortic root dilatation. Treatment is based on the control of the disease, the management of heart failure and surgical intervention of the involved valve, as directed.

  9. A disease activity score for ENT involvement in granulomatosis with polyangiitis (Wegener's)

    DEFF Research Database (Denmark)

    Del Pero, Marcos Martinez; Chaudhry, Afzal; Rasmussen, Niels

    2013-01-01

    Accurate assessment of disease activity in patients with otorhinolaryngological manifestations of granulomatosis with polyangiitis (Wegener's) (ENT/GPA) is necessary for treatment decisions and clinical trials. We have designed a disease activity score (ENT/GPA DAS) for this purpose....

  10. [Wegener's granulomatosis with anti-neutrophil cytoplasmic antibodies against anti-cathepsin G antigen].

    Science.gov (United States)

    Ocaña Pérez, E; Peña Casas, A M; del Campo Muñoz, T; Avila Casas, A; Luque Barona, R

    2013-12-01

    Wegener's granulomatosis belongs to the group of small vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies characterized by granulomatous inflammation and necrotising vasculitis in various organs with particular involvement of the upper and lower respiratory tracts and kidneys. Wegener's granulomatosis is a rare disorder in childhood and early diagnosis of this disease is critical to the long-term prognosis of the disease. The presence of positive cytoplasmic antineutrophil cytoplasmic antibody staining or a high titre of proteinase 3 antibodies were added as new criteria of vasculitis in childhood. This article presents a case of Wegener's granulomatosis, with the presence of anti-neutrophil cytoplasm antibodies with cytoplasmic pattern with absence of anti-proteinase 3 antibodies and presence of high levels of anti-cathepsin G antibodies, rarely described in Wegener's granulomatosis. Copyright © 2012 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  11. Total nasal skeletal reconstruction disfigured by granulomatosis with polyangitis (wegener granulomatosis).

    Science.gov (United States)

    Hafezi, Farhad; Naghibzadeh, Bijan; Ashtiani, Abbas Kazemi; Nouhi, Amir Hossein; Naghibzadeh, Ghazal

    2015-02-01

    Nasal deformity is a common disorder in Wegener granulomatosis, which is produced by polyangitis of medium-sized vessels. This process may cause necrosis and destruction of the nasal mucosa and osteocartilaginous framework. At this time, there is a lack of published data on nasal deformities associated with Wegener granulomatosis. We present a 53-year-old woman with completely necrotized nasal lining and osteocartilage. The patient had a history of granulomatosis with polyangitis that was currently in remission. We reconstructed the patient's nose with fifth and sixth rib cartilages over 3 successive surgeries over a 2-year period. During the 3-year follow-up period, neither infection nor significant cartilage graft resorption was observed, and the aesthetic result was acceptable both to the patient and her physicians. In spite of contamination to the nasal lining and immune-compromising medications, nasal reconstruction is usually successful when considering these factors. Access to strong and abundant fifth or sixth rib cartilage presents an opportunity to reconstruct nasal destruction due to iatrogenic or autoimmune processes.

  12. Impaired health-related quality of life in patients treated for Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Sigaard, Lene; Bjørner, Jakob Bue

    2010-01-01

    To investigate whether patients with Wegener's granulomatosis (WG) experience reduced health-related quality of life (HRQOL) after accomplishment of remission, and to study the influence of WG-associated organ damage on HRQOL.......To investigate whether patients with Wegener's granulomatosis (WG) experience reduced health-related quality of life (HRQOL) after accomplishment of remission, and to study the influence of WG-associated organ damage on HRQOL....

  13. Current and future prospects in the management of granulomatosis with polyangiitis (Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    Tarzi RM

    2014-04-01

    Full Text Available Ruth M Tarzi, Charles D PuseyRenal and Vascular Inflammation Section, Department of Medicine, Imperial College, London, UKAbstract: Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis is a multisystem autoimmune condition associated with anti-neutrophil cytoplasm antibodies. Management of GPA can be complex, owing to the sometimes fulminant and multisystem nature of the presentation, the age demographics of the affected population, and a significant incidence of disease relapse. In this paper, we discuss how some of the challenges in the management of GPA have been and continue to be addressed including: reducing the toxicity of induction therapy; developing biomarkers to determine who can safely stop maintenance immunosuppression; improving the efficacy of maintenance therapy for relapsing patients; managing localized disease; and management of disease and treatment-associated comorbidity. Consideration is also given to emerging therapeutics in the treatment of GPA.Keywords: anti-neutrophil cytoplasm antibody, vasculitis, immunosuppression, biologics

  14. Wegener's granulomatosis: chest computed tomography findings; Granulomatose de Wegener: aspectos na tomografia computadorizada de torax

    Energy Technology Data Exchange (ETDEWEB)

    Monteiro, Evelise de Azevedo; Marchiori, Edson; Martins, Erick Malheiro Leoncio [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Radiologia]. E-mail: edmarchiori@zipmail.com.br; Souza Junior, Arthur Soares [Faculdade de Medicina de Sao Jose do Rio Preto (FAMERP), SP (Brazil). Hospital de Base; Cerqueira, Elza Maria F.P. de [Universidade Estadual de Campinas, SP (Brazil). Faculdade de Ciencias Medicas. Dept. de Radiologia; Irion, Klaus L. [Pavilhao Pereira Filho, Porto Alegre, RS (Brazil); Araujo Neto, Cesar de [Bahia Univ., Salvador, BA (Brazil). Dept. de Radiologia; Souza, Rodrigo Azeredo de [Universidade Federal, Rio de Janeiro, RJ (Brazil). Faculdade de Medicina. Dept. de Radiologia

    2003-03-01

    Wegeners granulomatosis is an inflammatory systemic necrotizing idiopathic vasculitis affecting mainly small vessels. In this paper we analyzed the computed tomography findings of seven patients with Wegeners granulomatosis. The most common findings were masses and nodules (71.4%), areas of ground glass attenuation (57.1%), consolidation (42.8%), halo sign (42.8%), cavitation (42.8%), interlobular septa thickening (14.2%), bronchial wall thickening (14.2%) and pleural effusion (14.2%). Although radiological findings are mostly nonspecific, computed tomography and particularly high-resolution computed tomography are important tools in the evaluation of disease activity when evaluated in association with bronchoscopy findings and clinical data. (author)

  15. Wegener granulomatosis as an uncommon cause of panhypopituitarism in childhood.

    Science.gov (United States)

    Kara, Ozlem; Demirel, Fatma; Acar, Banu Celikel; Cakar, Nilgün

    2013-01-01

    Wegener granulomatosis (WG) is a cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA)-associated, multi-system, necrotizing granulomatous vasculitis. Inflammation of the nasal or oral mucosa, and lung and kidney involvements are typical in the course of the disease. In rare cases, pituitary involvement may occur and cause panhypopituitarism. Pituitary involvement is very rare, and only two pediatric case reports have been published to date out of a total of 24 cases. This is a case report of an adolescent patient who presented with panhypopituitarism symptoms and was later diagnosed with WG. A 16-year-old female patient complained of fever, headache, purulent nasal discharge and severe muscle and joint pain. Additionally, she had polyuria and polydipsia. Investigations revealed a pituitary mass and panhypopituitarism. Positivity of c-ANCA and renal biopsy result compatible with WG confirmed the diagnosis.

  16. Wegener granulomatosis presenting as refractory otitis media: a case report.

    Science.gov (United States)

    Mercan, Gül Caner; Mercan, Bahattin; Cukurova, Ibrahim

    2012-01-01

    Wegener's granulomatosis (WG) is a granulomatous widespread necrotizing vasculitis, sometimes progressing rapidly, characterized by a tendency to involve the upper and lower respiratory tracts, and in most cases, the kidneys. Otologic manifestations may be seen during the course of the disease, however, they are not frequently seen as the first and only presenting feature. The presence of anticytoplasmic antibodies versus neutrophil polymorphonucleate granules (c-ANCA) is highly specific for the diagnosis of WG, being positive in 97% of the cases. The early diagnosis and the timely medical treatment result in high rates of remission of this potentially lethal disease. In this article, we report a case of WG presenting with acute serous otitis media, progressing to suppurative otitis media and granulomas as the first and only symptom, which was refractory to medical therapy during follow-up and which was diagnosed with further examination.

  17. Treatment of orbital inflammation with rituximab in Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Baslund, Bo; Wiencke, Anne Katrine; Rasmussen, Niels

    2012-01-01

    OBJECTIVES: To study the efficacy of rituximab therapy for the treatment of orbital inflammation in patients with Wegener's granulomatosis (WG). METHODS: Ten WG patients with orbital inflammation were included in this case-series. None had symptoms suggestive of extra-orbital disease activity...... inflammation. All patients were treated with 1000 mg of rituximab administered twice with an interval of 14 days between the infusions. Six months after therapy, a physical examination and a control computerised tomography (CT) scan was performed. RESULTS: All patients had orbital inflammation demonstrated...... the size of the orbital mass was unchanged in eight patients. CONCLUSIONS: Rituximab therapy has positive effects on symptoms, visual acuity and/or granuloma size in some WG patients with orbital inflammation. Treatment with rituximab should be considered in WG patients with this serious manifestation...

  18. [Wegener granulomatosis and aneurysmal subarachnoid hemorrhage: an insignificant association?].

    Science.gov (United States)

    Marnet, D; Ginguené, C; Marcos, A; Cahen, R; Mac Gregor, B; Turjman, F; Vallée, B

    2010-08-01

    Wegener granulomatosis (WG) is an uncommon systemic necrotizing vasculitis that demonstrates renal and respiratory tropism. While the pathogenesis of WG remains controversial, autoimmune and inflammatory mechanisms are likely to be involved. The nervous system could be affected in up to 54% of cases. Although central nervous system involvement has been reported in 7-11% of cases, aneurysmal subarachnoid hemorrhage (SAH) occurrence is exceptional. We describe the third reported case of WG-related aneurysmal SAH and then discuss the diagnosis and pathogenesis of WG along with the physiopathology of intracranial aneurysm in light of recent data reported in the literature. A 63-year-old woman with WG was referred to our neurosurgical department for aneurysmal SAH. The vasculitis diagnosis had been established 4 years earlier when she presented with chronic sinusitis, recurrent cystitis, and renal failure. The cerebral angiography revealed an anterior communicating artery dysplastic aneurysm. The neurosurgical management of the aneurysm was scheduled but delayed because the patient was experiencing a vasculitis flare-up. Immunosuppressive therapy and intravenous corticotherapy were given, with the patient's improvement, allowing neurosurgical clipping of the aneurysm. Wegener granulomatosis-related aneurysmal SAH is an exceptional condition in neurovascular pathology. As inflammatory mechanisms are involved in the pathogenesis of aneurysm, the vasculitis flare-up could account for this SAH. The management of WG could benefit from anti-inflammatory therapy, as could the vasculitis-related SAH. SAH occurrence in patients with systemic vasculitis could indicate a vasculitis flare-up. Copyright (c) 2010 Elsevier Masson SAS. All rights reserved.

  19. Wegener granulomatosis as possible cause of vertigo: case report and review.

    Science.gov (United States)

    Goderis, J; De Schepper, S; Vannieuwenhuyze, P; Schmelzer, B

    2015-01-01

    Wegener granulomatosis (WG) or granulomatosis with polyangiitis (GPA) is a multi-system necrotizing granulomatous vasculitis that classically affects the upper respiratory tract, lungs and kidneys. We report the unusual clinical course of a patient with WG, and we present a literature review on the association between WG and vertigo. In our case, a 56-year-old female presented with a several-month history of chronic sinusitis and otalgia that was refractory to all initiated outpatient therapies. During hospitalization, a diagnostic evaluation revealed serious nasal crusting, chronic otitis media, and hematuria. Serologic testing showed elevated anti-neutrophil cytoplasmic antibodies (c-ANCAs). The tentative diagnosis of Wegener granulomatosis was confirmed by renal biopsy. Upon admission, the patient developed an acute vertigo with nystagmus, nausea, and vomiting, accompanied by sensorineural hearing loss in one ear. Vestibular symptoms may be associated with Wegener granulomatosis.

  20. Brief communication: high incidence of venous thrombotic events among patients with Wegener granulomatosis: the Wegener's Clinical Occurrence of Thrombosis (WeCLOT) Study.

    Science.gov (United States)

    Merkel, Peter A; Lo, Grace H; Holbrook, Janet T; Tibbs, Andrea K; Allen, Nancy B; Davis, John C; Hoffman, Gary S; McCune, W Joseph; St Clair, E William; Specks, Ulrich; Spiera, Robert; Petri, Michelle; Stone, John H

    2005-04-19

    Venous thrombotic events (VTEs) have been observed in Wegener granulomatosis, but the incidence rate is not known. To measure the incidence of VTEs in patients with Wegener granulomatosis. Prospective, observational cohort study. A multicenter, randomized, double-blind, placebo-controlled treatment trial for Wegener granulomatosis. 180 patients with Wegener granulomatosis enrolled during periods of active disease. Venous thrombotic events (deep venous thromboses or pulmonary emboli) were documented and confirmed prospectively. Incidence rates were calculated on the basis of time to first VTE. Thirteen patients had VTEs before enrollment. During 228 person-years of prospective follow-up, 16 VTEs occurred in 167 patients with no history of VTE. Median time from enrollment to VTE for patients with an event was 2.1 months. The incidence of VTE among patients with Wegener granulomatosis was 7.0 per 100 person-years (95% CI, 4.0 to 11.4). Although prospectively recorded, screening for VTEs did not occur. The incidence rate of VTEs in Wegener granulomatosis is high when compared with available rates in the general population, patients with lupus, and patients with rheumatoid arthritis. These results have important implications for clinical care of patients with Wegener granulomatosis.

  1. Tumor of the heart in a young woman; a rare manifestation of Wegener granulomatosis.

    Science.gov (United States)

    Singh, Remmi; Rosen, Seymour

    2012-02-01

    Wegener granulomatosis may rarely present with tumor masses that are in areas not typically involved by the disease. Although some cases have an associated positive anti-neutrophilic cytoplasmic antibodies, other cases do not, especially those of the limited form. To prevent misdiagnosis and ensure prompt treatment, it is extremely important to consider Wegener granulomatosis even in cases without the classic clinical findings. We report a case of a 19-year-old woman with no prior significant medical history who presented with persistent ventricular tachycardia and a papillary muscle mass in her left ventricle which upon excision and tissue evaluation demonstrated histologic changes of Wegener granulomatosis. Copyright © 2012 Elsevier Inc. All rights reserved.

  2. [Subglottic stenosis as the initial manifestation of Wegener's granulomatosis in a teenager. Case report].

    Science.gov (United States)

    Cuestas, Giselle; Rodríguez, Verónica; Doormann, Flavia; Pringe, Alejandra; Bellia Munzón, Patricio; Bellia Munzón, Gastón; Ortega, Carlos; Álvarez, Rubén

    2017-04-01

    Wegener's granulomatosis is a necrotizing granulomatous vasculitis of autoimmune origin that primarily affects the upper and lower airways and kidneys. It is very rare in children and adolescents. When started at a young age it is often associated with subglottic stenosis. Subglottic stenosis is a potentially fatal manifestation of Wegener's granulomatosis. Its diagnosis requires a high index of suspicion since it might develop in the absence of other signs of activity. Occasionally, subglottic stenosis may present as the initial manifestation of the disease. Diagnosis is confirmed by endoscopic visualization of the lesion. The treatment is complex, and it often requires repeated interventions due to restenosis. We present a 13-year-old patient with subglottic stenosis secondary to Wegener's granulomatosis. We describe the clinical manifestations, diagnosis and treatment of this rare cause of respiratory distress in the pediatric population. Sociedad Argentina de Pediatría.

  3. Strawberry gingivitis as the first presenting sign of wegener's granulomatosis: report of a case

    Science.gov (United States)

    2011-01-01

    Wegener's granulomatosis is a rare multi-system disease characterized by the classic triad of necrotizing granulomas affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. Oral lesions as a presenting feature are only encountered in 2% of these cases. Hyperplastic gingival lesions or strawberry gingivitis, is a characteristic sign of Wegener's granulomatosis. The latter consists of reddish-purple exophytic gingival swellings with petechial haemorrhages thus resembling strawberries. Recognition of this feature is of utmost importance for timely diagnosis and definitive management of this potentially fatal disease. A case of strawberry gingivitis as the first presenting sign of Wegener's granulomatosis affecting a 50-year-old Malay male is reported here. The differential diagnosis of red lesions that may present in the gingiva is discussed. PMID:21813375

  4. Chest CT findings in pediatric Wegener's granulomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Levine, Daniel [British Columbia Children' s Hospital, Department of Radiology and Nuclear Medicine, Vancouver, British Columbia (Canada); Akikusa, Jonathan [Royal Children' s Hospital Melbourne, Department of Rheumatology, Melbourne (Australia); Manson, David [Hospital for Sick Children, Department of Radiology, Toronto (Canada); Silverman, Earl; Schneider, Rayfel [Hospital for Sick Children, Department of Rheumatology, Toronto (Canada)

    2007-01-15

    Although pulmonary involvement occurs in the majority of children and adolescents with Wegener's granulomatosis (WG), relatively little has been published regarding the CT imaging manifestations in this group of patients. To determine the frequency and types of chest CT abnormalities in active pediatric WG (pWG). The study was a retrospective examination of 29 chest CT examinations performed at diagnosis (n=14) and during disease flares (n=15) in 18 children. The most common abnormalities were nodules (seen in 90% of examinations), ground-glass opacification (52%), and air-space opacification (45%). Of examinations with nodules, 73% demonstrated nodules >5 mm in diameter and 69% demonstrated more than five nodules; 17% had cavitary lesions. The only abnormality with a significant difference in prevalence between diagnosis and disease flares was air-space opacification, present in 71% and 20%, respectively (P < 0.01). In accordance with the findings of published adult studies and at variance with those of prior pediatric studies, our findings indicate that chest CT abnormalities in active pWG are frequent, most commonly comprising nodules and ground-glass opacification, which may be difficult to detect on plain radiography. We therefore advocate the routine use of chest CT for all affected patients, both at the time of presentation and during disease flares. (orig.)

  5. Massive Alveolar Hemorrhage During Wegener Granulomatosis: a Case Report

    Directory of Open Access Journals (Sweden)

    Gökhan Perincek

    2011-11-01

    Full Text Available This is a presentation of Wegener Granulomatosis (WG disease. Even though the lungs are rarely affected. massive alveolar hemorrhage is seen which leads to mortality. The patient was a 28 year old man. His illness was diagnosed as WG and glomerulonephritis a year previously and he was treated by administration of methylprednisolone orally. He had been treated irregularly. He applied to the emergency service with hemoptysis and asthma complaints two days earlier. After the results of his examination Hb: 3.6 gr/dl, Htc:10.3%, Üre:131 mg /dl, kreatini: 7.7 mg/dl, pH: 7.41, pO2: 55 mmHg, pCO2:33 mmHg, and being diagnosed as alveolar consolidation on lung X-ray, he was taken to the intensive care unit with a diagnosis of a massive alveolar hemorrhagei. He was intubated and attached to mechanical ventilation. He was treated with parenteral 1 mg/kg/day methylprednisolone and, siklofosfamid 2 mg/kg/day. He was extubated on the 21st day. He was taken to the chest service department on 24th day. He is still being treated.

  6. Hemorrhagic colitis as a presenting feature of Wegener granulomatosis.

    Science.gov (United States)

    Qian, Qi; Cornell, Lynn; Chandan, Vishal; Hartman, Robert; Caples, Sean

    2010-12-01

    Wegener granulomatosis (WG) is an idiopathic small vessel vasculitis involving primarily airway and kidneys. Intestinal involvement of WG is rare and usually occurs after several years of WG and its treatment. We report a case of WG, presented initially as rapid-onset hemorrhagic pancolitis without any preexisting or concurrent illness or any medication use. A 79-year-old previously healthy woman presented with 2-day duration of bloody diarrhea. Colonoscopy showed pancolitis with numerous ulcers; the biopsy showed foci of inflammation, ulceration and hemorrhage in the lamina propria, without features of chronic inflammatory bowel disease. Her stool studies were negative for infection. She subsequently developed pulmonary hemorrhage and kidney dysfunction. Kidney biopsy showed pauci-immune necrotizing glomerulonephritis. She responded to a combination therapy of steroids, cyclophosphamide and plasmapheresis with resolution of intestinal and pulmonary symptoms and improvement of kidney function. This case represents the first known example of colitis as an initial presentation of WG without confounding etiologic factors. Hemorrhagic colitis, although uncommon, may be the major presenting feature of WG.

  7. Imaging diagnosis of orbital Wegener granulomatosis: A rare case report.

    Science.gov (United States)

    Yang, Bin; Yin, Zhijian; Chen, Shuai; Yuan, Feng; Zhao, Wei; Yang, Yaying

    2017-06-01

    Wegener granulomatosis (WG) is a rare idiopathic autoimmune disease causing necrotizing granulomatous vasculitis. Whether as the first symptom or as part of systemic changes, ocular manifestations in WG patients are not specific. Any part of the eyes can be affected, with the anterior segment and orbit most commonly involved. So, early diagnosis and treatment are essential for controlling the progression of the disease and improving the quality of life for patients. Here we present a rare case of orbital WG of a 22-year-old woman was admitted to the hospital because of intense pain associated with decreased visual acuity in her right eye since 1 day. She had been previously diagnosed with WG at our hospital. Orbital computed tomography imaging showed diffuse swelling of intraorbital muscles, and space-occupying lesions were present in both eyes. Most postnasal anatomical structures were absent, appearing as a massive cavity shadow. Orbital magnetic resonance imaging showed a shadow of orbital soft tissues. WG is a serious, fatal disease. Early diagnosis and treatment are essential for controlling the progression of the disease and improving the quality of life for patients.

  8. Sellar Wegener Granulomatosis Masquerading as Cabergoline-Resistant Prolactinoma.

    Science.gov (United States)

    Eli, Ilyas M; Raheja, Amol; Corn, Heather J; Simmons, Debra L; Palmer, Cheryl Ann; Couldwell, William T

    2016-11-01

    Pituitary manifestation of Wegener granulomatosis (WG) is extremely rare. When there is pituitary involvement, the granulomatous inflammatory lesions involving the pituitary gland may appear several months to years after the primary diagnosis. We present a case of a 32-year-old woman who presented with galactorrhea, amenorrhea, and elevated serum prolactin levels. Imaging demonstrated a sellar lesion with characteristics of a pituitary macroadenoma. Treatment with cabergoline was initiated, but the tumor continued to grow during a 6-month period. Subsequent surgical exploration revealed a chronic inflammatory lesion; the patient subsequently was diagnosed with WG based on laboratory evaluation and further systemic manifestations. She had a favorable clinical and radiologic response with immunosuppressive doses of glucocorticoids and rituximab. This case appears to be the first reported of a patient with unknown WG in whom the diagnosis was established after she presented with a sellar lesion mimicking a prolactin-secreting pituitary adenoma on initial presentation requiring surgical resection. The only endocrine abnormality discovered was moderate hyperprolactinemia. Sellar lesions with only moderate elevations in serum prolactin, particularly those that are refractory to medical management with a dopamine agonist, should prompt further investigation to confirm the diagnosis. WG should be part of the differential diagnosis of inflammatory lesions in the sella, the identification of which can facilitate early diagnosis and treatment of this systemic disease for optimal outcome. Copyright © 2016 Elsevier Inc. All rights reserved.

  9. Rituximab for peripheral ulcerative keratitis with wegener granulomatosis.

    Science.gov (United States)

    Huerva, Valentín; Sanchez, María Carmen; Traveset, Alicia; Jurjo, Carmen; Ruiz, Agustín

    2010-06-01

    To demonstrate the effectiveness of Rituximab in the management of peripheral ulcerative keratitis (PUK) associated with Wegener granulomatosis (WG). A 50-year-old female with WG, confirmed by skin biopsy and positive anti-neutrophil cytoplasmic antibodies 3 years prior, was presented with a corneoscleral granulomatous mass that affected the superior limbus and with vasculitic PUK in the right eye. The mass was treated daily with oral prednisone (1 mg.kg-1.d-1) in conjunction with weekly maintenance treatment of 10 mg of methotrexate. After 2 months of treatment, the volume of the nodular granulomatous lesion decreased. However, the PUK and corneal thinning persisted, which presented a risk for corneal perforation. Subsequently, two 1000-mg infusions of rituximab were administered at weekly intervals. One week after the first administration of rituximab, we observed persistence of corneal thinning, flattening of the nodular lesion, and disappearance of the necrotic foci. After the second rituximab treatment, the patient exhibited no signs of corneal perforation and we further observed total epithelialization of the PUK. No systemic side effects were seen. After 2 months, no nodular lesion was observed, although conjunctival epithelialization from the previous PUK remained. Weekly methotrexate and 10 mg of prednisone per day were maintained. Rituximab seems to offer therapeutic promise in the treatment of refractory PUK associated with WG. Rituximab may be the elective treatment for severe anterior ocular inflammation associated with risk for corneal perforation, as a result of WG.

  10. Otologic symptoms as initial manifestation of wegener granulomatosis: diagnostic dilemma.

    Science.gov (United States)

    Wierzbicka, Małgorzata; Szyfter, Witold; Puszczewicz, Mariusz; Borucki, Lukasz; Bartochowska, Anna

    2011-08-01

    To show 7 cases of Wegener granulomatosis (WG) with early aural symptoms and to discuss the problems of otologic manifestation in WG. Retrospective case review. : Tertiary care university hospital. All patients were administered to the ENT University Department in Poznań in years 2002-2008 because of otitis media with effusion, facial palsy, sensorineural profound hypoacusis, hypoacusis combined with purulent discharge, and facial nerve palsy or progression of mixed type hypoacusis. Diagnostics and treatment. MAIN OUTCOME MEASURESL: Otologic symptoms as initial manifestation of WG diagnostic dilemma. The authors want to underline the young age of the patients, ranging from 32 to 46 years. The outcome of initially otologic cases, which developed generalized form of WG, was poor (the first patient died after 2 months, the second patient died after 7 days, the third had the pulmonary insufficiency in 2 months of observation, and the fourth had severe renal failure in 1 month), whereas the patients with localized disease have been successfully under control for 1 to 5 years. As WG often presents otologic symptoms, as an initial sign in some cases, it is important to take WG into consideration in atypical inflammatory states of the ear. The otologic onset of WG is very insidious, and prompt diagnosis in early stage of disease is a challenge. Focal and localized disease in the aural region might possibly require less aggressive therapy than acute-onset multi-organ disease and is connected with better prognosis.

  11. Severe impaired respiratory ciliary function in Wegener granulomatosis.

    Science.gov (United States)

    Ullrich, S; Gustke, H; Lamprecht, P; Gross, W L; Schumacher, U; Ambrosch, P; Laudien, M

    2009-06-01

    The pathogenesis of granulomatous inflammation in the respiratory tract and autoimmunity in Wegener granulomatosis (WG) are poorly understood. Since mucociliar clearance represents the first major line of defence in the respiratory tract and its breakdown facilitates chronic inflammation, we investigated ciliary beat frequency (CBF) in WG. Nasal epithelial cells were obtained from 30 patients with WG with involvement of the upper respiratory tract, 12 patients with other inflammatory rheumatic disease and 10 healthy controls. CBF was measured at 5 and 24 h after collection. were correlated with clinical data. Results: CBF was significantly reduced in WG compared to disease and healthy controls after 5 and 24 h. In WG, CBF almost stagnated after 24 h. Reduction of CBF correlated with the cumulative number of immunosuppressive agents in WG, but not in disease controls. No correlation was found between CBF impairment and cyclophosphamide levels, disease extent, disease activity, disease duration, serological and microbiological findings, or inflammation markers. CBF is severely impaired in WG, potentially influenced by immunosuppressive treatment. To what extent CBF impairment and subsequent barrier dysfunction are caused by other factors still has to be elucidated. Supportive measures to improve mucociliary clearance should be discussed in patients with WG.

  12. Cardiac involvement in Wegener granulomatosis diagnosed at autopsy.

    Science.gov (United States)

    Mukhopadhyay, Sanjay; Hensley, Richard G; Tazelaar, Henry D

    2010-01-01

    Wegener granulomatosis (WG) is a systemic vasculitis classically involving the lungs, kidneys, and upper respiratory tract. Involvement of other sites does occur but is less frequent. Clinically evident cardiac involvement is uncommon. There are only a few cases in the literature with documentation of the histologic appearance of cardiac involvement in WG. We report a case of a previously healthy 37-year-old man who presented with a one-week history of cough and weakness and a one-day history of shortness of breath. At presentation, he was hypoxic and required intubation. Upon hospitalization, he deteriorated rapidly, became bradycardic and expired the same night. Infection was suspected clinically as the cause of illness and death. At autopsy, the lungs showed diffuse alveolar hemorrhage with capillaritis, diffuse alveolar damage, and parenchymal necrosis with necrotizing granulomas. The heart was enlarged and showed a mixed inflammatory infiltrate composed of neutrophils, eosinophils, and histiocytes, with focal myocyte necrosis. Granulomas, giant cells, and vasculitis were absent. The esophagus showed submucosal vasculitis. A diagnosis of WG was made. Postmortem serology for c-ANCA was positive, and all cultures were negative, confirming the diagnosis. Cardiac involvement is an underrecognized and potentially fatal complication of WG. The histologic findings in the heart may consist of a non-specific inflammatory infiltrate without granulomas or vasculitis, raising a wide differential diagnosis. Copyright 2010 Elsevier Inc. All rights reserved.

  13. External dacryocystorhinostomy surgery in patients with Wegener granulomatosis.

    Science.gov (United States)

    Lee, Brian J; Nelson, Christine C; Lewis, Craig D; Perry, Julian D

    2012-01-01

    To determine surgical outcomes after external dacryocystorhinostomy (DCR) surgery in patients with Wegener granulomatosis (WG). The authors retrospectively reviewed the charts of consecutive patients with WG who underwent primary or secondary external DCR surgery between January 2001 and January 2010. Clinical data reviewed included patient demographics, systemic disease involvement and immunosuppression therapy, intraoperative biopsy findings, and postoperative outcomes and complications. Success was defined as resolution of epiphora. Sixteen primary external DCRs were performed on 9 patients with WG, and 2 secondary external DCRs were performed on 2 patients. At the time of surgery, all patients with WG were on systemic immunosuppressive agents including methotrexate, rapamycin, sirolimus, tacrolimus, azathioprine, cyclophosphamide, rituximab, and prednisone, and no patients received increased corticosteroids after surgery. Intraoperative biopsy in patients with WG revealed chronic inflammation (4 patients) and fibrosis (1 patient). Silicone stents were removed at an average of 5.8 months (range, 3-12 months). All surgeries were successful in resolving epiphora with an average follow up of 3.5 years (range, 10 months-6 years) and no complications. Primary and secondary external DCR surgery successfully treats nasolacrimal duct obstruction in patients with WG on systemic immunosuppression.

  14. Dorsal prevertebral lesions in Wegener granulomatosis: report on four cases.

    Science.gov (United States)

    Barreto, Paulo; Pagnoux, Christian; Luca, Luminata; Aouizerate, Jessie; Ortigueira, Isabel; Cohen, Pascal; Muller, Géraldine; Guillevin, Loïc

    2011-01-01

    Retroperitoneal fibrosis has been reported in several patients with Wegener granulomatosis (WG), but only three isolated cases of dorsal prevertebral lesions, closely resembling fibrosing mediastinitis, have been published so far. We describe four new WG patients (two men, two women), 49-59 years old at diagnosis, with dorsal prevertebral lesions, mainly right-sided, and with adjacent pleural thickening in two. These lesions were detected on computed-tomography scans at diagnosis in two patients, and occurred later in the two others. Only one of them had mild back pain. Two patients' lesions were biopsied, revealing granulomatous inflammation. In one patient, the lesion regressed under WG treatment. Lesion size did not change in the remainings. Intralesional calcifications appeared in two. None of the patients had local bone erosion, vascular or neurological complications. These prevertebral lesions might represent a dorsal form of retroperitoneal fibrosis in WG, but usually with a more benign presentation and course. WG should be included in the differential diagnosis of fibrosing mediastinitis (with tuberculosis, neoplastic diseases, sarcoidosis, histiocytosis and inflammatory pseudotumor), which may have a similar radiological appearance. Copyright © 2010 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  15. Bilateral acute angle-closure glaucoma as a first presentation of granulomatosis with polyangiitis (Wegener's

    Directory of Open Access Journals (Sweden)

    Alper Mete

    Full Text Available ABSTRACT We report a case of bilateral acute angle-closure glaucoma in a patient with undiagnosed granulomatosis with polyangiitis (Wegener's. A 59-year-old man presented with a severe headache, ocular pain, blurred vision, shortness of breath, and mild fever. Clinical examination revealed conjunctival chemosis, corneal edema, and shallow anterior chambers. Closed angles were observed bilaterally on gonioscopy. The patient was treated with intravenous mannitol, oral acetazolamide, and anti-glaucomatous eye drops. Over the following two days, his vision improved and intraocular pressures decreased. Subsequently, laser iridotomies were performed bilaterally and the patient attended consultations with our departments of respiratory medicine, nephrology, and rheumatology and was subsequently diagnosed with granulomatosis with polyangiitis. Bilateral acute angle-closure glaucoma is a very rare ocular manifestation of granulomatosis with polyangiitis. The association of this clinical entity with Wegener's granulomatosis remains unknown.

  16. Nasal mucosa narrow band imaging in granulomatosis with polyangiitis (Wegener granulomatosis): A preliminary study.

    Science.gov (United States)

    Trimarchi, Matteo; Bozzolo, Enrica; Pilolli, Francesco; Bertazzoni, Giacomo; Campochiaro, Corrado; Sabbadini, Maria Grazia; Bussi, Mario

    2015-01-01

    Narrow band imaging (NBI) endoscopy is a technique that allows for real-time visualization of mucosal and submucosal vascular patterns. Because granulomatosis with polyangiitis (GPA) (Wegener granulomatosis) is an autoimmune disease defined by vascular inflammation, we examined patients with GPA and with NBI to evaluate whether disease-specific mucosal vascular patterns were present. To the best of our knowledge, the use of NBI endoscopy for assessment of an immune system disease such as GPA has never been previously attempted. We conducted a prospective observational study by performing an endoscopic evaluation of upper airways with NBI on patients diagnosed with GPA; on patients with symptoms and signs suggestive for GPA, who were scheduled to undergo nasal biopsy to confirm diagnosis; on patients affected by chronic rhinosinusitis with nasal polyps; and on healthy controls. We enrolled 69 patients. NBI vascular patterns in patients with GPA were consistently and recognizably different from healthy mucosal patterns in 53% of our cases. In patients with GPA, biopsy and NBI results were for the most part comparable, except for three cases. Nasal mucosa NBI endoscopy can be considered a promising rapid and noninvasive live imaging technique for nasal mucosa GPA that, based on further study, could become a supplementary diagnostic tool in the complex workup of GPA and vasculitis.

  17. [Coexistence of Crohn disease and Wegener granulomatosis in a 15-year-old patient].

    Science.gov (United States)

    Sieczkowska, Agnieszka; Lewandowski, Piotr; Szumera, Małgorzata; Kamińska, Barbara

    2011-01-01

    Crohn disease is being diagnosed more and more frequently in children and teenagers. Clinical symptoms are mainly related to the gastrointestinal tract, however there are many reports in the literature about the coexistence of Crohn disease with other autoimmunological disorders such as celiac disease, autoimmune hypothyroidism, systemic lupus erythematosus and Wegener granulomatosis. We report a 15-year-old patient with Crohn disease who also developed Wegener granulomatosis. The presented case illustrates the difficulties in establishing the diagnosis when symptoms of the original disease are superimposed on symptoms of a different disorder.

  18. Wegener's granulomatosis in North India. Radiologic manifestations in eleven patients

    Energy Technology Data Exchange (ETDEWEB)

    Bambery, P.; Katariya, S.; Sakhuja, V.; Kaur, U.; Behera, D.; Malik, S.K.; Deodhar, S.D.

    The chest radiograph in Wegener's granulomatosis reveals several striking, but not pathognomonic abnormalities. Nine (82%) of eleven patients with Wegener's granulomatosis had abnormal chest radiographs. Infiltrates were seen in all patients and were nodular in seven (77%). Six patients had cavities which were most often unilateral and thick walled. Serial radiographs showed the simultaneous appearance and resolution of infiltrates in 4 of 7 instances. Bilateral pulmonary involvement (67%), pleural effusion (33%), and pericardial effusion (22%) were also seen. Paranasal sinus involvement was seen in 4 of 5 studies. The chest radiograph was interpreted as tuberculosis in eight patients, resulting in delayed diagnosis and incorrect treatment.

  19. New onset left bundle branch block with right axis deviation in a patient with Wegener's granulomatosis.

    Science.gov (United States)

    Khurana, C; Mazzone, P; Mandell, B

    2000-04-01

    Left bundle branch block (LBBB) is usually associated with a normal axis or left axis deviation (LAD). When it is seen in association with right axis deviation (RAD) it is felt to be a marker of diffuse advanced myocardial disease. We report a case of new onset LBBB with RAD in a patient with Wegener's granulomatosis who had an otherwise functionally and structurally normal heart. To our knowledge, this is the first case report of LBBB with RAD without severe cardiomyopathy, as well as the first case report of new onset LBBB as a result of Wegener's granulomatosis.

  20. Management Scheme for Cerebral Wegener Granulomatosis: An Unusual Pseudotumoral Skull Base Pathology.

    Science.gov (United States)

    Bernat, Anne Laure; Lefevre, Etienne; Sène, Damien; Herman, Philippe; Biassette, Homa Adle; Froelich, Sébastien

    2016-12-01

    Granulomatosis with polyangiitis (Wegener disease) is a rare antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis that seldom involves brain and meninges. We present a case of a 35-year-old woman with granulomatosis with polyangiitis involving the brain and the meninges. The histologic diagnosis required a complete resection of the lesion. Despite the possibility of postoperative wound infection, surgery was mandatory to ascertain the diagnostic and to treat locally. Copyright © 2016 Elsevier Inc. All rights reserved.

  1. Leucocyte membrane expression of proteinase 3 correlates with disease activity in patients with Wegener's granulomatosis

    NARCIS (Netherlands)

    Kobold, ACM; Kallenberg, CGM; Tervaert, JWC

    Wegener's granulomatosis (WG) is an inflammatory disorder characterized by granulomatous inflammation and vasculitis, and is strongly associated with antineutrophil cytoplasmic antibodies (ANCA). ANCA in patients with WG an directed against proteinase 3 (Pr3) in most of the cases. In vine, upon

  2. Patients with Wegener's granulomatosis : A long-term follow-up study

    NARCIS (Netherlands)

    de Leeuw, K.; van der Graaf, A. M.; Bijzet, J.; Stegeman, C. A.; Smit, A. J.; Kallenberg, C. G.; Bijl, M.

    2010-01-01

    Introduction. Atherosclerosis is accelerated in Wegener's granulomatosis (WG) patients. This study was aimed to assess which factors can predict progression of atherosclerosis in WG. Methods. 23 WG patients (14 men; age 47 +/- 9 years, mean +/- SD) and 21 controls (12 men; age 47 +/- 10 years) were

  3. Detection of immune deposits in skin lesions of patients with Wegener's granulomatosis

    NARCIS (Netherlands)

    Brons, RH; de Jong, MCJM; de Boer, NK; Stegeman, CA; Kallenberg, CGM; Tervaert, JWC

    2001-01-01

    Background-Wegener's granulomatosis (WG) is considered a pauci-immune systemic vasculitis based on the absence of immune deposits in renal biopsies of patients with active disease. In animal models of antineutrophil cytoplasmic antibody (ANCA) associated glomerulonephritis, immune deposits along the

  4. Cranial nerve palsy in Wegener's granulomatosis--lessons from clinical cases

    DEFF Research Database (Denmark)

    Nowack, Rainer; Wachtler, Paul; Kunz, Jürgen

    2009-01-01

    The problem of diagnosing vasculitic neuropathy is discussed based on case reports of two patients with Wegener's granulomatosis. One patient developed de novo 6(th) nerve palsy as an isolated relapse manifestation and the second patient a sequence of multiple cranial nerve palsies. Brain imaging...

  5. Long-term damage to the ENT system in Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Martinez Del Pero, Marcos; Walsh, Michael; Luqmani, Raashid

    2011-01-01

    The objectives of the study are to describe long-term ENT damage and assess risk factors in patients with newly diagnosed and treated Wegener's granulomatosis (WG) using the vasculitis damage index (VDI). Data from four randomised controlled trials carried out by the European Vasculitis Study Group...

  6. Incidence of granulomatosis with polyangiitis (Wegener's) in Greenland and the Faroe Islands

    DEFF Research Database (Denmark)

    Faurschou, M; Helleberg, M; Obel, N

    2013-01-01

    Previous studies suggest that the incidence of granulomatosis with polyangiitis (Wegener's; GPA) increases along a south-north gradient in the Northern Hemisphere with an incidence of 8.0/million/year reported for the population of Northern Norway. In the present study, we assessed the incidence...

  7. Bacterial infections in Wegener's granulomatosis : mechanisms potentially involved in autoimmune pathogenesis

    NARCIS (Netherlands)

    Tadema, Henko; Heeringa, Peter; Kallenberg, Cees G. M.

    Purpose of review Wegener's granulomatosis is associated with bacterial infection, in particular nasal carriage of Staphylococcus aureus. Infection may play a role in the induction of autoimmunity as well as in the effector phase of the disease. Here, the current hypotheses aiming to explain the

  8. Increased morbidity from ischemic heart disease in patients with Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Mellemkjaer, Lene; Sorensen, Inge Juul

    2009-01-01

    OBJECTIVE: Experimental studies indicate that patients with Wegener's granulomatosis (WG) experience accelerated atherosclerosis. The purpose of this study was to investigate whether the occurrence of overt ischemic heart disease (IHD) is increased in WG. METHODS: A total of 293 WG patients were...

  9. Structured clinical assessment of the ear, nose and throat in patients with granulomatosis with polyangiitis (Wegener's)

    DEFF Research Database (Denmark)

    Martinez Del Pero, Marcos; Rasmussen, Niels; Chaudhry, Afzal

    2013-01-01

    The objective of this prospective cross-sectional study is to describe the clinical otorhinolaryngological manifestations of granulomatosis with polyangiitis (Wegener's) (GPA) in a prospective cohort. All patients suffering from GPA seen in a tertiary centre between March 2007 and November 2008 had...

  10. Wegener's granulomatosis with unusual cavernous sinus and sella turcica extension

    Energy Technology Data Exchange (ETDEWEB)

    Hermann, M.; Bobek-Billewicz, B. [Dept. of Radiology, Medical University of Gdansk (Poland); Bullo, B.; Hermann, A.; Rutkowski, B. [Dept. of Nephrology, Medical University of Gdansk (Poland)

    1999-07-01

    Intracerebral extension of Wegener's granulomatosis (WG) is rare. We present a patient with oculomotor and trochlear nerve palsy with histologically proved WG. An MR examination revealed granulomatous tissue in nasal cavity, paranasal sinuses with meningeal infiltration, and uncommon penetration into cavernous sinus and sella turcica. The MR images before and during pharmacological therapy are presented. (orig.)

  11. Wegener´s granulomatosis in a young patient preceded by localized cutaneous manifestations

    Directory of Open Access Journals (Sweden)

    Jesper Smit

    2011-10-01

    Full Text Available Wegener´s granulomatosis (WG is a rare, systemic vasculitis involving multiple organs. The clinical presentation is highly diverse, and there is considerable risk of mortality if diagnosis and treatment are delayed. We present a case illustrating that patients with WG may initially present with localized cutaneous symptoms and signs.

  12. LATE RECURRENCE OF WEGENERS GRANULOMATOSIS PRESENTING AS SOLITARY UPPER LOBE PULMONARY MASS

    NARCIS (Netherlands)

    VANDERWERF, TS; STEGEMAN, CA; MEUZELAAR, KJ; TIMENS, W

    Recurrent Wegener's granulomatosis (WG) was diagnosed in a 40 year old man presenting with a solitary mass in the right lung apex and with possible lymph node enlargement in the anterior mediastinum, resembling malignancy. Eight years previously, a first episode of WG involving the upper airways and

  13. Neuropatia vasculítica na granulomatose de Wegener: relato de caso Vasculitic neuropathy in Wegener granulomatosis: case report

    Directory of Open Access Journals (Sweden)

    Hildo Azevedo Filho

    2001-06-01

    Full Text Available O acometimento neurológico no curso da granulomatose de Wegener atinge 15-50% dos pacientes, porém como sintomatologia inicial não é comentado na literatura. Relatamos o caso de uma paciente com mononeurite múltipla secundária a granulomatose de Wegener, enfocando os aspectos de antecipação do quadro neurológico em relação aos sintomas sistêmicos, a contribuição diagnóstica da biópsia do nervo sural e a boa evolução do quadro neurológico.The neurologic involvement during Wegener granulomatosis reachs 15-50% of patients, however as initial symptomatology is not commented in the literature. We describe a patient with Wegener's granulomatosis and mononeuritis multiplex, emphasizing the fact that neurologic manifestations antecipated systemic symptoms, and also focusing on the diagnostic contribution of sural biopsy and the good outcome of neurologic disease.

  14. Sinonasal imaging findings in granulomatosis with polyangiitis (Wegener granulomatosis): A systematic review.

    Science.gov (United States)

    D'Anza, Brian; Langford, Carol A; Sindwani, Raj

    2017-01-01

    Granulomatosis with polyangiitis (GPA) (Wegener granulomatosis) frequently presents in the head and neck, and the sinonasal cavity is among the most common areas affected. Although the clinical findings, histologic appearance, and laboratory workup have been described, characteristic findings and the distribution of disease on sinonasal imaging are not well established. The appropriate imaging modality to evaluate for sinonasal involvement is also unclear. To describe the imaging characteristics, distribution, and location of sinonasal pathology in patients with GPA as noted on computed tomography and magnetic resonance imaging modalities. A systematic review was conducted of English language articles, by using appropriate search terms, which reported the computed tomography and magnetic resonance imaging findings specific to sinonasal disease in adult subjects with GPA. Studies were included only when they focused primarily on specific imaging results in patients with GPA. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were followed. A total of 50 articles were identified on an initial search of medical literature data bases. There were seven articles that comprised 224 patients who met inclusion criteria. All the articles were retrospective case series and descriptive in nature. A high incidence of patients with GPA who were imaged showed evidence of pathologic findings. The prevalence of key radiographic findings in patients with GPA were the following: mucosal thickening (87.7% of patients), bony destruction (59.9%), and septal erosion (59.4%). There were no randomized or prospective studies that compared imaging findings between patients with GPA and the controls, and no studies that correlated imaging findings with prognosis. Sinus imaging in GPA revealed a spectrum of nonspecific findings. The constellation of septal erosion, mucosal thickening, and bony changes should raise suspicion for GPA. The current literature was

  15. Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's)

    DEFF Research Database (Denmark)

    Cabral, David A; Canter, Debra L; Muscal, Eyal

    2016-01-01

    OBJECTIVE: To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegener's) (GPA). METHODS: The European Medicines Agency...

  16. Characteristics and Outcomes of Granulomatosis With Polyangiitis (Wegener) and Microscopic Polyangiitis Requiring Renal Replacement Therapy

    DEFF Research Database (Denmark)

    Hruskova, Zdenka; Stel, Vianda S; Jayne, David

    2015-01-01

    providing individual RRT patient data to the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) Registry in 1993-2012 participated. PREDICTOR: Cause of primary kidney disease: AAV (ie, granulomatosis with polyangiitis [Wegener] and microscopic polyangiitis) versus 3 separate......-EDTA primary renal disease codes. RESULTS: 2,511 patients with AAV (1,755, granulomatosis with polyangiitis; 756, microscopic polyangiitis) were identified, representing an incidence of 1.05 per million population (pmp) for granulomatosis with polyangiitis (predominating in Northern Europe) and 0.45 pmp...... manifestations, treatment, and relapses. CONCLUSIONS: Geographical differences in the incidence of RRT for kidney failure due to granulomatosis with polyangiitis and microscopic polyangiitis copied their distribution in the general population. Overall survival on RRT after day 91 for patients with AAV...

  17. Long-term outcome of airway stenosis in granulomatosis with polyangiitis (Wegener granulomatosis): an observational study.

    Science.gov (United States)

    Martinez Del Pero, Marcos; Jayne, David; Chaudhry, Afzal; Sivasothy, Pasupathy; Jani, Piyush

    2014-11-01

    Airway stenosis occurs in patients with granulomatosis with polyangiitis (GPA or Wegener granulomatosis). It produces significant morbidity and contributes to mortality. To investigate the frequency and distribution of airway stenoses in GPA and evaluate the efficacy of local interventions in maintaining airway patency. A retrospective single-center study of 44 patients with GPA and airway stenosis assessed and treated as needed by a multidisciplinary team at a university medical center between 1997 and 2012. The median duration of observation for each patient from the time of diagnosis was 146 months. Patients who had critical stenoses underwent either dilatation or laser radial cuts to the lesion. In some cases, intralesional administration of steroids or topical mitomycin C was used. The main outcome measure was airway patency for at least 12 months and the number of interventions required to achieve this end point. Details of patients and interventions were recorded at baseline and at each treatment. The median age at diagnosis was 37.6 years; 73% of patients were women (n = 34). The median follow-up after the initial intervention was 62.5 months. Subglottic stenosis was found in 36 patients, lower airway stenosis in 30. There were 213 interventions in 39 patients, including balloon and bougie dilatation and laser treatment. Adjuvant local treatment was used in 71 interventions. A 12-month period of airway stability was achieved in 34 of 36 cases (97%) (5 had no procedures and 3 had follow-up shorter than 12 months). The median interval between procedures was 4.9 months, and after the last intervention recorded, patients had at least 27 months of airway stability. Fourteen adverse events were recorded (6.6%). The frequency and distribution of airway stenoses in 44 patients with GPA has been described. In the 39 patients who required intervention, multiple procedures were required, but 97% then achieved a prolonged period of airway patency. The procedures and

  18. Upper Respiratory Tract Symptoms, Renal Involvement and Vasculitis: A Case Report and Review of Wegener Granulomatosis

    Science.gov (United States)

    Said, Mohd Shahrir Mohamed

    2010-01-01

    Wegener’s Granulomatosis is a condition associated with systemic vasculitis which can present with upper respiratory tract symptoms initially. On September 2001, a 15-year-old girl presented with symptoms of nasal block for 3 weeks. She later developed joint pains and worsening renal status requiring dialysis. A renal biopsy was performed which showed pauci-immune cresentric glomerulonephritis. Her cANCA levels were positive. She was treated with oral cyclophosphamide and steroids and later responded. Keywords Wegener granulomatosis; Young girl; Cyclophophamide; cANCA PMID:21629538

  19. Otomastoiditis como manifestación clínica inicial de granulomatosis de Wegener Otomastoiditis as an initial clinical manifestation of Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    Jorge De All

    2011-02-01

    Full Text Available La granulomatosis de Wegener (GW forma parte del grupo de vasculitis primarias, de vasos pequeños y medianos, asociadas a anticuerpos anticitoplasma de neutrófilos (ANCA. Esta enfermedad puede afectar cualquier órgano, especialmente vías aéreas superiores, inferiores y el riñón. Muy raramente la primera y única manifestación clínica de GW generalizada es la otomastoiditis. Presentamos el caso de un paciente que inició su enfermedad con una inflamación del oído medio, sin respuesta al tratamiento habitual. Posteriormente agregó compromiso nasal, pulmonar y renal. La determinación de anticuerpos anticitoplasma de neutrófilos y la biopsia transbronquial confirmaron el diagnóstico de GW. Comunicamos este caso para referir que la otomastoiditis del adulto, refractaria al tratamiento habitual, puede raramente ser la primera y única manifestación clínica de la GW.Wegener's granulomatosis (WG forms part of a group of primary vasculitis of the small and medium-sized blood vessels, associated with antineutrophil cytoplasmic antibodies (ANCA. This disease may affect any body organ, especially the upper and lower airways and the kidneys. Hardly ever is otomastoiditis the first and only clinical manifestation of generalized Wegener's granulomatosis. We present the case of a patient whose disease started with the inflammation of the middle ear, which was unresponsive to the usual treatment. Later he developed nasal, pulmonary and renal compromise. The determination of ANCA and a transbronchial biopsy confirmed the diagnosis of WG. We report this case to express the view that otomastoiditis in adults, which is refractory to the usual treatment, may seldom be the first and only clinical manifestation of WG.

  20. Limited Wegener's granulomatosis presenting as lung nodules in a patient with rheumatoid arthritis: a case report

    OpenAIRE

    Pai, Sushma; Panda, Mukta

    2008-01-01

    Background Rheumatoid arthritis has varied pleuroparenchymal manifestations. Wegener's granulomatosis can develop in an established case of rheumatoid arthritis and this association although previously reported is very rare. Case presentation A 60-year-old lady had been diagnosed with rheumatoid arthritis on the basis of her clinical symptoms and serological tests which were positive RA factor and anti-CCP antibodies. Her rheumatoid arthritis activity had been mild and well controlled with hy...

  1. Pial involvement in Wegener's granulomatosis shown on MRI

    Energy Technology Data Exchange (ETDEWEB)

    Nusbaum, A.O. [Mount Sinai Hospital, New York (United States). Dept. of Radiology; Morgello, S.; Atlas, S.W. [Mount Sinai School of Medicine, New York (United States). Dept. of Pathology

    1999-11-01

    Involvement of the brain and meninges is rare in Wegener's granulomatosis (WG); it has been reported in 1.2-8 % of patients. Meningeal involvement in WG has been reported in imaging as being confined to the duramater, and is thought to represent granulomatous infiltration. We present a case of WG with abnormal pial enhancement and involvement of the perivascular spaces on MRI, pathologically proven to represent granulomatous infiltration due to the primary disease rather than to infection. (orig.)

  2. CT findings of sinonasal and orbital Wegener's granulomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Ri, Kyoushichi; Kushihashi, Tamio; Munechika, Hirotsugu (Showa Univ., Tokyo (Japan). School of Medicine) (and others)

    1994-02-01

    We reviewed the CT findings of four cases of Wegener's granulomatosis that presented as inflammatory masses in the sinonasal cavity or orbit. In the present study, an infiltrative nature and homogeneous texture with contrast enhancement were typical of the masses. In addition, the masses were frequently accompanied by infiltration of the pterygopalatine fossa or destruction of adjacent bone. However, no pathognomonic findings were observed. (author).

  3. Utilisation of plasma exchange in the treatment of digital infarcts in Wegener's granulomatosis.

    Science.gov (United States)

    Leung, Albert; Sung, Chee B; Kothari, Gargi; Mack, Christopher; Fong, Christopher

    2010-10-01

    Digital ischemia is a rare manifestation of Wegener's granulomatosis (WG) with previous management approaches based on the hypotheses of thromboembolic phenomenon and vasospasm. WG is an autoimmune, antineutrophil cytoplasmic antibody-predominant small-to-medium-vessel necrotising vasculitis which mainly affects the pulmonary-renal system. We report on a patient with WG presenting with digital infarction. We successfully treated this patient who had digital infarcts refractory to cyclophosphamide, steroids and vasodilator agents, with plasma exchange.

  4. Genetics Home Reference: granulomatosis with polyangiitis

    Science.gov (United States)

    ... area? Other Names for This Condition GPA Wegener granulomatosis Wegener's granulomatosis Related Information How are genetic conditions and ... Educational Resources (7 links) American College of Rheumatology: Granulomatosis with ... Vasculitis Disease InfoSearch: Wegener's granulomatosis Johns ...

  5. [A case of pulmonary-limited Wegener granulomatosis mimicking bacterial pneumonia caused by Staphylococcus aureus].

    Science.gov (United States)

    Ugajin, Motoi; Miwa, Seiichi; Suda, Takafumi; Shirai, Masahiro; Hayakawa, Hiroshi; Chida, Kingo

    2011-04-01

    A 56-year-old woman who had suffered from systemic lupus erythematosus and Sjögren syndrome was admitted complaining of persistent cough. Chest X-ray films showed an infiltrative shadow in the right middle lung field. Her serum PR3-ANCA titer was high, and granulomatous inflammation with Langhans giant cell was noted in a transbronchial biopsy specimen. About 3 months later, purulent sputum and high grade fever developed, with a new infiltrative shadow in the left upper lung field noted on a chest X-ray film. We treated her based on a diagnosis of bacterial pneumonia caused by methicillin-resistant Staphylococcus aureus, but her condition did not improve. We finally gave her a diagnosis of pulmonary-limited Wegener's granulomatosis. Her condition improved with the administration of sulfamethoxazole-trimethoprim, prednisolone and cyclophosphamide. We report a case of pulmonary-limited Wegener granulomatosis which mimicked bacterial pneumonia caused by methicillin-resistant Staphylococcus aureus. This case suggests that Wegener's granulomatosis should be considered on encountering pneumonia caused by Staphylococcus aureus.

  6. Thoracic manifestation of Wegener's granulomatosis: CT findings in 30 patients

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kyung Soo; Kim, Tae Sung; Kim, Eun A. [Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-Dong, Kangnam-Ku, Seoul 135-710 (Korea); Fujimoto, Kiminori [Department of Radiology, Kurume University School of Medicine, Kurume 830-0011 (Japan); Moriya, Hiroshi [Department of Radiology, Fukushima Medical University School of Medicine, Fukushima 960-1295 (Japan); Watanabe, Hideyuki [Department of Radiology, University of Occupational and Environmental Health, Japan School of Medicine, Kitakyushu 807-8555 (Japan); Tateishi, Ukihide [Department of Radiology, Hokkaido University Graduate School of Medicine, Sapporo 060-8638 (Japan); Ashizawa, Kazuoto [Department of Radiology, Nagasaki University School of Medicine, Nagasaki 852-8501 (Japan); Johkoh, Takeshi [Department of Radiology, Osaka University Graduate School of Medicine, Suita 565-0871 (Japan); Kwon, O. Jung [Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-Dong, Kangnam-Ku, Seoul 135-710 (Korea)

    2003-01-01

    Our objective was to describe the CT findings of thoracic Wegener's granulomatosis. At presentation, both conventional and thin-section CT scans were available in 30 patients with Wegener's granulomatosis. Serial CT scans (range of intervals: 1-25 months, mean 4.5 months) were available in 20 patients. The initial and follow-up CT scans were analyzed retrospectively by two observers in terms of pattern and extent of parenchymal and airway lesions. Positive CT findings were seen in 29 of 30 (97%) patients at initial presentation. The most common pattern was nodules or masses seen in 27 of 30 (90%) patients. They were multiple in 23 of 27 (85%) patients, bilateral in 18 (67%), subpleural in 24 (89%), and peribronchovascular in 11 (41%) in distribution. Bronchial wall thickening in the segmental or subsegmental bronchi was seen in 22 (73%) patients. Large airways were also abnormal in 9 (30%) patients. Patchy areas of consolidation and ground-glass opacity were seen in 7 (23%) patients, respectively. In 17 of 20 (85%) patients in whom follow-up CT scans were available, the parenchymal or airway lesion showed complete or partial improvement with treatment. The CT findings of Wegener's granulomatosis, although multiple and variable, consist mainly of bilateral subpleural or peribronchovascular nodules or masses and bronchial wall thickening in the segmental or subsegmental bronchi. Parenchymal and airway lesions improve with treatment in most patients. (orig.)

  7. Granulomatose de Wegener e estenose subglótica: descrição de caso Wegener's granulomatosis and subglottic stenosis: case description

    Directory of Open Access Journals (Sweden)

    Fernanda Guidolin

    2004-06-01

    Full Text Available Descreve-se o caso de um paciente com Granulomatose de Wegener, em tratamento com esteróides e ciclofosfamida, que, apesar do tratamento, evoluiu com dispnéia progressiva e rouquidão. A investigação mostrou tratar-se de uma estenose subglótica, sendo a dispnéia prontamente aliviada com a traqueostomia. São discutidos diagnósticos diferenciais e modalidades de tratamentos de estenose subglótica em portadores de Wegener.We describe a patient with Wegener Granulomatosis being treated with steroids and cyclofosfamide that developed progressive dyspnea and hoarseness despite of treatment. Investigation showed a subglotic stenosis. The dyspnea was relieved by tracheotomy. We discuss the differential diagnosis as well as a number of treatment modalities in patients with Wegener granulomatosis and subglottic stenosis.

  8. Wegener's granulomatosis of the maxillary sinus: CT evaluation - report of two cases-

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Yong Hyun [Masan Korea General Hospital, Masan (Korea, Republic of)

    1991-03-15

    Wegener's granulomatosis is a specific vasculitis described by Klinger in 1931 and Wegener in 1939 as being different from periarteritis nodosa. Wegener's gramulomatosis involving the paranasal sinuses is relatively rare and few cases are reported in the Korean literature. However, CT evaluation has been lacking in the Korean literature. Although radiological evaluations were performed in the previous reports, they were not studied by CT. Only one report of the CT evaluation is available in the literature. Since the author observed somewhat different CT manifestations compared with the previous report, the CT findings with a brief review of the literature are presented. Although the difference from the previously reported series by Paling is not clear, it may be affected by differences in he duration of illness and severity of the disease.

  9. Antiproteinase 3 antineutrophil cytoplasmic antibodies and disease activity in Wegener granulomatosis.

    Science.gov (United States)

    Finkielman, Javier D; Merkel, Peter A; Schroeder, Darrell; Hoffman, Gary S; Spiera, Robert; St Clair, E William; Davis, John C; McCune, W Joseph; Lears, Andrea K; Ytterberg, Steven R; Hummel, Amber M; Viss, Margaret A; Peikert, Tobias; Stone, John H; Specks, Ulrich

    2007-11-06

    The utility of antineutrophil cytoplasmic antibody (ANCA) levels to guide the management of patients with Wegener granulomatosis remains controversial. To determine whether pro-proteinase 3 (PR3)-ANCA levels are a better measure of disease activity than mature-PR3-ANCA levels, whether decreases in either level are associated with shorter time to remission, and whether increases are followed by relapse. Prospective, observational cohort study. 8 United States medical centers that participated in a treatment trial for Wegener granulomatosis. 156 patients with Wegener granulomatosis enrolled during periods of active disease. PR3-ANCA levels (by capture enzyme-linked immunosorbent assay) and disease activity (by the Birmingham Vasculitis Activity Score for Wegener granulomatosis). The ANCA levels were only weakly associated with disease activity across patients. The longitudinal association within patients was stronger, but changes in ANCA levels explained less than 10% of the variation in disease activity. Decreases in mature- and pro-PR3-ANCA levels were not statistically significantly associated with shorter time to remission, and increases in mature-PR3-ANCA levels (adjusted hazard ratio, 0.8 [95% CI, 0.4 to 1.9]; P = 0.67) and pro-PR3-ANCA levels (adjusted hazard ratio, 1.0 [CI, 0.5 to 2.1]; P = 0.99) were not associated with relapse. The proportion of patients who had relapse within 1 year of an increase in PR3-ANCA levels was 40% for mature-PR3 (CI, 18% to 56%) and 43% for pro-PR3 (CI, 22% to 58%). Samples were collected approximately every 3 months. Sensitivity and specificity of ANCA levels for detecting remission and relapse could not be calculated because each patient had different follow-up times. Pro-PR3-ANCA is no better than mature-PR3-ANCA as a measure of Wegener granulomatosis activity. Decreases in PR3-ANCA levels are not associated with shorter time to remission, and increases are not associated with relapse. These findings suggest that ANCA levels

  10. T-cell mediated immunity in Wegener's granulomatosis

    NARCIS (Netherlands)

    Abdulahad, Wayel Habib

    2008-01-01

    Although the pathogenetic mechanisms involved in Wegener’s granulomatosis (WG) are not completely understood, considerable evidence support the concepts that activated T-cells play an important role in disease expression. It is, however, not clear which subsets of T-cells are involved in the

  11. Wegener Granulomatosis with Oral Involvement as Primary Manifestation: A Case Study

    Directory of Open Access Journals (Sweden)

    Mahdokht Rashed

    2017-06-01

    Full Text Available Introduction: Wegener Granulomatosis is a rare multisystemic disease with an unknown cause, characterized by necrotic granulomatous lesions in respiratory tract, systemic vasculitis in small arteries and veins and necrotizing glomerulonephritis. Wegener can affect any organ including kidneys, eyes or other organs but classically affects upper and lower respiratory tract. One of the rare but important signs of this disease is oral involvement, generally occurring in 6-13% of patients, however, oral involvement as the primary manifestation of disease, occurs in only 5-6% of cases. The most common oral manifestation is strawberry gingivitis. Patients: Our patient was a 35 year-old man with gingival bleeding during brushing which began approximately 45 days before referring to the department of oral and maxillofacial diseases, Mashhad Dental School. In intraoral examination, his gingiva had a papillomatous appearance and was purple in color (strawberry appearance. Due to the presence of strawberry appearance in absence of plaque, primary diagnosis of Wegener granulomatosis was established and the patient was referred for histopathological evaluation. In laboratory tests, C-ANCA was positive and P-ANCA was negative. Finally, diagnosis of Wegener granulomatosis was confirmed and his treatment was started. Rheumatologic condition of patient's lungs was evaluated by chest X-ray and CT-scan and blood tests, biochemistry tests and urine analysis were performed for the patient. He did not have pulmonary or renal involvement. In our study, the patient was followed up after 1, 2 and 11 months from the first visit. Discussion: Up to now, few reports have been published on Wegener disease with oral involvement and in most of these articles, Wegener was diagnosed after respiratory symptoms and kidney or other organs involvement. Only in few studies was Wegener diagnosis confirmed on the basis of oral symptoms and gingival involvement. Immediate and aggressive

  12. Malignant Pyoderma Associated with Granulomatosis with Polyangiitis (Wegener Granulomatosis) as a Unique Indication for Facial Vascularized Composite Allotransplantation: Part I.

    Science.gov (United States)

    Gastman, Brian; Hashem, Ahmed M; Djohan, Risal; Bernard, Steven; Hendrickson, Mark; Schwarz, Graham; Gharb, Bahar B; Rampazzo, Antonio; Fernandez, Anthony; Zins, James; Hoffman, Gary S; Doumit, Gaby; Siemionow, Maria; Papay, Francis

    2016-06-01

    Granulomatosis with polyangiitis (Wegener granulomatosis) is a rare disease that commonly starts in the craniofacial region and can lead to considerable facial disfigurement. Granulomas and vasculitis, however, can involve many other tissues (especially pulmonary and renal). Dermatologic and subcutaneous components can lead to malignant pyoderma. The authors describe a unique pathologic condition, where significant Le Fort type trauma was associated with subsequent development of granulomatosis with polyangiitis and malignant pyoderma. Successive operations to excise necrotic tissue and reconstruct the defects were followed by worsening inflammation and tissue erosions. Trauma and surgery in proximity to the eye and sinuses masked the initial clinical presentation and led to delay in diagnosis and disease progression. The resultant facial disfigurement and tissue loss were substantial. Despite multiple confounding factors, accurate diagnosis was eventually established. This was based on persistence of sinus inflammations in the absence of infective agents, proven sterility of lung lesions, and antineutrophil cytoplasmic antibody positivity with proteinase 3 specificity. Skin lesion biopsy specimens were identified as pyoderma gangrenosum and later as malignant pyoderma. Institution of immunosuppressive therapy allowed successful control of the disease and wound healing. The resulting craniofacial destruction, however, necessitated facial vascularized composite allotransplantation. Recognition of this rare pathologic association is essential, to prevent delays in diagnosis and treatment that can lead to major craniofacial tissue loss. Therapeutic, V.

  13. Liver changes in Wegener's granulomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Lehmann, H.; Struve, C.

    1981-05-01

    A 54-year-old woman is described, who had been treated with immunosuppressive therapy for Wegener's granuloma for several years. The clinical course was surprisingly long and benign. An incidental sonographic and scintigraphic finding was a change in the liver which healed at the same time as the lung lesions and which is therefore assumed to have been due to inflammatory involvement of the liver.

  14. Granulomatose de Wegener: Envolvimento otológico, nasal, laringotraqueal e pulmonar Wegener granulomatosis: Otologic, nasal, tracheobronchial and pulmonary involvement

    Directory of Open Access Journals (Sweden)

    Sandra Figueiredo

    2009-10-01

    Full Text Available A granulomatose de Wegener é uma vasculite sistémica rara e idiopática caracterizada pelo atingimento dos pequenos vasos. A doença atinge, preferencialmente, as vias aérea superior e inferior e os rins, levando à formação de granulomas e necrose destes órgãos. As manifestações clínicas e o envolvimento orgânico variam largamente. O diagnóstico e tratamento precoce podem levar à recuperação total. No entanto, o atraso no diagnóstico pode ser fatal. Os autores apresentam o caso de uma doente de 33 anos com uma forma de apresentação grave e rara da doença, mas com uma evolução favorável após diagnóstico e tratamento adequado.Wegener granulomatosis is a rare systemic idiopathic disease characterized by involvement of small vessels - medium and small arteries, venules, arterioles and ocasionally large arteries. This disease has predilection for the upper and lower respiratory tract and the kidney, with granulomatous inflamation and necrosis. Clinical manifestations and organ involvement of the disease vary widely. Early diagnosis and treatment may lead to a full recovery. Without treatment, Wegener’s granulomatosis can be fatal. The authors present a case of a 33 year-old female, with severe disease, but with good outcome, after adequate diagnosis and treatment.

  15. Granulomatous renal pseudotumor in Wegener's granulomatosis: imaging findings in one case

    Energy Technology Data Exchange (ETDEWEB)

    Verswijvel, G.; Eerens, I.; Oyen, R. [Department of Radiology, University Hospitals. Catholic University of Leuven, Leuven (Belgium); Messiaen, T. [Department of Nephrology, University Hospitals. Catholic University of Leuven, Leuven (Belgium)

    2000-08-01

    Wegener's granulomatosis is a clinico-pathological entity characterized by necrotizing granulomatous angiitis involving the upper- and/or lower respiratory tract and the kidneys. Renal involvement is usually characterized by a rapidly progressive necrotizing glomerulonephritis. A case is presented of a patient who developed renal failure and presented a solitary pseudotumoral lesion in the upper pole of the left kidney. Imaging characteristics on US, CT and MR imaging are discussed. The diagnosis was confirmed with ultrasound-guided needle biopsy. (orig.)

  16. Indium-111 leukocyte scintigraphy in Wegener's granulomatosis involving the spleen

    Energy Technology Data Exchange (ETDEWEB)

    Morayati, S.J.; Fink-Bennett, D.

    1986-12-01

    Indium-111-labeled leukocyte scintigraphy was performed on a 44-yr-old man to exclude an occult abscess. Four- and twenty-four-hour images of the abdomen revealed splenic photopenia except for a rim of activity medially. A subsequent computed tomography (CT) study demonstrated necrosis or hemorrhage of the spleen except for a medial rim. Exploratory laparotomy demonstrated necrotizing vasculitis with granuloma formation consistent with Wegener's granulomatosis and a rim of viable splenic tissue corresponding to the radionuclide and CT studies.

  17. Orbital tumour due rhinosinusal extension in a patient with Wegener's granulomatosis.

    Science.gov (United States)

    Molina-Sócola, F E; Galván-Carrasco, M P; Del Estad-Cabello, A; Sánchez-Vicente, J L; Contreras-Díaz, M; Rueda-Rueda, T

    2016-09-01

    The case is presented of a 49 year-old woman with an orbital mass originating from the rhinosinus. She had a history of Wegener's granulomatosis, refractory to both biological and immunosuppressive therapy. Clinical examination showed proptosis, diplopia, and restriction of ocular movements. Orbital mass resection was performed, due to its rapid growth, and lack of response to medical treatment. Copyright © 2016 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.

  18. Lingual infarction in Wegener's Granulomatosis: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Brizman Eitan

    2008-08-01

    Full Text Available Abstract Wegener's granulomatosis (WG is a multi-system disease, characterised by the triad of necrotising granulomata affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. Oral lesions are associated with up to 50% of cases, although are rare as a presenting feature. The most common oral lesions associated with WG are ulceration and strawberry gingivitis. We review the literature regarding oral manifestations of WG and present a case of lingual infarction, an extremely rare oral lesion associated with WG, in a severe, rapidly progressive and ultimately fatal form of the disease.

  19. Imaging findings of pulmonary granulomatosis with polyangiitis (Wegener's granulomatosis): lesions invading the pulmonary fissure, pleura or diaphragm mimicking malignancy.

    Science.gov (United States)

    Guneyli, Serkan; Ceylan, Naim; Bayraktaroglu, Selen; Gucenmez, Sercan; Aksu, Kenan; Kocacelebi, Kenan; Acar, Turker; Savas, Recep; Alper, Hudaver

    2016-11-01

    Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis), in which pulmonary involvement often predominates, is a multisystem granulomatous, necrotizing vasculitis that affects small and medium-sized vessels. In this study we evaluated various radiological findings of pulmonary GPA and focused on spiculated pulmonary lesions invading the pulmonary fissure, pleura or diaphragm mimicking malignancy. This retrospective study included 48 patients, aged 28-73 (mean, 47.3) years, who showed either histopathological diagnosis of GPA (n = 39) or elevated levels of the cytoplasmic anti-neutrophilic cytoplasmic antibody serum marker (n = 9) between January 2003 and December 2013. All patients received a chest computed tomography (CT), and the types of pulmonary lesions were defined and evaluated. Among the 48 patients, 33 had abnormal pulmonary findings on CT. The most commonly detected pulmonary lesion types were nodules and masses (n = 126) observed in 24 patients. Cavitation, necrosis, spiculation and invasion of the fissure, pleura or diaphragm were observed in 14, 9, 10 and 6 patients, respectively. Consolidation was found in 14 patients and thickening of bronchial wall in 8 patients. Pulmonary lesion types of GPA have a wide spectrum, potentially mimicking a high number of diseases including malignancy, infection and noninfectious inflammatory diseases. A spiculated lung lesion invading the fissure, pleura or diaphragm is mostly present in malignancy, but it can be also seen in GPA.

  20. Tumour necrosis factor-alpha (TNF), lymphotoxin and TNF receptor levels in serum from patients with Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Jónasdóttir, O; Petersen, J; Bendtzen, K

    2001-01-01

    Wegener's granulomatosis (WG) is a systemic inflammatory disease with vasculitis as the key feature. Abnormal expression of tumour necrosis factor alpha (TNFalpha) is considered of prime pathogenic importance in several inflammatory diseases. The effects of TNFa are mediated by TNF receptors (TNF...

  1. High risk of pulmonary embolism and deep venous thrombosis but not of stroke in granulomatosis with polyangiitis (Wegener's)

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Obel, Niels; Baslund, Bo

    2014-01-01

    OBJECTIVE: To assess the incidence of stroke, pulmonary embolism (PE), and deep venous thrombosis (DVT) in granulomatosis with polyangiitis (Wegener's) (GPA). METHODS: Patients diagnosed with GPA at a Danish tertiary care center during 1993-2011 were identified (n = 180). Each patient was matched...

  2. Plasma exchange for induction and cyclosporine A for maintenance of remission in Wegener's granulomatosis--a clinical randomized controlled trial

    DEFF Research Database (Denmark)

    Szpirt, Wladimir M; Heaf, James G; Petersen, Jørgen

    2011-01-01

    The use of plasma exchange (PE) for induction treatment of anti-neutrophil cytoplasm autoantibody (ANCA)-associated vasculitis (AAV), including Wegener's granulomatosis (WG), is still controversial. The use of PE in AAV is not commonly accepted in patients with a plasma creatinine...

  3. Wegener`s granulomatosis and mucoromycosis: A case study and review of literature

    Directory of Open Access Journals (Sweden)

    Sayyed Mojtaba Abtahi

    2012-01-01

    Full Text Available Mucormycosis is a fatal invasive infection which mostly involves diabetic or immunosuppressed patients. Early diagnosis, improving immunosuppression, systemic antifungal therapy, and surgical debridement are necessary for successful treatment. In this case study, we represent a known case of Wegener′s granulomatosis (WG, with concomitant sinusal mucormycosis mimicking vasculitic disease relapse, which was successfully treated with surgical debridement, amphotericine, and intravenous immunoglobuline.

  4. Therapeutic implications of coexisting severe pulmonary hemorrhage and pulmonary emboli in a case of Wegener granulomatosis.

    Science.gov (United States)

    Dreyer, Gavin; Fan, Stanley

    2009-05-01

    Wegener granulomatosis classically involves the renal, respiratory, and ear, nose, and throat systems. Pulmonary hemorrhage is recognized as a severe respiratory complication. Untreated, the mortality rate approaches 90% at 2 years. We describe a case of Wegener granulomatosis with coexistent severe lung hemorrhage and pulmonary and deep vein thromboses. A 31-year-old man presented with features of vasculitis, including epistaxis, fever, and acute kidney injury with an increased serum creatinine level (3.27 mg/dL). Kidney biopsy confirmed pauci-immune crescentic glomerulonephritis, and antineutrophil cytoplasmic antibody showing a cytoplasmic staining pattern was strongly positive. Standard immunosuppression therapy (prednisolone and cyclophosphamide) was started. Eleven days later, the patient developed sudden dyspnea. A computed tomographic pulmonary angiogram showed pulmonary emboli, and ultrasound of the limbs showed ileofemoral thrombi bilaterally. Subcutaneous enoxaparin and warfarin therapy was started, but 8 days later, the patient had a massive pulmonary hemorrhage. Anticoagulation therapy was stopped, and plasma exchange was started to prevent further life-threatening hemorrhage. An inferior vena cava filter was inserted to prevent further pulmonary emboli during the period when anticoagulation was withheld. Kidney function improved, and pulmonary hemorrhage resolved after 5 plasma exchanges. Reintroduction of intravenous heparin and subsequently warfarin caused no further bleeding. We discuss the difficult management dilemma this combination of disease manifestations presents and review the current literature.

  5. Common and uncommon manifestations of Wegener granulomatosis at chest CT: radiologic-pathologic correlation.

    Science.gov (United States)

    Martinez, Felipe; Chung, Jonathan H; Digumarthy, Subba R; Kanne, Jeffrey P; Abbott, Gerald F; Shepard, Jo-Anne O; Mark, Eugene J; Sharma, Amita

    2012-01-01

    Wegener granulomatosis is an uncommon necrotizing vasculitis that classically manifests as a clinical triad consisting of upper and lower airway involvement and glomerulonephritis. Other less frequently involved organ systems include the central and peripheral nervous system and large joints. The diagnosis is based on a combination of clinical and laboratory findings. Because thoracic involvement often predominates, chest radiographic findings are often the first to suggest the diagnosis. However, chest computed tomography (CT) has superior sensitivity and specificity for evaluation of the airways, lung parenchyma, and mediastinum, particularly with the use of multiplanar reformatted and three-dimensional images. Common pulmonary radiologic findings include waxing and waning nodules, masses, ground-glass opacities, and consolidation. Airway involvement is usually characterized by circumferential tracheobronchial thickening, which can be smooth or nodular. Pleural effusions are the most common manifestation of pleural disease and can result from primary involvement or be secondary to renal failure. Mediastinal lymphadenopathy is a nonspecific finding and is usually reactive. Uncommon thoracic radiologic manifestations include involvement of the heart and great vessels. CT is the imaging modality of choice for diagnosis, surveillance, and follow-up in patients with Wegener granulomatosis. © RSNA, 2012.

  6. Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment.

    Science.gov (United States)

    Comarmond, Cloé; Cacoub, Patrice

    2014-11-01

    Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis, which affects small- and medium-sized blood vessels and is often associated with cytoplasmic ANCA. GPA occurs in patients between 45 and 60 years old of both genders, and is rarely observed in blacks. The prevalence of GPA increases along a south-north gradient in Europe (20 to 150/million). The main clinical characteristics involve the upper and/or lower respiratory tract and kidneys. Ear, nose and throat manifestations with recurrent sinusitis and crusting rhinorrhea are usually severe. Lung nodules are frequently seen, sometimes excavated. Renal involvement is characterized by rapidly progressive necrotizing glomerulonephritis with extracapillary crescents. Limited forms of GPA predominantly affect the upper respiratory tract, whereas generalized forms of GPA include renal manifestations and/or alveolar hemorrhage and/or vital organ involvement with an altered general condition. The combination of immunosuppressant drugs and corticosteroids has converted this typically fatal illness into one in which 80% of patients achieve remission. However, despite considerable therapeutic progress over the last decades, relapses remain frequent (50% at 5 years), and maintenance treatment is now the main therapeutic challenge. Copyright © 2014 Elsevier B.V. All rights reserved.

  7. [Granulomatosis with polyangiitis (Wegener). Description of 15 cases].

    Science.gov (United States)

    Martínez-Morillo, Melania; Grados, Dolors; Naranjo-Hans, Dolores; Mateo, Lourdes; Holgado, Susana; Olivé, Alejandro

    2012-01-01

    Granulomatosis with polyangiitis (GP) is a necrotizing vasculitis of unknown etiology that involves small and medium caliber vessels. It is associated with anti neutrophil cytoplasm antibodies (ANCA). It most often affects the respiratory tract and the kidneys and its most important pathologic feature is the presence of necrotizing granulomas. To detail the features of 15 patients with GP diagnosed in a university referral center. Retrospective study: between 1984 and 2009, 15 patients with GP were diagnosed in our center. Epidemiological, clinical, laboratory test as well as pathologic studies and treatment were retrospectively analyzed. Biopsy diagnosis of GP was considered as an inclusion criterion. Fifteen patients were diagnosed: 12 men and 3 women. Mean age at diagnosis: 52.2 years (14-78). 12 patients had a history of smoking. A biopsy was diagnostic in all patients. ANCA were positive in 11 cases, 6 had a cytoplasmic c-ANCA pattern. All patients had pulmonary involvement and seven (40%) had renal involvement. All patients received intravenous glucocorticoids and cyclophosphamide as induction therapy. During the disease progression 5 patients died. The clinical features of this series do not differ from those described by other authors. However, a history of smoking is more common than expected. Frequently used drugs were glucocorticoids and cyclophosphamide (oral and pulse therapy). The course was usually unfavorable, with outbreaks or complications due to immunosuppression, except for those with limited forms. Immunosuppressive therapy should be maintained indefinitely in most cases. Copyright © 2010 Elsevier España, S.L. All rights reserved.

  8. Scleritis in patients with granulomatosis with polyangiitis (Wegener).

    Science.gov (United States)

    Cocho, Lidia; Gonzalez-Gonzalez, Luis Alonso; Molina-Prat, Nicolas; Doctor, Priyanka; Sainz-de-la-Maza, Maite; Foster, C Stephen

    2016-08-01

    To describe and compare clinical features, complications and outcomes in patients with granulomatosis with polyangiitis (GPA)-associated scleritis with those seen in idiopathic and other autoimmune-associated scleritis, and to further describe the features that may serve as an indicator of life-threatening systemic disease. We retrospectively reviewed electronic health records of all patients with scleritis seen at two tertiary care centres. Of 500 patients, 14 had GPA-associated scleritis and were included in this analysis. Measures included were age, gender, laterality, visual acuity and underlying systemic or ocular diseases. Clinical features (location, pain, inflammation) and ocular complications of these patients (decrease of vision, concomitant anterior uveitis and ocular hypertension) were studied and correlated. Fourteen of 500 patients with scleritis were GPA associated. Most of the patients with GPA-associated scleritis presented with sudden onset, bilateral, diffuse anterior scleral inflammation, with moderate-or-severe pain. Vision loss was not significantly different, and pain was more severe in these patients than in those with idiopathic scleritis. When compared with patients with other underlying autoimmune diseases, there were no significant differences found in epidemiological or clinical signs. Necrotising scleritis and corneal involvement were more commonly observed in GPA than in idiopathic scleritis and other autoimmune diseases and are often the presenting feature of the disease. The presence of necrotising changes or corneal involvement in the setting of scleral inflammation is highly suggestive of an underlying systemic vasculitis, of which GPA is the most common. These features should alert the doctor/optometrist and prompt a thorough diagnostic approach and an aggressive treatment given that it could reveal a life-threatening disease. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a

  9. [A historic case of Wegener's granulomatosis: the physicist who discovered the electromagnetic waves: Heinrich Hertz].

    Science.gov (United States)

    Feldmann, H

    2005-06-01

    A diary and an extensive correspondence of Heinrich Hertz and his family provide a meticulous documentation of his life, made available by Albrecht Folsing in 1997 through a carefully investigated biography, which permit the conclusion that H. Hertz died of Wegener's granulomatosis, 45 years before this disease had been explored. WEGENER'S GRANULOMATOSIS: The symptoms of the granulomatosis, that was first described by Friedrich Wegener in 1936 and 1939 are presented in short with literal quotations of the author: It begins with a refractory cold that will last until the end, then follow involvement of the paranasal sinuses, ears, mucous lining of mouth and pharynx, slight fever, weakness, in the final phase dissemination with nephritis, septic fever, arthritis, myalgia, paralyses leading to total immobility, exitus. The etiology is not fully understood but probably based on immunoreaction. H. HERTZ: CURRICULUM VITAE: Born in Hamburg, Germany, in 1857: after secondary school academic training in physics at Dresden, Munic and Berlin. 1885 professor of physics in Karlsruhe, then starting in 1889 at Bonn. 1887/88 exploration of the electromagnetic waves, now the basis for all radio communication, and exploration of the photoelectric effect, the basis of solar techniques. Numerous highranking distinctions. H. HERTZ: The case history is presented in detail based on numerous literal quotations from personal letters and the diary. After a few banal affections involving the teeth and toes the systemic disease became manifest in summer 1892 with a refractory cold which remained the focus of treatment until death. Granulation tissue in the nose that was excised several times: no tuberculosis, no carcinoma. Otitis with purulent mastoiditis, in October 1892 mastoidectomy, persistent purulence. Early in 1893 diagnosis of nephritis. Extraction of a superior tooth, punturing of the maxillary sinus, then twice radical opening of the maxillary sinus. Painful ankylosis and paralyses

  10. Pseudotumor pulmonar como apresentação inicial de granulomatose de Wegener Lung pseudotumor as the initial presentation of Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    Marcelo Fouad Rabahi

    2009-04-01

    Full Text Available A granulomatose de Wegener é uma doença inflamatória multissistêmica de etiologia desconhecida, que se caracteriza por vasculite de pequeno e médio calibre. As manifestações clínicas mais comuns envolvem o trato respiratório superior, pulmões e rins e as alterações encontradas nos exames de imagem são geralmente opacidades pulmonares ou nódulos múltiplos, bilaterais e, em 50% dos casos, cavitações. O tratamento é feito com corticoides e imunossupressores. Descreve-se um caso atípico de um homem de 61 anos cuja investigação inicial de um tumor pulmonar unilateral evidenciou, na verdade, Granulomatose de Wegener.Wegener's granulomatosis is a multisystemic inflammatory illness of unknown etiology, characterized by vasculitis of small and medium caliber vessels. The most common clinical manifestations involve the upper respiratory tract, lungs and kidneys. Common alterations in imaging studies include pulmonary opacities and bilateral multiple nodules, cavitations occurring in 50% of the cases. Treatment includes corticosteroids and immunosuppressants. We describe an atypical case of a 61-year-old man initially investigated due to suspicion of a unilateral lung tumor, which proved to be, in fact, a case of Wegener's granulomatosis.

  11. [A fatal case of Listeria monocytogenes sepsis and meningitis in a patient with Wegener's granulomatosis].

    Science.gov (United States)

    Tonkić, Marija; Grgić, Dusanka; Goić-Barisić, Ivana; Novak, Anita; Milas, Ivo; Bradarić, Nikola

    2006-12-01

    The bacillus Listeria monocytogenes is widely distributed in the environment. Listeria monocytogenes most often causes infection in the neonates, pregnant women, elderly and immunosuppressed persons. We report on a case of fatal sepsis and meningitis in a 59-year-old woman receiving cyclophosphamide and glucocorticoid therapy for Wegener's granulomatosis over a 10-year period. Listeriosis should be suspected in case of sepsis and/or meningitis in patients who receive immunosuppressive agents. Since meningitis due to Listeria monocytogenes is not distinguishable clinically from other types of bacterial meningitis, antibiotics against Listeria monocytogenes should be included in the initial empirical therapy of bacterial meningitis in immunosuppressed patients, antibiotics against Listeria monocytogenes should be included.

  12. Limited-Form Wegener Granulomatosis Case: Anaesthetic Approach and Literature Review.

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    Sarıtaş, Tuba Berra; Şahin, Osman; Borazan, Hale; Otelcioğlu, Şeref

    2014-12-01

    Wegener granulomatosis (WG) is a kind of vasculitis that affects small and medium-sized arteries. Necrotizing granulomatous vasculitis of the upper and lower respiratory tracts and necrotizing glomerulonephritis of the kidneys are present. WG affects mainly Caucasian individuals between 15-75 years old, with a mean age of onset of 41 years. It affects both males and females equally. Kidney involvement is not present in the limited form of WG. Peripheral nerve blocks are good alternatives when general anaesthesia is risky. Popliteal block is blockade of the sciatic nerve at the popliteal region. Popliteal block is a kind of peripheral block for surgeries below the knee level. In this article, we report on the anaesthesia management of a 61-year-old limited-form WG patient for whom general anaesthesia was risky because of lung involvement.

  13. Detailed analysis of graft techniques for nasal reconstruction following Wegener granulomatosis.

    Science.gov (United States)

    Sepehr, Ali; Alexander, Ashlin J; Chauhan, Nitin; Gantous, Andres

    2011-12-01

    Saddle nose deformity secondary to Wegener granulomatosis (WG) presents a significant challenge for the reconstructive surgeon. Various grafting options have been proposed, but achieving good outcomes can be difficult. We conducted a retrospective analysis of 10 patients with WG who underwent reconstruction of saddle nose deformities by the senior author (A.G.) between 2005 and 2009. All patients were reconstructed using costal cartilage grafts. Primary surgery was functionally and cosmetically successful in 8 of 10 patients. Two patients experienced complications (graft resorption and columellar necrosis) requiring revision surgery; subsequently, they achieved good outcomes, with no further complications. There were no complications at the costal cartilage donor site. At the conclusion of the study, all patients were satisfied with their reconstructions. With a multidisciplinary team approach to pre- and postoperative patient management and careful surgical technique, reliable and excellent functional and cosmetic outcomes can be achieved with costal cartilage grafts.

  14. Airway Surgery in Tracheostomised Patients with Wegener Granulomatosis Leading to Subglottic Stenosis.

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    Altun, Demet; Sivrikoz, Nükhet; Çamcı, Emre

    2015-10-01

    Wegener granulomatosis (WG) is a multisystemic disorder characterised by granulomatous inflammation of the respiratory system. The growing of proliferative tissue towards the larynx and trachea may cause airway obstruction on account of subglottic stenosis. In this situation, the surgical goal is to eliminate the airway obstruction by providing natural airway anatomy. While mild lesions do not require surgical intervention, in fixed lesions, surgical intervention is required, such as tracheostomy, laser resection and dilatation. In tracheostomised patients, granuloma formation surrounding the tracheostomy cannula may occur in the trachea. Inflammation and newly formed granulation tissue result in severe stenosis in the airways. During surgical treatment of such patients, airway management is important. In this case report, we will discuss gas exchange and airway management with jet ventilation (JV) during excision of the granulation tissue with endolaryngeal laser surgery, leading to subglottic stenosis in tracheostomised patients in WG.

  15. Wegener's Granulomatosis: A Model of Auto-antibodies in Mucosal Autoimmunity

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    Kelley, James M.; Edberg, Jeffrey C.; Kimberly, Robert P.

    2009-01-01

    Wegener's granulomatosis (WG) is an autoimmune condition marked by vasculitis of small and medium sized vessels particularly affecting the upper respiratory tract and kidneys. There is a strong mucosal component similar to other autoimmune conditions such as systemic lupus erythematosus and Behçet's disease. While the pathogenesis of WG is not completely known, auto-antibodies such as IgG ANCAs have been implicated in endovascular damage and modulation of neutrophil / monocyte responses by Fc receptor (FcR) signaling. Due to the substantial mucosal involvement in WG (oral, nasal, and upper respiratory tract involvement), it is probable that IgA antibodies (perhaps IgA ANCAs) play a role in disease. Given discrepancies in associating ANCA levels with disease activity, future work should determine if IgA ANCAs are present in WG patients and examine the biology underlying the ANCAs' signaling partners - the FcRs. PMID:19482554

  16. Pyoderma gangrenosum and Wegener granulomatosis-like syndrome induced by cocaine.

    Science.gov (United States)

    Jiménez-Gallo, D; Albarrán-Planelles, C; Linares-Barrios, M; Rodríguez-Hernández, C; Martínez-Rodríguez, A; García-Moreno, E; Bravo-Monge, R

    2013-12-01

    Cocaine abuse is associated with various skin and rheumatological diseases that mimic primary autoimmune diseases, including retiform purpura with involvement of the ears, cocaine-induced midline destructive lesions (CIMDL), and eruptive pyoderma gangrenosum (PG). Previous reports have suggested the use of perinuclear antineutrophil cytoplasmic antibodies (pANCA) with specificity against human neutrophil elastase (HNE) to differentiate these cocaine-induced diseases from primary autoimmune diseases. We describe a case of a 54-year-old woman with a history of cocaine abuse, who had PG lesions on her legs with accompanying CIMDL and lung lesions similar to those seen in Wegener granulomatosis. Detection of HNE-positive pANCA, and improvement or clinical recurrence after cessation or consumption of cocaine, respectively, were key to differentiating this presentation from primary autoimmune disease. © 2013 British Association of Dermatologists.

  17. [Tracheal resection for post-intubation subglottic stenosis in a patient with granulomatosis with polyangiitis (Wegener)].

    Science.gov (United States)

    Stoica, Radu; Negru, Irina; Matache, Radu; MirunaTodor

    2014-01-01

    Granulomatosis with polyangiitis (GPA or Wegener) is a systemic autoimmune disease with inflammation of small- and medium-size vessels. It can affect practically any organ or system, but renal, respiratory andjoint systems are most frequently damaged. Positive pANCA antibodies can raise the suspicion of diagnosis. Subglottic stenosis is relatively frequent, in a quarter of patients, especially in the third decade women. The case presented is of an 80-year-old woman, recently diagnosed with pulmonary, renal and systemic manifestations of GPA and with a subglottic stenosis rapidly evolving towards endotracheal intubation, tracheostomy with mechanical ventilation and renal failure. Further evolution has been favorable under corticoid therapy. After weaning from the mechanical ventilation and30 days after the suppression of the tracheostomy, the patient developed a tracheal stenosis with mixed etiology, secondary to vasculitis and prolonged intubation with tracheostomy. Tracheal resection with termino-terminal anastomosis was performed in emergency with simple post-operative evolution and without late complications.

  18. Meningeal involvement in Wegener granulomatosis: case report and review of the literature.

    Science.gov (United States)

    Soriano, A; Lo Vullo, M; Casale, M; Quattrocchi, C C; Afeltra, A

    2012-01-01

    Wegener Granulomatosis (WG) is a multisystem autoimmune disorder characterized by necrotizing granulomatous vasculitis that most commonly involves the upper respiratory tract, lungs, and kidneys. The involvement of the central nervous system (CNS) is infrequent and can cause stroke, cranial nerve abnormalities, cerebrovascular events, seizures, and meningeal involvement. Meningeal involvement is rare and may occur due to local vasculitis, directly spread from adjacent disease in the skull base, paranasal or orbital region. We describe the case of a 20-year-old Caucasian man who was diagnosed with sinonasal WG with frontal focal meningeal involvement. A literature review on diagnosis and treatment of meningeal involvement in course of WG was carried out. The importance of an early diagnosis and treatment of localized WG has been emphasized, in order to avoid the progression to a severe form of disease, especially in younger patients and in paucisymptomatic cases.

  19. ANCA-negative Wegener's granulomatosis with multiple lower cranial nerve palsies.

    Science.gov (United States)

    Kim, Sung-Hee; Park, Jin; Bae, Jung Ho; Cho, Min-Sun; Park, Kee Duk; Jeong, Jee Hyang

    2013-11-01

    Wegener's granulomatosis (WG) is a systemic vasculitis affecting small and medium-sized vessels with granulomatous formation. Though it is known for respiratory tract and kidney involvement, neurologic manifestation has been also reported. Herein we report a patient who suffered pansinusitis with multiple lower cranial nerve palsies but reached remission by immunosuppressant after the diagnosis of WG. A 54-yr-old female visited with headache, hearing difficulty, and progressive bulbar symptoms. She experienced endoscopic sinus surgeries due to refractory sinusitis. Neurologic examination revealed multiple lower cranial nerve palsies. Vasculitic markers showed no abnormality. Nasal biopsy revealed granulomatous inflammation and vasculitis involving small vessels. Given cyclophosphamide and prednisolone, her symptoms were prominently improved. WG should be considered in the patient with multiple cranial nerve palsies, especially those with paranasal sinus disease. Because WG can be lethal if delayed in treatment, prompt immunosuppressant is warranted after the diagnostic tissue biopsy.

  20. Cardiopatía valvular en un paciente con granulomatosis de Wegener

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    Luis E. Silva, MD

    2012-01-01

    Full Text Available La granulomatosis de Wegener es una vasculitis necrotizante autoinmune cuya prevalencia reportada es de 3/100.000 habitantes. Tiene compromiso multisistémico, principalmente el tracto respiratorio superior e inferior, y el sistema nervioso central y renal. La frecuencia del compromiso cardiaco varía según las series estudiadas, pero oscila alrededor de 6%; sin embargo, las manifestaciones clínicas son poco frecuentes. El compromiso valvular se reporta como insuficiencia aórtica o mitral, secundaria a infiltración de las valvas o dilatación de la raíz aórtica. El tratamiento se basa en el control de la enfermedad, el manejo de la falla cardiaca y la intervención quirúrgica de la válvula comprometida según la indicación.

  1. Wegener's granulomatosis and pyoderma gangrenosum--rare causes of facial ulcerations.

    Science.gov (United States)

    Kędzierska, Karolina; Sindrewicz, Krzysztof; Smektała, Tomasz; Wiśniewska, Magda; Masiuk, Marek; Staniszewska, Ewelina; Sporniak-Tutak, Katarzyna; Gołembiewska, Edyta; Ciechanowski, Kazimierz

    2016-03-16

    Pyoderma gangrenosum (PG) is caused by immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all PG patients also suffer from autoimmunological diseases, one of which is Wegener granulomatosis (WG). The purpose of this article was to compare cases of patients with WG and PG in terms of their clinical course, histopathology, and applied treatment. In both, histopathological features are not fully distinct. Data from microbiological and immunological evaluation and clinical presentation are required to establish the diagnosis. We also present the case of a patient with WG and deep facial skin lesions not responding to standard treatment. Systematic review of the literature in PubMed using the search terms "Wegener granulomatosis AND Pyoderma gangrenosum" and case report. The finding of 22 reports in the literature (PubMed) suggests that it is a rare phenomenon. This study revealed a similar rate of comorbidity of WG and PG in both genders and an increased incidence of both diseases after the age of 50. Among skin lesions there was a dominance of ulceration, most often deep and painful, covering a large area with the presence of advanced necrosis and destruction of the surrounding tissue. The most common location proved to be the cervical-cephalic area. The most popular treatment included steroids with cyclophosphamide. The rarity of the coexistence of these two diseases results in a lack of effective therapy. In such cases sulfone derivatives are still effective and provide an alternative to standard immunosuppression methods. Hyperbaric therapy and plasmapheresis can also play an important complementary role.

  2. Granulomatosis de Wegener Semejando Cáncer Epidermoide de Nasofaringe y Metástasis Pulmonares.

    Directory of Open Access Journals (Sweden)

    Gustavo Adolfo Martín Small

    2011-01-01

    Full Text Available La granulomatosis de Wegener (GW es una enfermedad idiopática, con posible componente autoinmune, que aparece generalmente en la quinta década de vida, caracterizándose por lesiones granulomatosas necrotizantes y vasculitis en vías aéreas y riñón. Paciente femenino de 54 años, quien desde Noviembre del 2008, presenta rinorrea, prurito y eritema en borde nasal inferior izquierdo, recibe antibióticos sin mejoría de los síntomas. En las radiografías torácicas, se observan dos radiopacidades redondeadas, de 4 cm de diámetro, sugestivas de lesiones tumorales en ambos campos pulmonares. La biopsia reporta cáncer epidermoide de alto grado, sospechándose primario en nasofaringe. Es remitida, por deterioro de condiciones, al Servicio de Neumonología del Hospital Universitario de Caracas el 18/03/2009, presentando disnea, tos productiva, placas purpúricas dolorosas en manos y pies, hipoacusia, hiperpigmentación del paladar duro, leucoplaquias y lesiones costrosas en lengua, insuficiencia renal (creatinina en 6,11 mg/dL y trombocitopenia. En TAC de tórax, se evidencian tumores mayores de 5 cm de diámetro, algunos con bordes bien definidos y otros mal delimitados con broncograma aéreo. Diagnostico definitivo de GW por serología. La GW con su afectación sistémica puede simular otras entidades como neoplasias de cabeza y cuello con metástasis pulmonares. Cuando las manifestaciones sistémicas son floridas debe sospecharse GW como diagnóstico diferencial. Palabras claves: Wegener, Granulomatosis, Vasculitis, Cáncer epidermoide.

  3. Granulomatosis with polyangiitis (Wegener granulomatosis) as a differential diagnosis of sternal osteomyelitis: the challenges in diagnosis.

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    Kim, Sang-Dong; Kim, Gun-Woo; Kim, Tae-Eun; Nam, Eun-Jung; Han, Seung-Woo

    2013-12-01

    Although granulomatosis with polyangiitis (GPA) can affect a large number of organ systems and produce a broad spectrum of clinical symptoms, skeletal involvement is very rare, with the exception of facial bone involvement associated with destructive nasal and sinus inflammation. We describe here a 54-year-old man with sternal osteomyelitis and destructive arthritis around the sternoclavicular joint. Despite antibiotics and conventional immunosuppressive treatment, his symptoms deteriorated, and a new mass-like lung lesion was developed. A histopathologic analysis of the lung mass revealed chronic granulomatous inflammation with fibrinoid necrosis, and he was diagnosed with GPA. When a patient with a destructive inflammatory lesion has negative culture results and no response to conventional therapy, we propose that an aggressive approach is necessary for a pathologic diagnosis to exclude the possibility of GPA.

  4. Gingival granulomatosis with polyangiitis (Wegener's granulomatosis) as a primary manifestation of the disease.

    Science.gov (United States)

    Fonseca, F P; Benites, B M; Ferrari, Alv; Sachetto, Z; de Campos, G V; de Almeida, O P; Fregnani, E R

    2017-03-01

    Granulomatosis with polyangiitis (GPA) is a potentially lethal disease characterized by systemic necrotizing vasculitis, which affects small- and medium-sized blood vessels and is often associated with serum cytoplasmic antineutrophil cytoplasmic antibody. The upper and lower respiratory tract and kidney are the most involved sites, but oral lesions can be identified in 6-13% of the cases, whereas in only 2% of the cases, oral manifestations represent the first signal of the disease usually as gingival swellings or unspecific ulcerations. Without treatment, the mainstay of which is the combination of immunosuppressants and systemic corticosteroids, GPA may run a fatal course. In this report we describe an original case of GPA affecting a 75-year-old female patient referred to our service due to a gingival swelling with 3-month duration. Although the patient was correctly diagnosed and promptly treated, she died 3 months after the initial diagnosis. © 2016 Australian Dental Association.

  5. Association study with Wegener granulomatosis of the human phospholipase Cγ2 gene

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    Csernok Elena

    2005-02-01

    Full Text Available Abstract Background Wegener Granulomatosis (WG is a multifactorial disease of yet unknown aetiology characterized by granulomata of the respiratory tract and systemic necrotizing vasculitis. Analyses of candidate genes revealed several associations, e.g. with α(1-antitrypsin, proteinase 3 and with the HLA-DPB1 locus. A mutation in the abnormal limb mutant 5 (ALI5 mouse in the region coding for the hydrophobic ridge loop 3 (HRL3 of the phospholipaseCγ2 (PLCγ-2 gene, corresponding to human PLCγ-2 exon 27, leads to acute and chronic inflammation and granulomatosis. For that reason, we screened exons 11, 12 and 13 coding for the hydrophobic ridge loop 1 and 2 (HRL1 and 2, respectively and exon 27 of the PLCγ-2 protein by single strand conformation polymorphism (SSCP, sequencing and PCR/ restriction fragment length polymorphism (RFLP analyses. In addition, we screened indirectly for disease association via 4 microsatellites with pooled DNA in the PLCγ-2 gene. Results Although a few polymorphisms in these distinct exons were observed, significant differences in allele frequencies were not identified between WG patients and respective controls. In addition, the microsatellite analyses did not reveal a significant difference between our patient and control cohort. Conclusion This report does not reveal any hints for an involvement of the PLCγ-2 gene in the pathogenesis of WG in our case-control study.

  6. Association study with Wegener granulomatosis of the human phospholipase Cγ2 gene

    Science.gov (United States)

    Jagiello, Peter; Wieczorek, Stefan; Yu, Philipp; Csernok, Elena; Gross, Wolfgang L; Epplen, Joerg T

    2005-01-01

    Background Wegener Granulomatosis (WG) is a multifactorial disease of yet unknown aetiology characterized by granulomata of the respiratory tract and systemic necrotizing vasculitis. Analyses of candidate genes revealed several associations, e.g. with α(1)-antitrypsin, proteinase 3 and with the HLA-DPB1 locus. A mutation in the abnormal limb mutant 5 (ALI5) mouse in the region coding for the hydrophobic ridge loop 3 (HRL3) of the phospholipaseCγ2 (PLCγ-2) gene, corresponding to human PLCγ-2 exon 27, leads to acute and chronic inflammation and granulomatosis. For that reason, we screened exons 11, 12 and 13 coding for the hydrophobic ridge loop 1 and 2 (HRL1 and 2, respectively) and exon 27 of the PLCγ-2 protein by single strand conformation polymorphism (SSCP), sequencing and PCR/ restriction fragment length polymorphism (RFLP) analyses. In addition, we screened indirectly for disease association via 4 microsatellites with pooled DNA in the PLCγ-2 gene. Results Although a few polymorphisms in these distinct exons were observed, significant differences in allele frequencies were not identified between WG patients and respective controls. In addition, the microsatellite analyses did not reveal a significant difference between our patient and control cohort. Conclusion This report does not reveal any hints for an involvement of the PLCγ-2 gene in the pathogenesis of WG in our case-control study. PMID:15703080

  7. Clinical features and outcomes of 37 Argentinean patients with severe granulomatosis with polyangiitis (wegener granulomatosis).

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    Orden, Alberto Omar; Muñoz, Sebastián Andrés; Basta, María Cristina; Allievi, Alberto

    2013-03-01

    Most epidemiologic studies involving severe granulomatosis with polyangiitis (SGPA) patients have investigated populations from the northern hemisphere, whereas few studies have been conducted in South America. None of the South American studies have differentiated between localized GPA and SGPA. The present study was designed to describe a cohort of Argentinean patients who were diagnosed with SGPA and to compare this cohort with previously well-described cohorts. We performed a retrospective study that included 37 consecutive SGPA patients who were seen at 2 tertiary centers in Buenos Aires. Nineteen patients (51.3%) were male, and 18 patients (49.7%) were female. The mean age at the onset of symptoms was 48.5 ± 12.01 years. Antineutrophil cytoplasmic antibody (ANCA) was detected in 34 patients (91.89%): 32 patients (86.48%) had a cytoplasmic staining pattern, whereas 2 patients (5.40%) had a perinuclear pattern. Three patients were ANCA-negative. Twenty-four patients (64%) achieved remission, and 7 patients (19%) had response as defined by at least 50% reduction in the disease activity score. Nineteen relapses were observed in 12 patients, and 2 of the relapses were fatal. Overall, there were 14 deaths (37.83%). The present series demonstrated that Argentinean patients have similar demographics, clinical manifestations, and outcomes as the cohorts from the northern hemisphere. There was less granulomatous organ involvement (ear/nose/throat, lung granulomas) in the present cohort compared with other series.

  8. Subglottic stenosis in granulomatosis with polyangiitis (Wegener's granulomatosis): Report of 4 cases.

    Science.gov (United States)

    Horta-Baas, Gabriel; Hernández-Cabrera, María Fernanda; Catana, Rocío; Pérez-Cristóbal, Mario; Barile-Fabris, Leonor Adriana

    2016-01-01

    Subglottic stenosis (SGS) in granulomatosis with polyangiitis (GPA) may result from active disease or from chronic recurrent inflammation. The objective of the study was to describe the clinical features and treatment of patients with subglottic stenosis. We retrospectively reviewed the medical records of all patients with SGS due to GPA diagnosed at Rheumatology deparment between January 2000 and June 2015. We present 4 cases of SGS at our department during a period of 15 years. The interval between the presentation of the GPA and SGS varied between 2 and 144 months. The leading symptoms of SGS were dyspnoea on exertion and stridor. Three patients presented SGS without evidence of systemic activity. Two patients presented SGS grade i and received tracheal dilatation; two recurred and three needed a tracheostomy due to severe airway-limiting stenosis. SGS presents high morbidity. Even though subglottic dilatation provides symptomatic relief, recurrences may present. Severe airway-limiting stenosis often requires tracheostomy. Copyright © 2015 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  9. The presentation and management of granulomatosis with polyangiitis (Wegener's Granulomatosis) in the pediatric airway.

    Science.gov (United States)

    Lee, Pui Y; Adil, Eelam A; Irace, Alexandria L; Neff, Laura; Son, Mary Beth F; Lee, Edward Y; Perez-Atayde, Antonio; Rahbar, Reza

    2017-01-01

    Granulomatosis with polyangiitis (GPA) is a necrotizing inflammatory disease that can affect the airway. The purpose of this study was to present a case of pediatric laryngotracheal GPA and provide management recommendations based on a thorough review of the literature. Retrospective chart and literature review REVIEW METHODS: A pediatric patient with laryngotracheal and pulmonary manifestations of GPA who underwent chemotherapy and intralesional corticosteroid injection is described. An extensive literature review of pediatric GPA affecting the larynx/trachea was also performed. A pediatric patient presented with acute respiratory distress. Flexible laryngoscopy revealed a laryngeal mass. Magnetic resonance imaging showed circumferential subglottic stenosis, and chest computed tomography demonstrated multiple pulmonary nodules. Laryngeal and tracheal biopsy revealed granulation tissue and primary vasculitis. Labs demonstrated positive cytoplasmic antineutrophil cytoplasmic antibody, consistent with GPA. Methylprednisone, rituximab, cyclophosphamide, and intralesional steroid injection resulted in remission after 12 weeks. Review of the literature revealed two pediatric cases series and 10 case reports of GPA affecting the larynx or trachea. There is a higher prevalence of GPA of the airway in children when compared to adults. Biopsy of the airway lesion may not be necessary and has lower diagnostic yield compared to other GPA subsites. Medical management includes induction therapy followed by maintenance therapy once the disease is in remission. Most patients will require a surgical intervention to maintain the airway. NA Laryngoscope, 127:233-240, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  10. Role of gingival manifestation in diagnosis of granulomatosis with polyangiitis (Wegener's granulomatosis).

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    Sung, Iel-Yong; Kim, Young-Min; Cho, Yeong-Cheol; Son, Jang-Ho

    2015-12-01

    This report describes a case of granulomatosis with polyangiitis (GPA) in which the gingival manifestation was crucial in both making an early diagnosis and possibly in deciding the approach to treatment. A 57-year-old sailor presented to the Department of Dentistry at Ulsan University Hospital complaining of gingival swelling since approximately 2 months. He had orofacial granulomatous lesions and the specific gingival manifestation of strawberry gingivitis. The diagnosis of GPA was made on the basis of clinical symptoms and signs, and confirmed by the presence of the anti-neutrophil cytoplasmic antibody and a positive biopsy. The patient was admitted to the hospital and subsequently placed on a disease-modifying therapy regimen that included methotrexate and prednisone. Identification of the gingival manifestation of the disease permitted an early diagnosis and prompt therapy in a disease in which time is a crucial factor. Because of its rapid progression and potentially fatal outcome, an early diagnosis of GPA is important. Therefore, dentists should be aware of the oral signs and symptoms of such systemic diseases.

  11. Chronic thrombotic microangiopathy secondary to chemotherapy for urothelial carcinoma in a patient with a history of Wegener granulomatosis.

    Science.gov (United States)

    Thomas, Jacob G; Sethi, Sanjeev; Norby, Suzanne M

    2011-05-01

    We present the case of a 62-year-old man with a history of Wegener granulomatosis who developed chronic thrombotic microangiopathy attributed to gemcitabine chemotherapy. Wegener granulomatosis had been diagnosed 15 years earlier, and the patient was treated using cyclophosphamide and prednisone, then maintained on mycophenolate mofetil and prednisone. Four years previously, he had been treated with mitomycin C for urothelial carcinoma and at the time of presentation had developed significant anemia and thrombocytopenia after a course of gemcitabine and carboplatin due to metastasis of the carcinoma. He was managed using red blood cell and platelet transfusions but then developed acute kidney injury, along with progressive dyspnea and pulmonary infiltrates. Imaging studies showed bilateral ureteral obstruction requiring placement of nephrostomy tubes. Because of concern about a flare of Wegener granulomatosis after withdrawing maintenance immunosuppression in the context of the malignancy, a kidney biopsy was performed that showed chronic thrombotic microangiopathy, likely secondary to gemcitabine chemotherapy. Clinical, laboratory, and pathologic findings of this case are discussed to illustrate the natural history of thrombotic microangiopathy associated with gemcitabine chemotherapy. Copyright © 2011 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

  12. Clinical manifestations and treatment of idiopathic and Wegener granulomatosis-associated subglottic stenosis.

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    Taylor, Stanford C; Clayburgh, Daniel R; Rosenbaum, James T; Schindler, Joshua S

    2013-01-01

    To compare and contrast the manifestations and surgical management of subglottic stenosis in patients with airway obstruction attributed to granulomatosis with polyangiitis (GPA), previously known as Wegener granulomatosis, and those with idiopathic subglottic stenosis (iSGS). Retrospective medical chart review. Review of subglottic stenosis cases seen in the otolaryngology department of an academic medical center from 2005 through 2010. Data were obtained on disease presentation, operative management. and findings. Tertiary referral center. A total of 24 patients with iSGS and 15 patients with GPA-associated subglottic stenosis (GPA-SGS). All individuals with iSGS were female, and 40% of patients with GPA-SGS were male (P < .01). Patients with iSGS tended to have a higher Myer-Cotton stenosis grade at the time of dilation than those with GPA-SGS (P = .02). Individuals with GPA-SGS were more likely to undergo tracheotomy as a result of disease-related complications than individuals with iSGS (P < .01). No patients with an open airway reconstruction in the iSGS group required follow-up mechanical dilation. In contrast, all patients with open airway reconstructions in the GPA-SGS group underwent more than 1 subsequent airway dilation (P < .01). While surgical utilization is the mainstay of treatment in iSGS and GPA-SGS, iSGS occurs almost exclusively in females and presents with a greater degree of stenosis at the time of endoscopic dilation. In contrast, GPA-SGS is associated with greater rates of tracheotomy. Open airway reconstruction may be used in the treatment of iSGS and GPA-SGS but is much more effective in iSGS.

  13. Comprometimento da árvore respiratória na granulomatose de Wegener Laryngeal and tracheobronchial involvement in Wegener's granulomatosis

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    Ascedio Jose Rodrigues

    2012-04-01

    Full Text Available INTRODUÇÃO: A granulomatose de Wegener (GW é uma forma de vasculite sistêmica que envolve primariamente as vias aéreas superiores e inferiores e os rins. As manifestações mais frequentes nas vias aéreas são estenose subglótica e inflamações, estenoses da traqueia e dos brônquios. A visualização endoscópica das vias aéreas é a melhor ferramenta para avaliação, diagnóstico e manejo dessas alterações. OBJETIVOS: Descrever as alterações endoscópicas encontradas na mucosa das vias aéreas de um grupo de pacientes com GW submetido à broncoscopia no Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP e relatar as intervenções terapêuticas broncoscópicas utilizadas em alguns casos. MÉTODOS: Foram estudados 15 pacientes com diagnóstico de GW provenientes do Ambulatório de Vasculites da Disciplina de Pneumologia do HC-FMUSP, encaminhados para a realização de broncoscopia no serviço de Endoscopia Respiratória do HC-FMUSP no período de 2003 a 2007. RESULTADOS: Dos 15 pacientes avaliados, 11 eram mulheres (73,33% com idade média de 34 ± 11,5 anos. Foram encontradas alterações das vias aéreas em 80% dos pacientes, e o achado endoscópico mais frequente foi estenose subglótica (n = 6. Realizou-se broncoscopia terapêutica em três pacientes com estenose subglótica e em outros três com estenose brônquica, todos apresentando bons resultados. CONCLUSÃO: A broncoscopia permite diagnóstico, acompanhamento e tratamento das lesões de vias aéreas na GW, constituindo-se um recurso terapêutico pouco invasivo em casos selecionados.INTRODUCTION: Wegener's granulomatosis (WG is a form of systemic vasculitis that involves primarily the upper and lower airways and the kidneys. The most frequent airway manifestations include subglottic stenosis and inflammation, and tracheal and bronchial stenoses. The endoscopic visualization of the airways is the best tool for assessing, diagnosing and

  14. Wegener's granulomatosis: report of a case with rapidly progressive glomerulonephritis and diabetes mellitus.

    Science.gov (United States)

    Kakoi, H; Hiraide, F; Nishizawa, S; Inouye, T; Yoshizawa, N

    1986-01-01

    A patient with the classic form of Wegener's granulomatosis with severe dabetes mellitus and rapidly progressive glomerulonephritis is described. This 61-year-old male presented with epistaxis and nasal pain and obstruction. The nasal cavities were filled with crusts covering eroded mucosa. The diagnosis was made by biopsy of nasal and bronchial mucosa, and laboratory data. The epistaxis was stopped by 10 Gy irradiation over the nasal cavities. The patient had severe diabetes mellitus. His blood sugar was not controlled by diet and insulin injection. His general condition worsened rapidly as the growth of granuloma in the nose and lung. Accordingly, prednisolone therapy reinitiated to suppress the granuloma although it has a reverse effect on diabetes mellitus. Approximately one month after admission, he died of acute renal failure. Autopsy was carried out. Granulomatous lesions were noted in the nasal cavities, lungs and spleen. Many petechiae were found macroscopically over the cortex of the kidney. Hyalinization or sclerosis with crescent formation was found microscopically in estimated 85% of the glomeruli. Immunohistologic analysis of the renal tissue demonstrated an irregular linear pattern deposition of IgG, IgA and C3 and a granular pattern deposition of IgM and C1q.

  15. The interface of inflammation and subclinical atherosclerosis in granulomatosis with polyangiitis (Wegener's): a preliminary study.

    Science.gov (United States)

    Hajj-Ali, Rula A; Major, Jennifer; Langford, Carol; Hoffman, Garry S; Clark, Tiffany; Zhang, Li; Sun, Zhiyuan; Silverstein, Roy L

    2015-10-01

    The objective of this study is to assess the relationship between inflammatory disease in granulomatosis with polyangiitis (GPA, Wegener's) and the development of subclinical atherosclerosis. A total of 46 adult patients with GPA were enrolled. Disease status was measured by Birmingham vasculitis assessment scores as modified for GPA, vasculitis damage index, disease duration, and number of relapses. Classic atherosclerotic risk factors, platelet aggregation responses, and circulating microparticle (MP) levels were recorded. All patients underwent carotid artery intima-media thickness (IMT) measurement as outcome for subclinical atherosclerosis. In univariate analyses, systolic and diastolic blood pressure, creatinine, and age were significantly associated with higher IMT (ρ values 0.37, 0.38, 0.35, and 0.054, respectively [P subclinical atherosclerosis. The correlation with subclinical atherosclerosis could be because of glucocorticoid use and not the inflammatory process in GPA, giving the inherent bias that exits with the use of glucocorticoid with each relapse. The findings of increased levels of circulating leukocyte-derived MPs and enhanced platelet reactivity during relapse suggest possible roles for MPs and platelets in disease pathogenesis and support a growing literature that links inflammation, atherosclerosis, and platelet activation. This hypothesis is further substantiated by our demonstration that MPs isolated from plasma of GPA patients can activate platelets and vascular endothelial cells. Copyright © 2015 Elsevier Inc. All rights reserved.

  16. Otomastoiditis como manifestación clínica inicial de granulomatosis de Wegener

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    Jorge De All

    2011-02-01

    Full Text Available La granulomatosis de Wegener (GW forma parte del grupo de vasculitis primarias, de vasos pequeños y medianos, asociadas a anticuerpos anticitoplasma de neutrófilos (ANCA. Esta enfermedad puede afectar cualquier órgano, especialmente vías aéreas superiores, inferiores y el riñón. Muy raramente la primera y única manifestación clínica de GW generalizada es la otomastoiditis. Presentamos el caso de un paciente que inició su enfermedad con una inflamación del oído medio, sin respuesta al tratamiento habitual. Posteriormente agregó compromiso nasal, pulmonar y renal. La determinación de anticuerpos anticitoplasma de neutrófilos y la biopsia transbronquial confirmaron el diagnóstico de GW. Comunicamos este caso para referir que la otomastoiditis del adulto, refractaria al tratamiento habitual, puede raramente ser la primera y única manifestación clínica de la GW.

  17. [A case of Wegener granulomatosis after treatment for non-tuberculous mycobacteriosis].

    Science.gov (United States)

    Fukusumi, Munehisa; Miyazaki, Kenji; Shibata, Masahiko; Aizawa, Toyoaki; Sakai, Toshihiko; Takemura, Tamiko; Tojima, Hirokazu

    2011-01-01

    A 67-year-old woman with a history of non-tuberculous mycobacteriosis due to Mycobacterium avium in 2002 and 2006 was admitted because of low-grade fever and cavitary lesions in both lungs on chest computed tomography (CT). Bronchial brushing cytology by bronchoscopy did not yield mycobacterium. Her serum antineutrophil cytoplasmic antibodies (MPO-ANCA) level was elevated and PR3 ANCA was negative. A surgical lung biopsy revealed basophilic necrosis with cavitary lesions and necrotizing vasculitis compatible with Wegener granulomatosis (WG). In the resected specimen, several hyalinizing nodules with caseous necrosis, probably associated with previous mycobacterium infection, were also seen. Because her disease was limited to the lungs, we successfully treated her with corticosteroids and methotrexate. WG is considered to be a type of ANCA-associated vasculitis, and microbial infections have been associated with initiation and relapse of WG. This case clearly demonstrated that lesions of WG occurred after mycobacterial infection in the lung. We believe that this case was ANCA production in association with bacterial infection.

  18. Sinonasal Wegener granulomatosis: a single-institution experience with 120 cases.

    Science.gov (United States)

    Cannady, Steven B; Batra, Pete S; Koening, Curry; Lorenz, Robert R; Citardi, Martin J; Langford, Carol; Hoffman, Gary S

    2009-04-01

    Wegener granulomatosis (WG) is intimately associated with the sinonasal tract, with involvement reported in 85% of patients during the course of the disease process. The objectives of the study were: 1) to describe sinonasal symptoms and signs at the time of initial otolaryngologic evaluation; and 2) to review indications and outcomes for rhinologic surgery in WG at our institution. A retrospective analysis of 120 patients presenting with WG and referred for otolaryngology consultation at a tertiary care center was performed. Eighty-nine percent of patients exhibited sinonasal involvement including nasal crusting (69%), chronic rhinosinusitis (CRS) (61%), nasal obstruction (58%), bloody nasal discharge (52%), septal perforation (33%), saddle-nose deformity (23%), epiphora (13%), and mucocele formation (3.3%). Thirty-nine (33%) patients underwent primary functional endoscopic sinus surgery or dacryocystorhinotomy (DCR) prior to referral. Nineteen (16%) patients underwent noncosmetic, functional sinonasal procedures at our institution. These included endoscopic sinus surgery for CRS or mucocele decompression in 14 patients, DCR for chronic epiphora in seven patients, and orbital decompression for pseudotumor in three patients. Individual symptom recurrence within the first year was greatest for visual impairment from pseudotumor (50%), followed by CRS (14%) and nasolacrimal duct obstruction (11%). The majority of patients with WG are referred to otolaryngology because of involvement of the sinonasal tract. Noncosmetic functional sinonasal procedures are indicated in a minority of patients. Sinonasal morbidity remains significant even after surgery. It is greatest for orbital pseudotumor, but also common for CRS and nasolacrimal duct obstruction.

  19. Structure, chromosomal assignment, and expression of the gene for proteinase-3. The Wegener's granulomatosis autoantigen.

    Science.gov (United States)

    Sturrock, A B; Franklin, K F; Rao, G; Marshall, B C; Rebentisch, M B; Lemons, R S; Hoidal, J R

    1992-10-15

    Proteinase-3 (PR-3) is a neutral serine proteinase present in the azurophil granules of human polymorphonuclear leukocytes. It degrades a variety of extracellular matrix proteins including elastin in vitro and causes emphysema when administered by tracheal insufflation to hamsters. It is identical to the target autoantigen (c-ANCA) associated with Wegener's granulomatosis and to myeloblastin, a serine proteinase first identified in HL-60 leukemia cells. In this study, the gene encoding PR-3 was cloned and sequenced. The gene spans approximately 6.5 kilobase pairs and consists of five exons and four introns. The genomic organization of PR-3 is similar to that of the other serine proteinases expressed in hemopoietic cells. Each residue of the catalytic triad of PR-3 is located on a separate exon, and the positions of the residues within the exons are similar to those in human leukocyte elastase and cathepsin G. The phase and placement of the introns in the PR-3 gene are also similar to those in human leukocyte elastase and cathepsin G. The 400-base pair (bp) 5'-flanking sequence of the PR-3 gene contains a TATA box at position 379. There is no CAAT box promoter element. The 3'-untranslated region is 200 bp, extending from a TGA stop codon to the site of polyadenylation 10 bp after the canonical AATAAA signal. Amplification of PR-3 from a human/hamster hybrid cell line localizes the gene to human chromosome 19. Evidence from Northern analysis suggests that PR-3 expression is primarily confined to the promyelocytic/myelocytic stage of bone marrow development.

  20. Urogenital manifestations in Wegener granulomatosis: a study of 11 cases and review of the literature.

    Science.gov (United States)

    Dufour, Jean-François; Le Gallou, Thomas; Cordier, Jean-François; Aumaître, Olivier; Pinède, Laurent; Aslangul, Elisabeth; Pagnoux, Christian; Marie, Isabelle; Puéchal, Xavier; Decaux, Olivier; Dubois, Alain; Agard, Christian; Mahr, Alfred; Comoz, François; Boutemy, Jonathan; Broussolle, Christiane; Guillevin, Loïc; Sève, Pascal; Bienvenu, Boris

    2012-03-01

    We describe the main characteristics and treatment of urogenital manifestations in patients with Wegener granulomatosis (WG). We conducted a retrospective review of the charts of 11 patients with WG. All patients were men, and their median age at WG diagnosis was 53 years (range, 21-70 yr). Urogenital involvement was present at onset of WG in 9 cases (81%), it was the first clinical evidence of WG in 2 cases (18%), and was a symptom of WG relapse in 6 cases (54%). Symptomatic urogenital involvement included prostatitis (n = 4) (with suspicion of an abscess in 1 case), orchitis (n = 4), epididymitis (n = 1), a renal pseudotumor (n = 2), ureteral stenosis (n = 1), and penile ulceration (n = 1). Urogenital symptoms rapidly resolved after therapy with glucocorticoids and immunosuppressive agents. Several patients underwent a surgical procedure, either at the time of diagnosis (n = 3) (consisting of an open nephrectomy and radical prostatectomy for suspicion of carcinoma, suprapubic cystostomy for acute urinary retention), or during follow-up (n = 3) (consisting of ureteral double J stents for ureteral stenosis, and prostate transurethral resection because of dysuria). After a mean follow-up of 56 months, urogenital relapse occurred in 4 patients (36%). Urogenital involvement can be the first clinical evidence of WG. Some presentations, such as a renal or prostate mass that mimics cancer or an abscess, should be assessed to avoid unnecessary radical surgery. Urogenital symptoms can be promptly resolved with glucocorticoids and immunosuppressive agents. However, surgical procedures, such as prostatic transurethral resection, may be mandatory in patients with persistent symptoms.

  1. Results of endoscopic surgery and intralesional steroid therapy for airway compromise due to tracheobronchial Wegener's granulomatosis

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    S. A. R. Nouraei

    2008-04-01

    Full Text Available Background: Upper airway compromise due to tracheobronchial stenosis commonly occurs in patients with Wegener's Granulomatosis (WG. There is at present no consensus on the optimal management of this life-threatening condition. Objective: To assess the results of laryngo-tracheo-bronchoscopy, intralesional steroid therapy, laser surgery, and dilatation in managing obstructive tracheobronchial WG. Methods: Records of eighteen previously-untreated stridulous patients with obstructive tracheobronchial WG, treated between 2004 and 2006 were prospectively recorded on an airway database and retrospectively reviewed. Information about patient and lesion characteristics and treatment details were recorded. Treatment progress was illustrated using a timeline plot, and intervention-free intervals were calculated with actuarial analysis. Results: There were nine males and the average age at presentation was 40 (16 years [range 13–74]. There were thirteen patients with tracheal, and five patients with tracheal and bronchial lesions. The average tracheal lesion height was 8 (3 mm, located 23 (9 mm below the glottis. There were 1, 10 and 7 Myer-Cotton grade I, II and III lesions respectively. Mean intervention-free interval following minimally-invasive treatment was 26 (2.8 months. Following endobronchial therapy the median intervention-free interval was 22 months (p>0.8 vs. tracheal lesions. No patient required a tracheostomy or endoluminal stenting. Conclusions: Intralesional steroid therapy and conservative endoluminal surgery is an effective strategy for treating airway compromise due to active tracheal and bronchial WG, obviating the need for airway bypass or stenting. We recommend the combination of endotracheal dilatation, conservative laser surgery and steroid therapy as the standard of care for treating airway compromise due to obstructive tracheobronchial WG.

  2. Two-dimensional speckle-tracking echocardiography reveals systolic abnormalities in granulomatosis with polyangiitis (Wegener's).

    Science.gov (United States)

    Miszalski-Jamka, Tomasz; Szczeklik, Wojciech; Nycz, Krzysztof; Sokołowska, Barbara; Górka, Jacek; Bury, Krzysztof; Musiał, Jacek

    2012-08-01

    Two-dimensional speckle-tracking echocardiography (STE) is a novel technique providing accurate assessment of myocardial function. However, its value in granulomatosis with polyangiitis (Wegener's) (WG) has not been studied. To assess the presence and frequency of systolic left ventricular (LV) dysfunction using STE and to determine incremental value of STE over standard echocardiography to detect myocardial abnormalities in WG. Twenty-two WG patients (11 males, 11 females, mean age 46.8 ± 12.3 years) and 22 sex- and age-matched healthy subjects underwent standard and STE. Global longitudinal, circumferential, and rotational deformation parameters were calculated. All patients had LV ejection fraction (EF) >50%. LVEF was 65.0 ± 7.5% and LV end-diastolic volume index 44.8 ± 11.8 mL/m(2) . Regional LV wall motion abnormalities were found in 7 (32%), while abnormal global STE determined systolic dysfunction in 16 (73%) subjects (P = 0.008). Global longitudinal, circumferential and radial peak-systolic deformational parameters (strain or strain rate) were decreased in 11 (50%), 9 (41%), and 3 (14%) patients (P = 0.02), respectively. Comparing patients with abnormal and normal STE derived global systolic function, the former had higher cumulative disease extent index (10.6 ± 3.0 vs 7.5 ± 1.8; P = 0.03) and vasculitis damage index (7.9 ± 1.9 vs 6.0 ± 1.7; P = 0.04). Despite normal LVEF the global systolic LV abnormalities detected by STE are common in WG. They correspond to the extent and severity of WG and are more frequent than regional wall motion abnormalities in standard echocardiography. © 2012, Wiley Periodicals, Inc.

  3. Granulomatosis de Wegener Semejando Cáncer Epidermoide de Nasofaringe y Metástasis Pulmonares.

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    Gustavo Adolfo Martín Small

    2009-01-01

    Full Text Available La granulomatosis de Wegener (GW es una enfermedad idiopática, con posible componente autoinmune, que aparece generalmente en la quinta década de vida, caracterizándose por lesiones granulomatosas necrotizantes y vasculitis en vías aéreas y riñón. Paciente femenino de 54 años, quien desde Noviembre del 2008, presenta rinorrea, prurito y eritema en borde nasal inferior izquierdo, recibe antibióticos sin mejoría de los síntomas. En las radiografías torácicas, se observan dos radiopacidades redondeadas, de 4 cm de diámetro, sugestivas de lesiones tumorales en ambos campos pulmonares. La biopsia reporta cáncer epidermoide de alto grado, sospechándose primario en nasofaringe. Es remitida, por deterioro de condiciones, al Servicio de Neumonología del Hospital Universitario de Caracas el 18/03/2009, presentando disnea, tos productiva, placas purpúricas dolorosas en manos y pies, hipoacusia, hiperpigmentación del paladar duro, leucoplaquias y lesiones costrosas en lengua, insuficiencia renal (creatinina en 6,11 mg/dL y trombocitopenia. En TAC de tórax, se evidencian tumores mayores de 5 cm de diámetro, algunos con bordes bien definidos y otros mal delimitados con broncograma aéreo. Diagnostico definitivo de GW por serología. La GW con su afectación sistémica puede simular otras entidades como neoplasias de cabeza y cuello con metástasis pulmonares. Cuando las manifestaciones sistémicas son floridas debe sospecharse GW como diagnóstico diferencial.

  4. Patients' perceptions of the effects of systemic lupus erythematosus on health, function, income, and interpersonal relationships : A comparison with Wegener's granulomatosis

    NARCIS (Netherlands)

    Boomsma, MM; Bijl, M; Stegeman, CA; Kallenberg, CGM; Hoffman, GS; Tervaert, JWC

    2002-01-01

    Objective. To describe the patients' perceptions of the effects of systemic lupus erythematosus (SLE) and Wegener's granulomatosis (WG) on health, function, income, and interpersonal relationships. Methods. 114 patients with SLE, and 79 patients with WG completed a self-administered questionnaire.

  5. The prevalence of the Staphylococcus aureus tst gene among community- and hospital-acquired strains and isolates from Wegener's Granulomatosis patients

    NARCIS (Netherlands)

    Deurenberg, Ruud H; Nieuwenhuis, Rutger F; Driessen, Christel; London, Nancy; Stassen, Frank R; van Tiel, Frank H; Stobberingh, Ellen E; Vink, Cornelis

    2005-01-01

    To allow rapid identification of toxic shock syndrome toxin-1 (TSST-1)-producing Staphylococcus aureus strains, a real-time PCR assay for the detection of the tst gene, which encodes TSST-1, was developed. The assay was applied to S. aureus isolates from patients with Wegener's Granulomatosis (WG),

  6. Esclerite como manifestação inicial de granulomatose de Wegener: descrição de caso Wegener's granulomatosis presenting as scleritis: case report

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    Leonardo Schmidt

    2007-04-01

    Full Text Available Esclerite é um processo inflamatório ocular caracterizado por edema e infiltração celular da esclera, que cursa com vermelhidão e dor locais e causa perda importante da acuidade visual. Pode ser a primeira manifestação de uma doença reumática sistêmica como lúpus, artrite reumatóide e policondrite recidivante, embora seja mais comum que apareça bastante tempo depois da doença já estabelecida. Os autores apresentam um caso de granulomatose de Wegener no qual essa forma de envolvimento ocular precedeu o aparecimento de glomerulonefrite em 18 meses.Scleritis is an ocular inflammatory condition characterized by edema and cellular infiltration of the sclera that causes local redness, pain and loss of visual acuity. It can be the first manifestation of a systemic rheumatic disease such as lupus, rheumatoid arthritis and relapsing polycondritis, although it is more frequently found in a well established disease. The authors describe a case of Wegener's granulomatosis where this form of ocular involvement preceded the glomerulonephritis by eighteen months.

  7. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation

    DEFF Research Database (Denmark)

    Ruperto, Nicolino; Ozen, Seza; Pistorio, Angela

    2010-01-01

    To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria.......To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria....

  8. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria

    DEFF Research Database (Denmark)

    Ozen, Seza; Pistorio, Angela; Iusan, Silvia M

    2010-01-01

    To validate the previously proposed classification criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA).......To validate the previously proposed classification criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA)....

  9. Progressive multifocal encephalopathy after cyclophosphamide in granulomatosis with polyangiitis (Wegener) patients: case report and review of literature.

    Science.gov (United States)

    Pugnet, G; Pagnoux, C; Bézanahary, H; Ly, K H; Vidal, E; Guillevin, L

    2013-01-01

    Progressive multifocal encephalopathy (PML) is a rare demyelinating disorder targeting the central nervous system and resulting from JC virus reactivation. PML occurs in patients immunocompromised because of haematological malignancies, HIV infection or treatment with cytotoxic drugs. Herein, we describe PML occurring in 2 granulomatosis with polyangiitis (Wegener) patients treated with steroids and cyclophosphamide. The outcome was progressively favourable after immunosuppressant discontinuation for 1 patient and fatal for the other. Four previously reported GPA patients developed PML in the course of their disease. One of them improved gradually after immunosuppressant withdrawal. PML should be strongly suspected whenever unusual central neurological manifestations appear in this context. No effective treatment is available, but immunosuppressants should be discontinued if possible.

  10. 18F-FDG PET/CT findings preceded elevation of serum proteinase 3 antineutrophil cytoplasmic antibodies in Wegener granulomatosis.

    Science.gov (United States)

    Ito, Kimiteru; Minamimoto, Ryogo; Yamashita, Hiroyuki; Morooka, Miyako; Okasaki, Momoko; Mimori, Akio; Kubota, Kazuo

    2014-01-01

    A 67-year-old woman underwent F-FDG PET/CT after developing a fever of unknown origin. PET/CT revealed intensive FDG uptake at the nasal and lung lesions. On the laboratory data, serum myeloperoxidase antineutrophil cytoplasmic antibodies (ANCA) titer was elevated, although serum directed against proteinase 3 (PR3) ANCA titer was within normal limits. One month after treatment, follow-up PET/CT revealed decreased FDG uptake at the lesions. One year later, serum PR3-ANCA titer elevated, which finally led to a diagnosis of Wegener granulomatosis (WG). WG lesions may be detected earlier by FDG PET/CT than by serum PR3-ANCA titers.

  11. Intense FDG uptake on PET/CT in the upper and lower respiratory system indicative of Wegener's granulomatosis.

    Science.gov (United States)

    Aydin, M; Akkoyunlu, M E; Yakar, F; Ergul, N; Sezer, M

    2014-01-01

    Wegener's granulomatosis (WG) is an uncommon systemic vasculitis, which involves the upper and lower respiratory tracts and the kidneys. Because the patients generally present with clinical manifestations that are similar to common diseases, WG may be initially misdiagnosed as infection or malignancy. We report the case of a 55-year-old male presenting with weight loss, cough, hemoptysis, low-grade fever, and pulmonary nodules detected on the thoracic CT scan. Malignancy was initially suspected, so a PET/CT was performed. It demonstrated intense FDG uptake in the upper and lower respiratory system. The diagnosis of WG was based on PET findings, elevated serum levels of inflammatory markers, and the presence of c-ANCA. We consider that the knowledge of FDG-PET/CT findings may help to make an easier and earlier diagnosis of WG. Copyright © 2013 Elsevier España, S.L. and SEMNIM. All rights reserved.

  12. Tumour necrosis factor-alpha (TNF), lymphotoxin and TNF receptor levels in serum from patients with Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Jónasdóttir, O; Petersen, J; Bendtzen, K

    2001-01-01

    Wegener's granulomatosis (WG) is a systemic inflammatory disease with vasculitis as the key feature. Abnormal expression of tumour necrosis factor alpha (TNFalpha) is considered of prime pathogenic importance in several inflammatory diseases. The effects of TNFa are mediated by TNF receptors (TNF......-R), and these receptors are often found in soluble forms (sTNF-R), which can modulate TNFalpha actions. To evaluate the clinical importance of the TNF family of cytokines, the serum levels of TNFalpha, TNFbeta, now termed lymphotoxin (LTalpha), and sTNF-R1 and sTNF-R2 were measured by ELISA in 8 patients with WG during...... of the relative amounts of TNFalpha and sTNF-R indicated that TNFalpha was mostly bound to its soluble receptors. In WG, the serum levels of sTNF-R1 and sTNF-R2 were dramatically increased (p...

  13. Serial High-Resolution Computed Tomography Imaging in Patients with Wegener Granulomatosis: Differentiation Between Active Inflammatory and Chronic Fibrotic Lesions

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    Lohrmann, C.; Uhl, M.; Schaefer, O.; Ghanem, N.; Kotter, E.; Langer, M. [Univ. Hospital of Freiburg (Germany). Dept. of Radiology

    2005-08-01

    PURPOSE: To evaluate pulmonary pathologies in Wegener granulomatosis with sequential computed tomography (CT) in order to differentiate active inflammatory lesions from chronic fibrotic lesions. MATERIAL AND METHODS: Serial CT findings in 38 patients with Wegener granulomatosis were retrospectively analyzed (mean follow-up period, 21 months). The presence, extension, and distribution of the following findings were evaluated with CT: parenchymal nodules, masses, ground-glass attenuation, airspace consolidation, bronchial wall-thickening, bronchiectasis, linear areas of attenuation, pleural irregularities, pleural effusions, hilar and mediastinal lymphadenopathy. RESULTS: Observed in 92% of patients, nodules were the most common CT pathology. Areas of ground-glass attenuation, consolidation, masses of linear attenuation, and tracheal/bronchial wall-thickening were detected in 24%, 26%, 32%, 39%, and 68% of patients. At follow-up, the clearance of lesions was most consistent for areas of ground-glass attenuation (89%), masses (87%), and cavitated nodules (85%). In the follow-up scan, 58% of all nodules, 47% of pulmonary consolidations, and 66% of bronchial wall-thickening were completely resolved. Areas of bronchiectasis and septal/non-septal lines remained stable in 70% and 71% of patients. CONCLUSION: The majority of the lesions decreased or resolved completely with or without areas of linear attenuation. Ground-glass attenuation, cavitated nodules and masses appear to represent active inflammatory lesions. In most probability, areas of bronchiectasis and septal/non-septal lines more often represent chronic fibrotic changes rather than active inflammatory changes. In combination with clinical evaluation and bronchoscopy, CT assists in the assessment of disease activity.

  14. Granulomatosis with Polyangiitis

    Science.gov (United States)

    Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a rare disease. It is a type of vasculitis, or inflammation of the blood vessels. The inflammation limits the flow of blood ...

  15. On the Wegener granulomatosis associated region on chromosome 6p21.3

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    Csernok Elena

    2006-03-01

    Full Text Available Abstract Background Wegener granulomatosis (WG belongs to the heterogeneous group of systemic vasculitides. The multifactorial pathophysiology of WG is supposedly caused by yet unknown environmental influence(s on the basis of genetic predisposition. The presence of anti-neutrophil cytoplasmic antibodies (ANCA in the plasma of patients and genetic involvement of the human leukocyte antigen system reflect an autoimmune background of the disease. Strong associations were revealed with WG by markers located in the major histocompatibility complex class II (MHC II region in the vicinity of human leukocyte antigen (HLA-DPB1 and the retinoid X receptor B (RXRB loci. In order to define the involvement of the 6p21.3 region in WG in more detail this previous population-based association study was expanded here to the respective 3.6 megabase encompassing this region on chromosome 6. The RXRB gene was analysed as well as a splice-site variation of the butyrophilin-like (BTNL2 gene which is also located within the respective region. The latter polymorphism has been evaluated here as it appears as a HLA independent susceptibility factor in another granulomatous disorder, sarcoidosis. Methods 150–180 German WG patients and a corresponding cohort of healthy controls (n = 100–261 were used in a two-step study. A panel of 94 microsatellites was designed for the initial step using a DNA pooling approach. Markers with significantly differing allele frequencies between patient and control pools were individually genotyped. The RXRB gene was analysed for single strand conformation polymorphisms (SSCP and restriction fragment length polymorphisms (RFLP. The splice-site polymorphism in the BTNL2 gene was also investigated by RFLP analysis. Results A previously investigated microsatellite (#1.0.3.7, Santa Cruz genome browser (UCSC May 2004 Freeze localisation: chr6:31257596-34999883, which was used as a positive control, remained associated throughout the whole two

  16. On the Wegener granulomatosis associated region on chromosome 6p21.3

    Science.gov (United States)

    Szyld, Paweł; Jagiello, Peter; Csernok, Elena; Gross, Wolfgang L; Epplen, Joerg T

    2006-01-01

    Background Wegener granulomatosis (WG) belongs to the heterogeneous group of systemic vasculitides. The multifactorial pathophysiology of WG is supposedly caused by yet unknown environmental influence(s) on the basis of genetic predisposition. The presence of anti-neutrophil cytoplasmic antibodies (ANCA) in the plasma of patients and genetic involvement of the human leukocyte antigen system reflect an autoimmune background of the disease. Strong associations were revealed with WG by markers located in the major histocompatibility complex class II (MHC II) region in the vicinity of human leukocyte antigen (HLA)-DPB1 and the retinoid X receptor B (RXRB) loci. In order to define the involvement of the 6p21.3 region in WG in more detail this previous population-based association study was expanded here to the respective 3.6 megabase encompassing this region on chromosome 6. The RXRB gene was analysed as well as a splice-site variation of the butyrophilin-like (BTNL2) gene which is also located within the respective region. The latter polymorphism has been evaluated here as it appears as a HLA independent susceptibility factor in another granulomatous disorder, sarcoidosis. Methods 150–180 German WG patients and a corresponding cohort of healthy controls (n = 100–261) were used in a two-step study. A panel of 94 microsatellites was designed for the initial step using a DNA pooling approach. Markers with significantly differing allele frequencies between patient and control pools were individually genotyped. The RXRB gene was analysed for single strand conformation polymorphisms (SSCP) and restriction fragment length polymorphisms (RFLP). The splice-site polymorphism in the BTNL2 gene was also investigated by RFLP analysis. Results A previously investigated microsatellite (#1.0.3.7, Santa Cruz genome browser (UCSC) May 2004 Freeze localisation: chr6:31257596-34999883), which was used as a positive control, remained associated throughout the whole two-step approach

  17. Magnetic resonance imaging and computed tomography of the orbit in Wegener's granulomatosis. Magnetresonanztomographie und Computertomographie der Wegenerschen Granulomatose der Orbita

    Energy Technology Data Exchange (ETDEWEB)

    Muhle, C. (Klinik fuer Radiologische Diagnostik, Kiel (Germany)); Noelle, B. (Klinik fuer Ophthalmologie, Univ. Kiel (Germany)); Brinkmann, G. (Klinik fuer Radiologische Diagnostik, Kiel (Germany)); Schubert, F. (Klinik fuer Radiologische Diagnostik, Kiel (Germany)); Reinhold-Keller, E. (Poliklinik fuer Klinische Rheumatologie, Univ. Luebeck (Germany) Medizinische Krankenhausabteilung, Rheumaklinik Bad Bramstedt (Germany)); Spielmann, R.P. (Klinik fuer Radiologische Diagnostik, Kiel (Germany)); Reuter, M. (Klinik fuer Radiologische Diagnostik, Kiel (Germany))

    1994-09-01

    Of a total of 121 patients with histological proven Wegener's Granulomatosis, orbital involvement occurred in 12 cases. Eight of them underwent magnetic resonance imaging (MRI) of the head and brain, in 2 cases computed tomography (CT) was additionally performed. With MRI orbital granulomas had a low signal intensity in T[sub 1]- and T[sub 2]-weighted spin-echo sequences. After i.v. contrast medium administration an inhomogeneous enhancement was found. Because of the multiple imaging planes and high soft tissue contrast, orbital granulomas were better delineate with regard to the intraorbital muscles and the optic nerve using MRI than with CT. Osseous destruction and sclerosis of the orbital walls were demonstrated more effectively using CT. MRI and CT are thus complementary for diagnosing and staging, of orbital granulomas and periorbital involvement in Wegener's Granulomatosis. (orig.)

  18. High-resolution CT (HRCT) of the lung in Wegener's granulomatosis. High-Resolution CT (HRCT) der Lunge bei Wegenerscher Granulomatose

    Energy Technology Data Exchange (ETDEWEB)

    Schubert, F. (Klinik fuer Radiologische Diagnostik, Kiel Univ. (Germany)); Muhle, C. (Klinik fuer Radiologische Diagnostik, Kiel Univ. (Germany)); Schnabel, A. (Medizinische Abt., Rheumaklinik Bad Bramstedt (Germany) Abt. fuer Klinische Rheumatologie, Luebeck Univ. (Germany)); Handrock, K. (Medizinische Abt., Rheumaklinik Bad Bramstedt (Germany) Abt. fuer Klinische Rheumatologie, Luebeck Univ. (Germany)); Reuter, M. (Klinik fuer Radiologische Diagnostik, Kiel Univ. (Germany)); Spielmann, R.P. (Klinik fuer Radiologische Diagnostik, Kiel Univ. (Germany)); Gross, W.L. (Medizinische Abt., Rheumaklinik Bad Bramstedt (Germany) Abt. fuer Klinische Rheumatologie, Luebeck Univ. (Germany)); Heller, M. (Klinik fuer Radiologische Diagnostik, Kiel Univ. (Germany))

    1994-07-01

    HRCT of the lung and plain chest X-ray were performed to reveal pulmonary manifestation in primary diagnostics or reevaluation of 35 patients with Wegener's granulomatosis. Pleural and parenchymal pathology was detected in chest X-ray of 20 (57%) and in HRCT of 30 (85,7%) patients. Granulomas with and without cavitations and with smooth or spiculated margins were deemed pathognomonic. Nonspecific findings were infiltrates, thickened interlobular septae and fibrotic changes of parenchyma and pleura. Ground glass opacities, traction bronchiectasis and small cysts were only visible on HRCT. As expected HRCT proved to be more sensitive in detecting subtle lung alterations than plain film chest X-ray. It helps to differentiate acute inflammatory and thus potentially curable processes from chronic fibrotic changes in Wegener's granulomatosis. (orig.)

  19. Lymphomatoid granulomatosis

    African Journals Online (AJOL)

    lymphomas. Angiocentric immunoproliferative lesions are thought to represent a variant ofT-cell lymphoma. Wegener's granulomatosis, which is essentially a granulomatous vasculitis, is distinguished from lymphomatoid granulomatosis because of its unique appearance on histological examination and because it does not ...

  20. Amphipathic variable region heavy chain peptides derived from monoclonal human Wegener's anti-PR3 antibodies stimulate lymphocytes from patients with Wegener's granulomatosis and microscopic polyangiitis

    Science.gov (United States)

    Peen, E; Malone, C; Myers, C; Williams, R C; Peck, A B; Csernok, E; Gross, W L; Staud, R

    2001-01-01

    Amphipathic variable-region heavy chain 11-mer peptides from monoclonal human IgM antiproteinase-3 antibodies were studied for peripheral blood lymphocyte stimulation in 21 patients with Wegener's granulomatosis (WG) or microscopic polyangiitis (MPA), connective tissue disease controls and normal control subjects. Positive T-cell activation was observed in most experiments with WG patients' lymphocytes using amphipathic VH-region peptides from four different human monoclonal anti-PR3 antibodies. Control peptides of the same length but without amphipathic characteristics along with other amphipathic peptides not derived from monoclonal anti-PR3 sequence were employed as controls. No significant lymphocyte stimulation was observed with normal controls, but positive stimulation with amphipathic VH peptides was also recorded in other connective tissue disease controls mainly patients with rheumatoid arthritis. Amphipathic peptides not derived from anti-PR3 sequence did not stimulate WG lymphocytes. Our findings indicate that lymphocyte reactivity as an element of cell-mediated immunity may be activated by amphipathic VH-region amino acid sequences of autoantibodies which are themselves associated with diseases such as WG. PMID:11529926

  1. Low dose radiotherapy as an effective treatment in a patient with solitary Wegener's granulomatosis resistant to systemic treatment – case report

    Science.gov (United States)

    Rutkowski, Tomasz; Składowski, Krzysztof; Hejduk, Beata

    2013-01-01

    Wegener's granulomatosis is a rare disease (10 per 1 000 000 new cases per year), etiologically connected with necrotizing vasculitis of small to medium-size vessels. The disease occurs predominantly in the upper respiratory tract, lungs and kidneys, but any organ may be affected during the course of the illness. It may be difficult to diagnose, especially when c-ACNA antibodies (serologic symptom of Wegener's granulomatosis) are undetectable and chest X-ray is normal. Early diagnosis is crucial for treatment results. Untreated disease may lead to death. Cyclophosphamide used simultaneously with prednisone is the treatment of choice as the first line procedure. Resistance to standard systemic treatment may be a significant problem. New drugs (rituximab, infliximab) are still under clinical investigation, with promising results. Very limited data concerning effectiveness of radiation therapy exist. We present a report of a female patient with solitary form of Wegener's granulomatosis located in the facial region, who underwent successful radiation therapy with a complete response. PMID:23788974

  2. Extracorporeal membrane oxygenation rescue in adolescent with bronchiolitis obliterans-organizing pneumonia like Wegener's granulomatosis.

    Science.gov (United States)

    Falk, Lars; Broman, Lars Mikael

    2017-01-01

    We report a 17-year-old woman with bronchiolitis obliterans-organizing pneumonia (BOOP)-like granulomatosis with polyangiitis developing severe airway obliterations. Pending age, phase and grade of autoimmune treatment, and offering ECMO treatment may be crucial for survival but occasionally preface futility. ECMO-treated patient with BOOP-like GPA has never been described before.

  3. Neutrophil proteinase 3 and dipeptidyl peptidase I (cathepsin C) as pharmacological targets in granulomatosis with polyangiitis (Wegener granulomatosis).

    Science.gov (United States)

    Korkmaz, Brice; Lesner, Adam; Letast, Stephanie; Mahdi, Yassir K; Jourdan, Marie-Lise; Dallet-Choisy, Sandrine; Marchand-Adam, Sylvain; Kellenberger, Christine; Viaud-Massuard, Marie-Claude; Jenne, Dieter E; Gauthier, Francis

    2013-07-01

    Neutrophils are among the first cells implicated in acute inflammation. Leaving the blood circulation, they quickly migrate through the interstitial space of tissues and liberate oxidants and other antimicrobial proteins together with serine proteinases. Neutrophil elastase, cathepsin G, proteinase 3 (PR3), and neutrophil serine protease 4 are four hematopoietic serine proteases activated by dipeptidyl peptidase I during neutrophil maturation and are mainly stored in cytoplasmic azurophilic granules. They regulate inflammatory and immune responses after their release from activated neutrophils at inflammatory sites. Membrane-bound PR3 (mbPR3) at the neutrophil surface is the prime antigenic target of antineutrophil cytoplasmic autoantibodies (ANCA) in granulomatosis with polyangiitis (GPA), a vasculitis of small blood vessels and granulomatous inflammation of the upper and/or lower respiratory tracts. The interaction of ANCA with mbPR3 results in excessive activation of neutrophils to produce reactive oxygen species and liberation of granular proteinases to the pericellular environment. In this review, we focus on PR3 and dipeptidyl peptidase I as attractive pharmacological targets whose inhibition is expected to attenuate autoimmune activation of neutrophils in GPA.

  4. A case of squamous cell carcinoma of the nasal cavity in a patient with granulomatosis with polyangiitis (Wegener granulomatosis).

    Science.gov (United States)

    Kuan, Edward C; Peng, Kevin A; Gonzalez, Lyndon O; Sercarz, Joel A

    2018-01-01

    We report a rare case of squamous cell carcinoma (SCC) of the nasal cavity arising in a patient with granulomatosis with polyangiitis (GPA). The patient was a 35-year-old man who had been diagnosed 15 years earlier with GPA and treated medically for sinonasal, pulmonary, and renal involvement. He presented to us with left-sided orbital and cheek pain and nasal obstruction. Endoscopy detected a friable, exophytic mass that involved the left lateral nasal wall and septum. Biopsy analysis identified the mass as an SCC. A definitive endoscopic resection was performed, followed by chemoradiation, but the patient exhibited progression of disease 2 months after the cessation of therapy. He then underwent an open craniofacial resection and a second round of chemoradiation. At 7 months of follow-up, he remained disease-free. Sinonasal symptoms in GPA are consistent with those in chronic rhinosinusitis, but the presence of unilateral symptoms may suggest a neoplastic process. Immunosuppressants are implicated in the pathophysiology of this malignancy, but equally plausible is the oncogenic role of chronic inflammation.

  5. [A rare mode of revelation of Wegener's granulomatosis: myocarditis associated with a fibroblastic endocarditis].

    Science.gov (United States)

    Arous, Salim; Bensahi, Ilham; Noureddine, Malika; Habbal, Rachida

    2016-01-01

    We report, through this observation, a rare case of Wegener's disease revealed by myocarditis associated with fibroblastic endocarditis. The patient was initially admitted to hospital with global heart failure. The ECG showed atrial flutter rhythm disorder. Echocardiographic examination revealed non-dilated left ventricle with severe dysfunction and positive troponin dosage. Severe renal failure was discovered fortuitously and required hemodialysis. It was associated with an inflammatory anaemia confirmed by serum ferritin and myelogram. c-ANCA dosage was strongly positive, confirming the diagnosis. Chest CT scanning objectified right basal pneumonia. After starting appropriate treatment, including corticotherapy and immunosuppressive treatment, evolution was favorable with the normalization of left ventricular systolic function. Although obvious clinical cardiac events are rare, cardiac involvement in Wegener's disease is described, requiring rapid diagnostic orientation and rigorous knowledge of this serious disease.

  6. Cutaneous manifestations of Wegener's granulomatosis: a clinicopathologic study of 17 patients and correlation to antineutrophil cytoplasmic antibody status.

    Science.gov (United States)

    Comfere, Nneka I; Macaron, Nada C; Gibson, Lawrence E

    2007-10-01

    Wegener's granulomatosis (WG), a systemic vasculitis, can be associated with cutaneous signs and symptoms before, during or after the diagnosis of systemic disease. We reviewed clinical and histologic features of cutaneous lesions from 17 patients with WG. The temporal relationship between development of cutaneous symptoms and onset of systemic disease was determined, and antineutrophil cytoplasmic antibody (ANCA) status of the patients was also established. In six patients, systemic and cutaneous disease developed concurrently. In eight patients, cutaneous disease developed after patients received the diagnosis of systemic disease. In three patients, cutaneous disease preceded systemic disease. Cytoplasmic ANCA or proteinase-3-ANCA [c-ANCA/proteinase 3 (PR3)-ANCA] serologic test results were negative for one patient when cutaneous disease developed, and one patient had c-ANCA/PR3-ANCA seroconversion a year before systemic disease developed. Histopathologic features of cutaneous WG were not limited to leukocytoclastic vasculitis; they also included acneiform perifollicular and dermal granulomatous inflammation and palisaded neutrophilic and granulomatous inflammation. Patients with WG can present initially with cutaneous symptoms. Histopathologic patterns vary, but leukocytoclastic vasculitis is most commonly noted. Patients with WG and skin lesions are likely to have positive c-ANCA/PR3-ANCA serologic test results.

  7. Distinct proteinase 3-induced cytokine patterns in Wegener´s granulomatosis, Churg-Strauss syndrome, and healthy controls.

    Science.gov (United States)

    Fagin, Ursula; Csernok, Elena; Müller, Antje; Pitann, Silke; Fazio, Juliane; Krause, Kristina; Bremer, Philip; Wipfler-Freissmuth, Edith; Moosig, Frank; Gross, Wolfgang L; Lamprecht, Peter

    2011-01-01

    To analyse whether a specific cytokine pattern is elicited in response to the autoantigen proteinase 3 (PR3) in active Wegener's granulomatosis (WG). Six-colour flow cytometry was used to analyse cytokine production and surface markers of the total CD4+ T-cell population ex vivo and in PR3-stimulated T-cell lines of patients with active PR3-ANCA-positive WG, PR3-ANCA-negative Churg-Strauss syndrome (CSS), and healthy controls (HC). The cytokine response of the total PB CD4+ T cell population was skewed towards distinct pro-inflammatory cytokine patterns in WG (Th1-type) and CSS (Th17, Th1-/Th2-type). Th2-type as well as Th17 cell populations including Th17/Th1, Th17/Th2 and Th22 cells were elicited in response to PR3 stimulation in WG. In contrast, CSS patients displayed a Th2-type dominated response following PR3 stimulation. These data suggest that the cytokine response of the total CD4+ T-cell population and PR3-specific cells is influenced by the underlying disorder.

  8. Epidemiology and etiology of wegener granulomatosis, microscopic polyangiitis, churg-strauss syndrome and goodpasture syndrome: vasculitides with frequent lung involvement.

    Science.gov (United States)

    Gibelin, Aude; Maldini, Carla; Mahr, Alfred

    2011-06-01

    This review focuses on the epidemiological characteristics and etiologies of four primary systemic vasculitides with frequent lung involvement, namely Wegener granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS), and Goodpasture syndrome (GPS). Elucidation of the mechanisms underlying these vasculitides with frequent lung involvement is complicated by their rarity, which hampers the undertaking of large-scale studies; difficulties in classification; and their multifaceted clinical presentations, which infer the existence of several etiologic pathways. Notwithstanding, epidemiological research showed some evidence for international, interethnic, and temporal variations of the frequencies of these four vasculitides; led to the identification of several genetic and environmental risk factors; and provided insight on the extent to which genes and environment might contribute to their development. Available data support the concept that WG, MPA, CSS, and GPS have unique and shared risk determinants. Although the precise causes of these vasculitides are not yet fully understood and the development of prevention strategies is out of our reach at present, current knowledge enables the formulation of etiologic hypotheses to provide caregivers and their patients with valuable information on the nature of these rare entities. © Thieme Medical Publishers.

  9. Desmopressin-related myocardial infarction in a patient with Wegener's granulomatosis: a case report and review of the literature.

    Science.gov (United States)

    Pape, E; Béné, J; Buchdahl, A-L; Gautier, S; Hatron, P-Y; Lambert, M

    2013-02-01

    Desmopressin is a synthetic vasopressin analog that increases the plasma levels of coagulation factor VIII, von Willebrand factor, and tissue plasminogen activator. This hemostatic agent, which can be administered either parenterally or intranasally, has been approved for use in the prevention and treatment of hemorrhagic events during surgery in patients with hemophilia A, in cases of prolonged idiopathic bleeding, and for complications associated with platelet antiaggregant therapy. This case report describes cardiac toxicity associated with desmopressin administered according to the recommended indications: a 55-year-old woman diagnosed with Wegener's granulomatosis (WG) was treated with desmopressin to improve hemostasis and shorten bleeding time before a planned renal biopsy. She developed cardiac arrest within 60 minutes of the desmopressin injection. Cardiopulmonary resuscitation began immediately and was successful, although the patient subsequently died of WG-associated complications. Desmopressin administration thus appears, in some cases, to be associated with a high risk of thrombotic events, possibly by stimulating the rapid release of endothelial factors such as an abnormal multimeric form of von Willebrand factor, which might cause platelet aggregation. Clinicians should be aware of the possible occurrence of this little-known but potentially serious cardiac event associated with desmopressin administration and be prepared to initiate cardiopulmonary resuscitation immediately if needed. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  10. Mastoidite e paralisia facial como manifestações iniciais de Granulomatose de Wegener Mastoiditis and facial paralysis as initial manifestations of Wegener's Granulomatosis

    Directory of Open Access Journals (Sweden)

    André Souza de Albuquerque Maranhão

    2012-04-01

    Full Text Available A Granulomatose de Wegener (GW é caracterizada por granulomas necrotizantes e vasculite. Sem tratamento a doença tem prognóstico pobre com índice de mortalidade de 90% em 2 anos. Diversos autores citam as manifestações otológicas no curso da GW, entretanto não é especificado em que momento da doença elas apareceram, isto é, se como manifestação inicial ou subsequente a outros achados. Objetivo: Descrever três casos confirmados de GW que apresentaram inicialmente mastoidite e evoluíram com paralisia facial periférica (PFP. Material e Método: Estudo de série de casos. Pacientes diagnosticados com GW que apresentaram inicialmente manifestações otológicas são descritos. Resultados: Os três casos descritos abriram o quadro com otalgia, otorreia e hipoacusia unilateral, associada a paralisia facial periférica ipsilateral. Tiveram resposta inadequada aos tratamentos instituídos o que motivou uma investigação de outras doenças associadas. Nessas circunstâncias, detectaram-se títulos positivos de ANCA-C em todos pacientes, confirmando-se o diagnóstico de GW, após período variável de investigação. Institui-se o tratamento para GW observando-se melhora do quadro clínico, regressão da PFP e melhora parcial dos limiares auditivos. Conclusão: Complicações de otites médias agudas (mastoidite e PFP refratárias as terapêuticas habituais impõem a investigação de doenças associadas e a GW deverá ser pesquisada para que se possa fazer o diagnóstico o mais precocemente possível, alterando desta forma o prognóstico destes pacientes.Wegener's Granulomatosis (WG is characterized by necrotizing granulomas and vasculitis. If left untreated, the prognosis is poor - a 90% mortality rate within 2 years. Several authors have described the otologic manifestations of WG; these authors, however, have not mentioned the stage of the disease in which these findings present - whether as initial manifestations or subsequent to

  11. Antimicrobial peptides in nasal secretion and mucosa with respect to S. aureus colonisation in Wegener´s granulomatosis.

    Science.gov (United States)

    Hui, Yuan; Wohlers, Janet; Podschun, Rainer; Hedderich, Jürgen; Lamprecht, Peter; Ambrosch, Petra; Laudien, Martin

    2011-01-01

    Nasal S. aureus carrier rates are significantly higher in patients with Wegener's granulomatosis (WG) compared to healthy controls (HC), and nasal colonisation is a risk-factor for relapse. Antimicrobial peptides (AMP) are important defence molecules maintaining an intact barrier function. It is the aim of this study to see if there is a possible link between the nasal AMP pattern and S. aureus colonisation, a link which has not been investigated so far. ELISA was applied to quantify LL-37 and hBD-3 concentrations in nasal secretions (14 WG patients, 13 HC) with and without nasal S. aureus colonisation. Immunohistochemistry was used to detect the cellular sources of AMP in the nasal mucosa. Functional analyses of primary nasal epithelial cell cultures (NEC) of these groups stimulated with S. aureus were performed. LL-37 was found in significantly higher concentrations in colonised individuals (WG: p=0.001; HC: p=0.014).Using immunohistochemistry, local cellular sources for AMP could be demonstrated. After stimulation with S. aureus, significantly higher concentrations of LL-37 and hBD-3 could be detected in the supernatant of NEC of WG patients (LL-37: p=0.001; hBD-3: p=0.001) and HC (LL-37: p=0.019; hBD-3: p=0.001). HBD-3 concentrations were significantly lower in the supernatant of stimulated NEC of WG patients compared to the NEC of HC (p=0.032), and the dynamic range of the hBD-3 answer was significantly smaller in WG compared to HC (p=0.016). The dynamic response towards challenges with microbes is dysregulated in WG, and this might be one reason for higher S. aureus colonisation rates in WG.

  12. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation

    DEFF Research Database (Denmark)

    Ruperto, Nicolino; Ozen, Seza; Pistorio, Angela

    2010-01-01

    To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria....

  13. Granulomatosis with polyangiitis (Wegener's disease): An updated review of ocular disease manifestations

    Science.gov (United States)

    Kubaisi, Buraa; Abu Samra, Khawla; Foster, C. Stephen

    2016-01-01

    Summary Granulomatosis with polyangiitis (GPA) is a potentially lethal systemic disorder that is characterized by necrotizing vasculitis of small arteries and veins. The respiratory system is most commonly affected in limited forms of the disease, however upper and lower respiratory system, systemic vasculitis, and necrotizing glomerulonephritis are the characteristic components of the disease triad. The peak incidence is observed at 64–75 years of age, with a prevalence of 8–10 per million depending on geographic location. In this review we focus on the ocular manifestations of the disease which occur in nearly in one third of the patients. In addition we describe the neuro-ophthalmic complications which occur in up to half of cases. We also discuss the current systemic treatment options including corticosteroids, cyclophosphamide, azathioprine, and the available biologic response modifiers including rituximab. The disease remains difficult to diagnose due to the generalized symptomatic presentation of patients with GPA. As a result, several sets of diagnostic criteria have been developed which include clinical, serological, and histopathological findings to varying extents. Early diagnosis and multi-specialty collaboration among physicians is necessary to adequately manage the disease and the potential complications that may result from drugs used in the treatment of the disease. Despite recent advances, more research is necessary to prevent the high rates of mortality from the disease itself and from therapeutic side effects. PMID:27195187

  14. Granulomatosis with polyangiitis (Wegener's disease): An updated review of ocular disease manifestations.

    Science.gov (United States)

    Kubaisi, Buraa; Abu Samra, Khawla; Foster, C Stephen

    2016-05-01

    Granulomatosis with polyangiitis (GPA) is a potentially lethal systemic disorder that is characterized by necrotizing vasculitis of small arteries and veins. The respiratory system is most commonly affected in limited forms of the disease, however upper and lower respiratory system, systemic vasculitis, and necrotizing glomerulonephritis are the characteristic components of the disease triad. The peak incidence is observed at 64-75 years of age, with a prevalence of 8-10 per million depending on geographic location. In this review we focus on the ocular manifestations of the disease which occur in nearly in one third of the patients. In addition we describe the neuro-ophthalmic complications which occur in up to half of cases. We also discuss the current systemic treatment options including corticosteroids, cyclophosphamide, azathioprine, and the available biologic response modifiers including rituximab. The disease remains difficult to diagnose due to the generalized symptomatic presentation of patients with GPA. As a result, several sets of diagnostic criteria have been developed which include clinical, serological, and histopathological findings to varying extents. Early diagnosis and multi-specialty collaboration among physicians is necessary to adequately manage the disease and the potential complications that may result from drugs used in the treatment of the disease. Despite recent advances, more research is necessary to prevent the high rates of mortality from the disease itself and from therapeutic side effects.

  15. Estenose subglótica como manifestação clínica da granulomatose de Wegener em adolescentes: relato de caso e revisão de literatura Subglottic stenosis as a clinical manifestation of Wegener's granulomatosis in adolescents: report of a case and review of literature

    Directory of Open Access Journals (Sweden)

    Márcia C. Machado

    2003-10-01

    Full Text Available A Granulomatose de Wegener é uma patologia caracterizada por vasculite sistêmica e inflamação granulomatosa necrotizante que compromete o trato respiratório alto, pulmões e rins, cujas manifestações iniciais, na maioria das vezes, ocorre em maiores de 20 anos de idade. As queixas clínicas otorrinolaringológicas estão, com freqüência, presentes nas fases iniciais da doença, sendo rinite, sinusopatia de repetição e epistaxe as mais comuns. A estenose subglógica parece estar correlacionada com a Granulomatose de Wegener em adolescentes. Relatamos o caso de uma paciente portadora de Granulomatose de Wegener cujo início dos sintomas foi na infância, tendo evoluído com estenose laríngea durante o curso da doença.Wegener's Granulomatosis is a well-characterized systemic vasculitis and necrotising granulomatous inflammation of the upper, lower respiratory tracts and the kidneys. The initial manifestations of the disease usually occur in patients over than twenty years old. The otolaryngologic symptoms like rhinorrhea, recurrent sinusitis and epistaxis are commonly present in early course of the disease. It seems that subglottic stenosis is correlated to Wegener's granumatosis in adolescents. We describe a case of a patient that developed subglottic stenosis associated with Wegener's granulomatosis in childhood during the course of the disease.

  16. Rheumatoid factor could be used as a marker of response to therapy in a patient with Wegener's granulomatosis: A case report

    Directory of Open Access Journals (Sweden)

    Vekić Davor

    2011-01-01

    Full Text Available Wegener's granulomatosis is rare disease of unknown etiology. It could be classified as vasculitis, which involves lungs, kidneys, skin and other organs with characteristic necrotizing granulomas. Ninety percent of patients with this disease have respiratory or renal symptomatology. We present a case of a male patient, 68 years old, with symptoms of pneumonia, bilateral conjunctivitis, hemoptysis, 10 kg weight loss and gradual development of chronic renal failure. The symptomatology was caused by granulomatous vasculitis accompanied with high serum levels of rheumatoid factor, which was recorded at the beginning of the disease, before introduction of therapy. This case report underlines possible role of rheumatoid factor in estimation of the patient's response to administered therapy. .

  17. Vasculitis de Wegener: diferentes presentaciones pulmonares en el diagnóstico inicial y durante la evolución de la enfermedad Wegener granulomatosis: different pulmonary abnormalities at initial diagnostic and during the course of disease

    Directory of Open Access Journals (Sweden)

    Marta Hernandorena González

    2009-09-01

    Full Text Available La enfermedad de Wegener es una vasculitis granulomatosa necrotizante, con afectación característica del tracto respiratorio superior, pulmones y riñones. El 90% de los pacientes tienen afectación pulmonar y los hallazgos radiográficos más frecuentes son nódulos o masas bilaterales múltiples que pueden mostrar cavitación. También se puede presentar como consolidación lobar o segmentaria o como hemorragia pulmonar difusa. El reconocimiento precoz de las alteraciones específicas es crítico para realizar un diagnóstico apropiado y para reducir la morbi-mortalidad. Con el objetivo de lograr una mejor caracterización de las distintas manifestaciones torácicas de esta entidad presentamos cuatro casos que muestran sus distintas manifestaciones radiológicas.Wegener granulomatosis is a necrotizing granulomatous vasculitis that affects the upper respiratory tract, lungs, and kidneys. At least 90% of patients have pulmonary involvement, and the typical radiographic findings are bilateral multiple nodules or masslike lesions, some of which show cavitation. Other frequent findings are consolidations or diffuse pulmonary hemorrhage. Early recognition of specific abnormalities is critical for appropriate intervention and reduced morbidity. To better characterize the diversity of thoracic manifestations in this disease we present four cases to show different radiologic findings in this population.

  18. Characteristics and Outcomes of Granulomatosis With Polyangiitis (Wegener) and Microscopic Polyangiitis Requiring Renal Replacement Therapy: Results From the European Renal Association-European Dialysis and Transplant Association Registry.

    Science.gov (United States)

    Hruskova, Zdenka; Stel, Vianda S; Jayne, David; Aasarød, Knut; De Meester, Johan; Ekstrand, Agneta; Eller, Kathrin; Heaf, James G; Hoitsma, Andries; Martos Jimenéz, Carmen; Ravani, Pietro; Wanner, Christoph; Tesar, Vladimir; Jager, Kitty J

    2015-10-01

    This study describes the incidence and outcomes of European patients requiring renal replacement therapy (RRT) for kidney failure due to antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Cohort study. 12 renal registries providing individual RRT patient data to the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) Registry in 1993-2012 participated. Cause of primary kidney disease: AAV (ie, granulomatosis with polyangiitis [Wegener] and microscopic polyangiitis) versus 3 separate matched control groups without AAV: (1) primary glomerulonephritis, (2) diabetes mellitus, and (3) disease other than diabetes mellitus as the cause of primary kidney disease, including glomerulonephritis (termed "nondiabetes"). Incidence, causes of death, and survival. ERA-EDTA primary renal disease codes. 2,511 patients with AAV (1,755, granulomatosis with polyangiitis; 756, microscopic polyangiitis) were identified, representing an incidence of 1.05 per million population (pmp) for granulomatosis with polyangiitis (predominating in Northern Europe) and 0.45 pmp for microscopic polyangiitis (prevailing in Southern Europe). Kidney transplantation was performed in 558 (22.2%) patients with vasculitis. The 10-year probability for survival on RRT after day 91 was 32.5% (95% CI, 29.9%-35.1%) in patients with vasculitis. Survival on RRT after day 91 did not differ between AAV and matched nondiabetes patients. Patient and transplant survival after kidney transplantation, adjusted for time period and country, was better in AAV than in matched nondiabetes patients (HRs of 0.81 [95% CI, 0.67-0.99] and 0.82 [95% CI, 0.69-0.96], respectively). No data for extrarenal manifestations, treatment, and relapses. Geographical differences in the incidence of RRT for kidney failure due to granulomatosis with polyangiitis and microscopic polyangiitis copied their distribution in the general population. Overall survival on RRT after day 91 for patients

  19. Active disease and residual damage in treated Wegener's granulomatosis: an observational study using pulmonary high-resolution computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Komocsi, Andras [Poliklinik fuer Rheumatologie, Universitaet Luebeck, Ratzeburger Allee 160, 23538 Luebeck (Germany); Reuter, Michael; Heller, Martin [Klinik fuer Diagnostische Radiologische, Universitaet Kiel, Arnold-Heller-Strasse 7, 24105 Kiel (Germany); Murakoezi, Henriette; Gross, Wolfgang L.; Schnabel, Armin [Poliklinik fuer Rheumatologie, Universitaet Luebeck, Ratzeburger Allee 160, 23538 Luebeck (Germany); Rheumaklinik Bad Bramstedt, Ratzeburger Allee 160, 23538 Luebeck (Germany)

    2003-01-01

    The purpose of this study was to determine to what extent high-resolution computed tomography (HRCT) of the lungs can distinguish active inflammatory disease from inactive cicatricial disease in patients treated for Wegener's granulomatosis (WG). Twenty-eight WG patients with active pulmonary disease underwent a first HRCT examination immediately before standard immunosuppressive treatment and a second examination after clinical remission had been achieved. Lesions remaining after treatment were categorized as residual damage and were compared with findings during active disease to see by what features active and cicatricial disease can be distinguished. During active disease 17 patients had nodules/masses, 12 had ground-glass opacities, 6 had septal lines and 6 had non-septal lines. After treatment, ground-glass opacities had resolved completely. Nodules/masses had resolved in 8 patients and had diminished in 7 patients. Residual nodules were distinguished from nodules/masses in active disease by lack of cavitation and a diameter of mostly <15 mm. In one-third of patients lines resolved, but in 8 instances new lines evolved during immunosuppression. During a follow-up period of a median 26.5 months (range 20.0-33.8), patients with residual nodules or lines had no more relapses than patients with completely cleared lungs. Treated pulmonary WG leaves substantial residual damage. High-resolution CT does assist in the distinction between active and inactive lesions. Ground-glass opacities, cavitating nodules/masses and masses measuring more than 3 cm represent active disease ordinarily. Non-cavitary small nodules and septal or non-septal lines can be either active or cicatricial lesions. The nature of these lesions needs to be clarified by longitudinal observation. (orig.)

  20. la granulomatose de wegener a propos de trois localisations orl ...

    African Journals Online (AJOL)

    16 juin 2006 ... SUMMARY. Wegener's Granulomatosis (WG) is a necrotizing granulomatous vasculitis that has a strong affinity for the upper respi- ratory tract. We report 3cases of rare ORL locations of Wegener's Granulomatosis (orbit, larynx and tongue). The aim of our study is to analyse the characteristic features of WG.

  1. Lesões destrutivas da linha média induzidas por cocaína com ANCA positivo mimetizando a granulomatose de Wegener Cocaine-induced midline destruction lesions with positive ANCA test mimicking Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    Letícia Stahelin

    2012-06-01

    Full Text Available O uso crônico de cocaína por inalação pode causar lesões destrutivas de linha média (LDLMIC, que podem ser difíceis de distinguir das lesões da granulomatose de Wegener (GW nos ouvidos, nariz e garganta. Descrevemos o caso de uma paciente de 43 anos admitida com história de dois anos de obstrução nasal e rinorreia. Ela havia recebido o diagnóstico de GW há cinco meses e estava em tratamento com prednisona e ciclofosfamida. Ao exame físico apresentava perfuração de septo nasal e palato. Exames de laboratório mostraram elevação das proteínas de fase aguda e teste p-ANCA positivo. Ensaios ELISA antiproteinase 3 e mieloperoxidase foram negativos. Tomografia computadorizada (TC dos seios paranasais mostrou destruição de septo nasal e palato, bem como sinusite maxilar bilateral. TC de tórax resultou normal. Biópsia da mucosa nasal revelou infiltrado inflamatório sem granuloma ou vasculite. Quando questionada, admitiu ser usuária de cocaína há cinco anos. Os imunossupressores foram suspensos e a paciente não mais fez uso da droga. Ela está sendo monitorada há seis meses e não desenvolveu novas lesões ou sintomas de outros órgãos. O diagnóstico diferencial em pacientes com LDLMIC pode ser desafiador. A avaliação deve incluir pesquisa de uso intranasal de cocaína. Embora o teste de ANCA não diferencie claramente o ANCA encontrado em alguns pacientes com LDLMIC daqueles em pacientes com GW, o envolvimento localizado e os achados de biópsia não típicos de vasculite granulomatosa de pequenos vasos devem ser reconhecidos como características das lesões induzidas por cocaína.Chronic use of cocaine by inhalation may induce midline destructive lesions (CIMDL, which can sometimes be difficult to distinguish from the ear, nose and throat lesions of Wegener's Granulomatosis (WG. We describe the case of a 43-year-old female patient admitted with a two-year history of nasal obstruction and rhinorrhea. She had been

  2. Intra- and inter-rater reliability of endonasal activity estimation in granulomatosis with polyangiitis (Wegener´s).

    Science.gov (United States)

    Garske, Ulrike; Haack, Andrea; Beltrán, Olga; Flores-Suárez, Louis F; Bremer, Jan P; Lamprecht, Peter; Hedderich, Jürgen; Quetz, Joachim; Gross, Wolfgang L; Ambrosch, Petra; Laudien, Martin

    2012-01-01

    Granulomatosis with polyangiitis (GPA) frequently starts with an affection of the nasal and paranasal mucosa. Localised GPA of the nasal mucosa or persistent disease activity ('grumbling disease') is often encountered even under immunosuppressive therapy. Necessity for reconstructive surgery is common and careful scheduling to prevent failure and minimise revision rates is crucial. Therefore, reliable estimation of GPA activity in the upper airways using a score is mandatory for diagnosis, follow-up and scheduling reconstructive surgery. Fifty endoscopic, endonasal images of 45 patients with GPA were used. Twelve (4 German, 8 Mexican) experienced (n=7) and inexperienced (n=5) physicians assessed GPA-activity at two times (T1/T2) in dichotomy and in a grading approach (none, mild, moderate and high activity) using the novel ENT Activity Score (ENTAS). All documents were written in English. Estimation of activity in dichotomy (none vs. mild/moderate/high): Cohen's Kappa (κ) for intra-rater reliability T1/T2 in inexperienced and experienced physicians was κ=0.58 (agreement 85%) and κ=0.72 (agreement 91%). The inter-rater reliability (Fleiss's κ) T1/T2 for inexperienced and experienced physicians was κ=0.62/κ=0.59 and κ=0.50/κ=0.58 respectively. Estimation of activity in grading approach (none, mild, moderate, high): for inexperienced physicians the intra-rater reliability T1/T2 was κ=0.67 (agreement 56%) and the inter-rater reliability at T1/T2 was κ=0.29 (intraclass correlation coefficient, ICC=0.69) and κ=0.27 (ICC=0.59). For experienced physicians the intra-rater reliability T1/T2 was κ=0.80 (agreement 67%) and the inter-rater reliability at T1 and T2 was κ=0.41 (ICC=0.77) and κ=0.39 (ICC=0.75) respectively. Intra-rater reliability is high in decision in dichotomy and even in grading activity. There is no difference for experienced or inexperienced physicians. Inter-rater reliability is high in dichotomy, but low for activity grading. Thus, the ENTAS

  3. Kinetic analysis of the blood clearance and organ uptake curves of IgG-coated red cells in HLA-typed controls and patients with Wegener's granulomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Woude, F.J. van der; Hauw The, T.; Beekhuis, H.; Beelen, J.M.

    1986-07-01

    Reticuloendothelial function and HLA type were studied in 22 controls and 15 patients with Wegener's granulomatosis (WG). IgG-coated red cells were injected intravenously and half-life of blood disappearance and liver spleen uptake curves were related to the degree of antibody coating. Erythrocytes with 13,990 molecules of IgG per cell gave biexponential blood disappearance curves and were suitable for measuring splenic reticuloendothelial function. Half-life times thus obtained were not significantly different for individuals with the HLA-DR2 or DR3 phenotype. In WG patients with major disease activity, blood clearance of the injected cells was considerably decreased. Kinetic analysis of blood disappearance and spleen uptake curves revealed that this was partly due to a decrease in reversible trapping of the cells in the spleen. This suggests that a blocking effect of circulating immune complexes on splenic Fc receptors is not likely to be the sole cause of the observed hyposplenism.

  4. Early extracorporeal life support as rescue for Wegener granulomatosis with diffuse alveolar hemorrhage and acute respiratory distress syndrome: a case report and literature review.

    Science.gov (United States)

    Joseph, Mark; Charles, Anthony G

    2011-12-01

    The study's objective was to report a case and review the literature on the use of extracorporeal life support in the face of severe pulmonary hemorrhage for acute respiratory distress syndrome. This study is a single case report of a pediatric patient who was successfully managed on venovenous extracorporeal life support for severe acute respiratory distress syndrome with acute pulmonary hemorrhage secondary to Wegener disease. Extracorporeal life support can be used successfully in selected patients with respiratory failure with pulmonary hemorrhage. The cautious use of anticoagulation should be balanced with the risk of bleeding, mindful of the need for other measures to mitigate severe bleeding if this should occur.

  5. Granulomatosis with polyangiitis and constrictive pericarditis--a case report.

    Science.gov (United States)

    Horne, A E; Henriksen, P A; Amft, E N

    2014-01-01

    Polyangiitis with granulomatosis, previously known as Wegener's granulomatosis, is a systemic necrotising granulomatous vasculitis. It predominantly affects the upper and lower respiratory tracts and the kidneys, but can potentially affect any organ system. It is diagnosed by clinical features, immunology (anti-neutrophil cytoplasmic antibodies) and histology. Cardiac involvement occurs in 6 to 44% of cases. We present a case of polyangiitis with granulomatosis and constrictive pericarditis, which occurred despite vigorous immunosuppression and which required surgical pericardectomy.

  6. Wegener′s Granulomatosis An unusual Presentation

    Directory of Open Access Journals (Sweden)

    Criton S

    2000-01-01

    Full Text Available Wegen’s granulomatosis is characterized by necortising granulomatous inflammation and vasculitis affecting predominantly small arteries and veins. In its generalized from there is involvement of upper and lower respiratory tract, kidney and often other organs including skin. It may also present as limited organ involvement. A case of Wegener’s granuolmatosis limited to skin is reported

  7. Oesophageal lesions as first manifestation of necrotising sarcoid granulomatosis.

    Science.gov (United States)

    Attou, Rachid; Duttmann, Ruth; Devriendt, Arnaud; De Bels, David

    2015-07-06

    Necrotising sarcoid granulomatosis is a rare disease associating sarcoid-like granulomas, vasculitis and necrosis in the lungs and seldom in extrapulmonary locations. We report the case of a 23-year-old woman with an initial symptom consisting of epigastric pain. Oesophagoscopy demonstrated oesophagitis. Evolution was marked by fever, dyspnoea and pulmonary lesions. The first presumptive diagnosis was Wegener disease but biopsies (of the lungs and the oesophagus) demonstrated the diagnosis of necrotising sarcoid granulomatosis. Evolution was favourable under corticoid therapy. Necrotising sarcoid granulomatosis may present as an extrapulmonary disease and is an important differential diagnosis of other disease such as Wegener disease. 2015 BMJ Publishing Group Ltd.

  8. MACULA RETINAL VASCULITIS AND CHOROIDITIS ASSOCIATED WITH GRANULOMATOSIS WITH POLYANGIITIS.

    Science.gov (United States)

    Lim, Lik Thai; Vasudevan, Vinaya; Shelton, Julie; Vitale, Albert T; Moorthy, Ramana

    2017-01-01

    To report a case series of two cases of granulomatosis with polyangiitis, previously known as Wegener granulomatosis, which developed macular necrosis, not previously associated with granulomatosis with polyangiitis, healed with fibrosis, despite aggressive immune-modulating therapy and good control of systemic disease. Case series of two cases with observation of treatment progress. The results reported the progress of response to treatment in the two cases, which resulted in the final outcome of fibrosis in the macula region, despite being on aggressive immune-modulating therapy and good systemic control. Granulomatosis with polyangiitis can be associated with macular necrosis leading to fibrosis, unresponsive to immune-modulating therapy.

  9. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria.

    Science.gov (United States)

    Ozen, Seza; Pistorio, Angela; Iusan, Silvia M; Bakkaloglu, Aysin; Herlin, Troels; Brik, Riva; Buoncompagni, Antonella; Lazar, Calin; Bilge, Ilmay; Uziel, Yosef; Rigante, Donato; Cantarini, Luca; Hilario, Maria Odete; Silva, Clovis A; Alegria, Mauricio; Norambuena, Ximena; Belot, Alexandre; Berkun, Yackov; Estrella, Amparo Ibanez; Olivieri, Alma Nunzia; Alpigiani, Maria Giannina; Rumba, Ingrida; Sztajnbok, Flavio; Tambic-Bukovac, Lana; Breda, Luciana; Al-Mayouf, Sulaiman; Mihaylova, Dimitrina; Chasnyk, Vyacheslav; Sengler, Claudia; Klein-Gitelman, Maria; Djeddi, Djamal; Nuno, Laura; Pruunsild, Chris; Brunner, Jurgen; Kondi, Anuela; Pagava, Karaman; Pederzoli, Silvia; Martini, Alberto; Ruperto, Nicolino

    2010-05-01

    To validate the previously proposed classification criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA). Step 1: retrospective/prospective web-data collection for children with HSP, c-PAN, c-WG and c-TA with age at diagnosis

  10. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation.

    Science.gov (United States)

    Ruperto, Nicolino; Ozen, Seza; Pistorio, Angela; Dolezalova, Pavla; Brogan, Paul; Cabral, David A; Cuttica, Ruben; Khubchandani, Raju; Lovell, Daniel J; O'Neil, Kathleen M; Quartier, Pierre; Ravelli, Angelo; Iusan, Silvia M; Filocamo, Giovanni; Magalhães, Claudia Saad; Unsal, Erbil; Oliveira, Sheila; Bracaglia, Claudia; Bagga, Arvind; Stanevicha, Valda; Manzoni, Silvia Magni; Pratsidou, Polyxeni; Lepore, Loredana; Espada, Graciela; Kone-Paut, Isabella; Paut, Isabelle Kone; Zulian, Francesco; Barone, Patrizia; Bircan, Zelal; Maldonado, Maria del Rocio; Russo, Ricardo; Vilca, Iris; Tullus, Kjell; Cimaz, Rolando; Horneff, Gerd; Anton, Jordi; Garay, Stella; Nielsen, Susan; Barbano, Giancarlo; Martini, Alberto

    2010-05-01

    To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria. The preliminary Vienna 2005 consensus conference, which proposed preliminary criteria for paediatric vasculitides, was followed by a EULAR/PRINTO/PRES - supported validation project divided into three main steps. Step 1: retrospective/prospective web-data collection for HSP, c-PAN, c-WG and c-TA, with age at diagnosis

  11. Granulomatose de Wegener – Forma limitada

    Directory of Open Access Journals (Sweden)

    I. Gil

    2002-01-01

    Full Text Available RESUMO: Doente do sexo feminino, 53 anos de idade, internada por lesões nodulares pulmonares sugestivas de doença metastática.Antecedentes de Doença de Graves e de Pyoderma gangrenosum.Biópsia pulmonar compatível com Granulomatose de Wegener.REV PORT PNEUMOL 2002; VIII (1: 87-93 ABSTRACT: Female, 53 years old, with nodular lung lesions suggesting metastatic disease.This patient had clinical antecedents of Graves disease and Pyoderma gangrenosum.The lung biopsy was compatible with Wegener’ Granulomatosis.REV PORT PNEUMOL 2002; VIII (1: 87-93 Palavras-chave: granulomatose, Wegener, forma limitada, vasculite, Key-words: granulomatosis, Wegener, limited, vasculitis

  12. Prevalência das manifestações clínicas iniciais da granulomatose de Wegener no Brasil: relato de seis casos e revisão da literatura Wegener's granulomatosis: prevalence of the initial clinical manifestations - report of six cases and review of the literature

    Directory of Open Access Journals (Sweden)

    Carlos Ewerton Maia Rodrigues

    2010-04-01

    Full Text Available OBJETIVOS: Descrever as manifestações clínicas iniciais da Granulomatose de Wegener (GW diagnosticada no Brasil. PACIENTES E MÉTODOS: Análise retrospectiva de seis prontuários do Serviço de Reumatologia do Hospital Geral de Fortaleza (HGF, assim como a realização de um levantamento bibliográfico dos casos de GW descritos no Brasil obtidos dos bancos de dados LILACS, SciELO e MEDLINE. RESULTADOS: O estudo identificou 49 pacientes; 15 (31% do sexo masculino e 34 (69% do sexo feminino. A forma sistêmica ocorreu em 35 pacientes (73%: 28 adultos, cinco crianças e dois adolescentes. A doença limitada ocorreu em 13 adultos e uma criança. A média da idade adulta no início da doença foi de 42,2 anos (18 a 65 anos. O quadro clínico agudo, com sintomas há menos de três meses do diagnóstico, ocorreu em 41% (20/49 da casuística e a forma insidiosa, em 59% (29/49 dos pacientes. A prevalência das manifestações clínicas iniciais nos adultos com doença sistêmica (n = 28 foi 64% (18/28 das vias aéreas superiores (VAS, 36% (10/28 pulmonares, 18% (5/28 renais, 25% (7/28 oculares, 11% (3/28 cutâneas, 25% (7/28 musculoesqueléticas e 7% (2/28 neurológicas. Na forma limitada do adulto (n = 13, os sintomas prevalentes foram 84% (11/13 VAS, 23% (3/13 oculares e 15% (2/13 pulmonares. CONCLUSÃO: No Brasil, a prevalência das manifestações clínicas iniciais da GW foi semelhante aos resultados da literatura. A falta de especificidade dos sintomas pode retardar o diagnóstico na forma insidiosa da doença e aumentar a morbimortalidade das formas agudas.OBJECTIVES: To describe the initial clinical manifestations of Wegener's Granulomatosis (WG in Brazil. PATIENTS AND METHODS: Retrospective analysis of six medical records of WG patients followed-up at the Rheumatology Department of Hospital Geral of Fortaleza (HGF, as well as a bibliographic survey of cases of WG in Brazil on LILACS, SciELO, and MEDLINE databases. RESULTS: The study

  13. Granulomatosis with Polyangiitis (Wegener Granulomatosis Mimicking Infective Endocarditis

    Directory of Open Access Journals (Sweden)

    Imène Rachdi

    2014-10-01

    IE should be ruled out before initiation of immunosuppressive treatment. If the disease progresses despite antimicrobial treatment, vascular diseases should be rapidly taken into account in differential diagnosis and treated early to avoid fatal complications.

  14. Adolescente femenino con granulomatosis de Wegener fulminante

    Directory of Open Access Journals (Sweden)

    S C Scheffler-Mendoza

    2017-01-01

    Full Text Available Adolescente femenino de 13 años de edad, originaria de Guerrero, grupo étnico mixteco, padres analfabetos y con poco entendimiento del español. Sin otros antecedentes de importancia para el padecimiento. Inició un mes previo con la aparición de una pápula hipercrómica en párpado superior derecho que en los 10 días previos al ingreso aumentó de volumen generando proptosis. Al interrogatorio se negó dolor, lagrimeo, fiebre, o pérdida de peso. Ingresó con fiebre (39.0°C, palidez generalizada, proptosis derecha con exposición de córnea y conjuntiva, eritema periorbitario, movimientos oculares y agudeza visual disminuidos (i.e. contaba dedos a 1 m, pupila con tendencia a midriasis, hiperreactividad a la luz, y fondo de ojo con papila hiperémica edematosa y tortuosidad peripapilar. El resto de exploración no mostró datos relevantes. Se hospitalizó para iniciar tratamiento antimicrobiano parenteral de amplio espectro (i.e. ceftriaxona y vancomicina y abordar proptosis unilateral.

  15. Accelerated atherosclerosis in patients with Wegener's granulomatosis

    NARCIS (Netherlands)

    de Leeuw, K; Sanders, J-S; Stegeman, C; Smit, A.; Kallenberg, C G; Bijl, M

    2005-01-01

    BACKGROUND: Several autoimmune disorders are complicated by excess cardiovascular disease. In addition to traditional risk factors, non-traditional risk factors such as endothelial activation and excessive vascular remodelling might be determinants of the progression of atherosclerosis in patients

  16. Prostatitis and acute urinary retention as first manifestations of Wegnener's granulomatosis.

    Science.gov (United States)

    Pereira Beceiro, Javier; Rodríguez Alonso, Andrés; Bonelli Martín, Carlos; Pérez Valcárcel, Javier; Mosquera Seoane, Teresa; Cuerpo Pérez, Miguel Ángel

    2014-01-01

    We present a case of prostatitis with acute urinary retention as a rare initial manifestation of Wegener' Granulomatosis. The case was a 48-year-old male with symptoms of prostatitis over ten days. The patient presented urinary retention, with partial response to antibiotic treatment. High levels of cytoplasmic antineutrophil cytoplasmic antibody and a prostatic biopsy were compatible with Wegener' Granulomatosis. After starting treatment with glucocorticoids and cyclophosphamide, a significant improvement to the point of disappearance of symptoms was observed. At 3 months pulmonary and upper airway symptoms began, requiring higher doses of cyclophosphamide to control symptoms. Wegener's Granulomatosis is a multisystem entity whose presentation as prostatitis with urinary retention is rare. Copyright © 2012 Elsevier España, S.L.U. All rights reserved.

  17. Orofacial granulomatosis

    OpenAIRE

    Zhang, Wenyi; Wang, Jia; Yu, Xixi; Wang, Wanchun

    2017-01-01

    Abstract Rationale: Orofacial granulomatosis (OFG) is a rare disease characterized by noncaseating granulomatous inflammation. The most common clinical presentation is persistent swelling of the soft tissues in the oral and maxillofacial regions. The precise cause of OFG is unknown. Corticosteroids are the first-line and best treatment, but there is lack of uniform treatment prescription and standard. It is important to identify the pathogen in order to improve treatment specificity. Patient ...

  18. Gravidez e granulomatose de Wegener: urn caso clinic

    Directory of Open Access Journals (Sweden)

    P. Monteiro

    1998-11-01

    Full Text Available RESUMO: A granulomatose de Wegener (GW é uma vasculite com manifestações multissistémicas, atingindo frequentemente o aparelho respiratório, e o rim.Descreve-se um caso clinico cuja manifestação inaugural da doença ocorreu no pulmão (hemorragia alveolar difusa numa mulber grávida, situação esta pouco frequente. A doente apresentava envolvimento renal assintomático. 0 diagnóstico baseou-se num quadro clinico coerente com envolvimento do polmão e rim e na existência de um cANCA positivo.REV PORT PNEUMOL 1998; IV (6: 611-615 ABSTRACT: Wegener granulomatosis (WG is a vasculitis with multisystemic manifestations, affecting more frequently the upper and lower respiratory tract and the kidney. The authors describe a case where the ftrst symptoms of WG (a severe pulmonary hemorrage appeared in a pregnant woman, which is a rare situation.The patient bad assymptomatic kidney involvement. The diagnosis was supported by a typical and consistent clinical findings (lung and kidney involvement and a positive c ANCA.REV PORT PNEUMOL 1998; IV (6: 611-615 Key-words: Wegener granulomatosis, Pregnancy, Alveolar pulmonary hemorrhage, Palavras-chave: Granulomatose de Wegener, Gravidez, Hemorragia alveolar

  19. Granulomatosis With Polyangiitis-Associated Hypertrophic Pachymeningitis Mimicking Spontaneous Intracranial Hypotension: A Case Report.

    Science.gov (United States)

    Jung, Young Hee; Lee, Mi Ji; Lee, Chungbin; Cha, Jihoon; Chung, Chin-Sang

    2017-03-01

    Dural enhancement is a characteristic finding in both spontaneous intracranial hypotension and hypertrophic pachymeningitis. Positional headache is the most important feature that distinguishes the two diseases. We report a patient with granulomatosis with polyangiitis (formerly Wegener's granulomatosis) who initially manifested like spontaneous intracranial hypotension. We report here the case of a 63-year old man who presented with severe positional headache. The patient had typical symptoms, symmetric dural enhancement, and a recent history of nontraumatic subdural hygroma which led to the diagnosis of spontaneous intracranial hypotension, but was finally diagnosed as granulomatosis with polyangiitis-associated secondary hypertrophic pachymeningitis. Cyclophosphamide therapy was effective for the maintenance of remission. Hypertrophic pachymeningitis associated with granulomatosis with polyangiitis can present with positional headache and subdural hygroma, mimicking spontaneous intracranial hypotension. Granulomatosis with polyangiitis should be suspected when patients with spontaneous intracranial hypotension or hypertrophic pachymeningitis show atypical features. © 2016 American Headache Society.

  20. Diagnostic Consideration for Sinonasal Wegener’s Granulomatosis Clinically Mistaken for Carcinoma

    Directory of Open Access Journals (Sweden)

    Cristina La Rosa

    2013-01-01

    Full Text Available We report a case of Wegener’s granulomatosis clinically mistaken for carcinoma in a 21-year-old girl presenting with an ulcerated mass of the nasopharynx associated with enlarged laterocervical nodes. The lesion was clinically suspected as malignant on the basis of clinical and radiological findings (namely, computed tomography scan and positron emission tomography. However, multiple biopsies were not conclusive for malignancy showing histological change suggestive of Wegener’s granulomatosis. A serum determination of cANCA supported the diagnosis of Wegener’s granulomatosis. Clinical findings and image studies suggested an erroneous diagnosis of malignancy whereas a definitive diagnosis of Wegener’s granulomatosis was achieved only after repeated biopsies thus leading to a correct therapeutic approach. The Wegener granulomatosis must be added to the list of the differential diagnoses of the masses of the nasopharynx associated with or without enlarged laterocervical nodes.

  1. Atlantoaxial subluxation and nasopharyngeal necrosis complicating suspected granulomatosis with polyangiitis.

    Science.gov (United States)

    Mohapatra, Anand; Holekamp, Terrence F; Diaz, Jason A; Zebala, Lukas; Brasington, Richard

    2015-04-01

    Granulomatosis polyangiitis (GPA, formerly Wegener granulomatosis) is a vasculitis that typically involves the upper respiratory tract, lungs, and kidneys. The 2 established methods to confirm a suspicion of GPA are the antineutrophil cytoplasmic antibody (ANCA) test and biopsy. However, ANCA-negative cases have been known to occur, and it can be difficult to find biopsy evidence of granulomatous disease.We report a case of suspected granulomatosis with polyangiitis limited to the nasopharynx. With a negative ANCA and no histological evidence, our diagnosis was founded on the exclusion of other diagnoses and the response to cyclophosphamide therapy. This case is unique because the patient's lesion resulted in atlantoaxial instability, which required a posterior spinal fusion at C1-C2. This is the first reported case of suspected GPA producing damage to the cervical spine and threatening the spinal cord.

  2. Cranial manifestation of Wegener's granuolomatosis: CT and MRI characteristics; Kraniale Manifestation des Morbus Wegener: Aktueller Stand der Diagnostik

    Energy Technology Data Exchange (ETDEWEB)

    Hartmann, M.; Siebert, S.; Rohde, S. [Abt. Neuroradiologie, Neurologische Klinik, Universitaetsklinikum Heidelberg (Germany)

    2006-01-01

    Wegener's granulomatosis is a rare systemic granulomatous disease which primarily involves the upper and lower respiratory tract and the kidneys. In addition the orbits, the peripheral and the central nervous system may also be affected. Computed tomography and magnetic resonance imaging have been proven to be quite useful for the detection of these lesions, for assessment of the extent of involvement, and for documentation of response to therapy. The drawback of CT and MR studies is that they lack specifity. Characteristic imaging findings for WG is a medial wall destruction of the maxillary sinus with gross thickening of the anterior and infratemporal walls with relatively low soft tissue proliferation. On T2-weighted MR images the granulomatous tissue appears markedly hypointense with dense enhancement. Although these findings are not specific, if they are obvious, Wegener's granulomatosis should be considered in the differential diagnosis. (orig.)

  3. Demonstration of Wegener's granuloma on mammography

    Energy Technology Data Exchange (ETDEWEB)

    Deininger, H.K.

    1984-08-01

    Communication about two patients with Wegener's granulomatosis of the mamma. In both cases the rare disease was detected on the occasion of a mammographic examination. The results of the mammography could cause misinterpretations, e.g. it might be mistaken for an advanced mamma carcinoma. Up to now, the manifestation of a Wegener's disease of the female breast was neither mentioned in collective statistics of more than 324 cases nor could it be found in examining the publications of additional 231 cases. Both patients showed an unusual course of the disease with a slow progression as well as an unimportant affection of the respiratory tract. The regression of the tumor like lesions in the breast was remarkable. 8 figs.

  4. Th2 dominance in nasal mucosa in patients with Wegener’s granulomatosis

    Science.gov (United States)

    Balding, C E J; Howie, A J; Drake-Lee, A B; Savage, C O S

    2001-01-01

    Wegener's granulomatosis initially affects upper respiratory tract organs including the nasal mucosa in more than 90% of patients. The inflammation is typically granulomatous with associated vasculitis. T lymphocytes are usually a prominent component of the leucocyte infiltrate. Previous studies using peripheral blood T cells have implicated IFN-γ rich Th1-type responses. This study addressed the cytokine milieu in nasal mucosa from 10 patients with active Wegener's granulomatosis using immunohistochemistry. Increased levels of CD3+ T cells and eosinophils were present compared with normal and disease controls. There was increased expression of IL-4, down-regulation of IL-2 and no detectable IFN-γ. There was increased expression of the chemokine receptor CCR3 by infiltrating cells, consistent with an IL-4 dominant, Th2-biased response. In contrast, renal biopsy tissue from 10 patients with active Wegener's granulomatosis showed expression of IL-2 and IL-4. The Th2-type environment within nasal mucosa, often the initial site of disease activity in Wegener's, is consistent with a local allergic response in these patients. PMID:11529927

  5. Wegner's granulomatosis developing for the first time in a patient eight years after starting maintenance hemodialysis.

    Science.gov (United States)

    Ali, Ahmad Ramadan; El-Reshaid, Kamel

    2015-11-01

    Wegener's granulomatosis is a serious autoimmune disorder characterized by necrotizing small-vessel vasculitis. It is a multisystem disease that primarily affects the lung and kidneys. Previous studies indicated few relapses of vasculitis after hemodialysis due to uremic immunosuppression. Our case report describes an end-stage renal failure patient who had developed non-caseating lung granulomata with giant cell formation and fibrinoid necrosis of arterial media that is consistent with Wegner's granulomatosis for the first time and eight years after initiation of maintenance hemodialysis. We believe that such a phenomenon has rarely been reported.

  6. Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness.

    Science.gov (United States)

    Kim, Sang Hoon; Jung, A Ra; Kim, Su Il; Yeo, Seung Geun

    2016-04-01

    Granulomatosis with polyangiitisis [(GPA) or Wegener granulomatosis] is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. GPA classically shows involvement of the respiratory tracts and the renal system. However, locoregional disease is common and may include otologic manifestations. Although otologic involvement can occur during the course of GPA, no report has described facial palsy with sudden sensorineural total deafness with vertigo as the presenting feature of GPA. This case describes a patient with multiorgan involving resistant form of GPA initially presenting with bilateral profound sudden sensorineural hearing loss and left facial paralysis with vertigo. The condition responded well to treatment with rituximab.

  7. Coexistence of granulomatosis with polyangiitis (GPA) and Crohn's disease or multiorgan manifestation of the same disease?

    Science.gov (United States)

    Jóźwiak, Lucyna; Ławnicka, Izabela; Książek, Andrzej

    2016-01-01

    Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis of unknown aetiology, often related to the antineutrophil cytoplasmic antibody (ANCA). GPA was previously named Wegener's granulomatosis (WG). The disease frequently has multisystemic presentation, targeting mainly the respiratory tract and kidneys, but gastrointestinal involvement is uncommon. Crohn's disease (CD) is an inflammatory bowel disease (IBD) with many extraintestinal manifestations. Clinically, symptoms of WG and CD can mimic each other. In this paper a case of GPA manifested initially by severe multiorgan damage including colitis, regarded to be coexistent CD, is presented. The case illustrates the difficulties in establishing the diagnosis when symptoms of the diseases mimic each other.

  8. Thoracic gallium uptake in patients with lymphomatoid granulomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Tien, R.; Moore, W.H.; Glasser, L.M.; Dhekne, R.D.; Long, S.E.

    1988-12-01

    Lymphomatoid granulomatosis (LG) is a rare condition with histological similarities to Wegener's granulomatosis and malignant lymphoma. Characteristically there is an angiocentric, angiodestructive lymphoreticular cell infiltrate. The lungs are usually affected, and, less frequently, the skin, nervous system, kidney, and bowel are involved. The prognosis is poor and frank lymphoma develops, in some cases terminally. The usual radiological appearance of the lungs consists of bilateral nodular lower zone opacities. The authors report two patients (siblings) with LG, and their gallium scans are presented. In each case there was a significant accumulation of gallium in the lungs at times of clinically active disease. The limited role of gallium imaging in this disease is discussed.

  9. Genetic Variability of RXRB, PPARA, and PPARG in Wegener's Granulomatosis

    Directory of Open Access Journals (Sweden)

    Stefan Wieczorek

    2009-01-01

    In order to reveal any hitherto unknown and potentially disease-relevant variation of the RXRB gene, we have genotyped four tagging SNPs of this genomic region and have directly sequenced selected WG patients and controls representing disease-associated haplotypes. Additionally, we have genotyped 2 SNPs each in the genes for PPARα and PPARγ (PPARA and PPARG. Hence, we confirmed the strong association of the RXRB locus with WG but could not reveal any novel variation in RXRB. None of the PPARA and PPARG SNPs showed association with WG. Moreover, no epistatic effect was seen between RXRB and PPARA/PPARG alleles. These results do not support an etiopathological role of PPAR in WG. Analyses of further genes functionally linked to RXRB may provide additional data useful to evaluate the RXRB association found in WG.

  10. A Presentation of Cerebritis Secondary to Granulomatosis with Polyangiitis (Wegener

    Directory of Open Access Journals (Sweden)

    James Norman

    2014-01-01

    Full Text Available Neurological manifestations of GPA are common, most frequently as a peripheral neuropathy. Cerebritis as a principal presentation is extremely rare. We report a patient who presented with subacute progression of ataxia, confusion, and vacant episodes. An MRI of her brain showed bilateral signal abnormalities in the cingulate and superior sagittal gyrus while a staging CT revealed a mass in the right upper lobe of the patient’s lung with a satellite nodule. C-ANCA antibodies specific for PR3 at high titres were positive and a diagnosis of GPA was made. The patient was commenced on intravenous methylprednisolone followed by cyclophosphamide and responded well to treatment. GPA is a rare and treatable differential diagnosis for confused patients with acute or subacute neurological features and unusual MRI findings.

  11. A presentation of cerebritis secondary to granulomatosis with polyangiitis (wegener).

    Science.gov (United States)

    Norman, James; Pande, Ira; Taylor, Timothy; Gran, Bruno

    2014-01-01

    Neurological manifestations of GPA are common, most frequently as a peripheral neuropathy. Cerebritis as a principal presentation is extremely rare. We report a patient who presented with subacute progression of ataxia, confusion, and vacant episodes. An MRI of her brain showed bilateral signal abnormalities in the cingulate and superior sagittal gyrus while a staging CT revealed a mass in the right upper lobe of the patient's lung with a satellite nodule. C-ANCA antibodies specific for PR3 at high titres were positive and a diagnosis of GPA was made. The patient was commenced on intravenous methylprednisolone followed by cyclophosphamide and responded well to treatment. GPA is a rare and treatable differential diagnosis for confused patients with acute or subacute neurological features and unusual MRI findings.

  12. Cancer preceding Wegener's granulomatosis: a case-control study

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Mellemkjaer, Lene; Sorensen, Inge J

    2009-01-01

    OBJECTIVE: To investigate whether patients with WG have an increased risk of malignancies prior to and/or around the time of the vasculitis diagnosis, as suggested by previous studies. METHODS: A total of 293 WG patients were included in the study. Ten gender- and age-matched controls were select...

  13. Wegener’s granulomatosis and pyoderma gangrenosum – rare causes of facial ulcerations

    OpenAIRE

    Karolina Kędzierska; Krzysztof Sindrewicz; Tomasz Smektała; Magda Wiśniewska; Marek Masiuk; Ewelina Staniszewska; Katarzyna Sporniak- Tutak; Edyta Gołembiewska; Kazimierz Ciechanowski

    2016-01-01

    Background: Pyoderma gangrenosum (PG) is caused by immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all PG patients also suffer from autoimmunological diseases, one of which is Wegener granulomatosis (WG). The purpose of this article was to compare cases of patients with WG and PG in terms of their clinical course, histopathology, and applied treatment. In both, histopathological features are not fully distinct. Data from microbiological and im...

  14. Granulomatosis with Polyangiitis initially misdiagnosed as lung cancer

    Directory of Open Access Journals (Sweden)

    S. Campainha

    2013-01-01

    Full Text Available Granulomatosis with Polyangiitis (GPA, which was formerly named Wegener's Granulomatosis (WG is a systemic disease characterized by necrotizing granulomatous inflammation and vasculitis that primarily involves upper and lower respiratory tract, as well as kidneys. Diagnosing GPA on the basis of transthoracic fine needle aspiration (TFNA may be problematic, as it can be misdiagnosed as cancer. We describe a patient with a probable GPA which was originally diagnosed as malignancy, but who responded to lung cancer chemotherapy. Resumo: A granulomatose com poliangeíte (GPA, previamente denominada granulomatose de Wegener, é uma doença sistémica caracterizada por inflamação granulomatosa necrotizante e vasculite que envolve principalmente o trato respiratório superior e inferior, bem como os rins. O diagnóstico de GPA com base em biópsia por agulha fina (BAF transtorácica pode ser problemático, levando ao diagnóstico incorreto de cancro. Os autores descrevem o caso de um paciente com provável GPA, inicialmente diagnosticado como cancro do pulmão, que curiosamente respondeu à quimioterapia para cancro do pulmão. Keywords: Systemic vasculitis, Non-small-cell lung carcinoma, Fine-needle biopsy, Palavras-chave: Vasculite sistémica, Carcinoma não-pequenas células, Biópsia por agulha fina

  15. Three atypical manifestations of granulomatosis with polyangiitis: lateral medullary syndrome, anterior cheek mass and melting scleritis of eye.

    Science.gov (United States)

    Shenavandeh, Saeedeh; Petramfar, Peyman

    2017-01-01

    Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis) is a vasculitis with various organ involvement. There have been a few cases of CNS stroke and rare cases of lateral medullary infarction (LMI) as a manifestation of GPA. Also there have been reports of sinuses, nose and laryngeal masses mistakenly referred as carcinomas and subsequently GPA was diagnosed in their pathological reports. Another severe fulminant manifestation can be necrotizing scleritis leading to perforation of sclera. Therefore, here we present some rare and fulminant manifestations of GPA in 3 separate cases for further emphasis of the unusual manifestations of GPA that should always be kept in mind.

  16. Granulomatose de Wegener – Envolvimento otológico, nasal, laringotraqueal e pulmonar

    Directory of Open Access Journals (Sweden)

    Sandra Figueiredo

    2009-09-01

    Full Text Available Resumo: A granulomatose de Wegener é uma vasculite sistémica rara e idiopática caracterizada pelo atingimento dos pequenos vasos. A doença atinge, preferencialmente, as vias aérea superior e inferior e os rins, levando à formação de granulomas e necrose destes órgãos. As manifestações clínicas e o envolvimento orgânico variam largamente. O diagnóstico e tratamento precoce podem levar à recuperação total. No entanto, o atraso no diagnóstico pode ser fatal. Os autores apresentam o caso de uma doente de 33 anos com uma forma de apresentação grave e rara da doença, mas com uma evolução favorável após diagnóstico e tratamento adequado. Abstract: Wegener granulomatosis is a rare systemic idiopathic disease characterized by involvement of small vessels – medium and small arteries, venules, arterioles and ocasionally large arteries. This disease has predilection for the upper and lower respiratory tract and the kidney, with granulomatous inflamation and necrosis. Clinical manifestations and organ involvement of the disease vary widely. Early diagnosis and treatment may lead to a full recovery. Without treatment, Wegener's granulomatosis can be fatal. The authors present a case of a 33 year-old female, with severe disease, but with good outcome, after adequate diagnosis and treatment. Palavras-chave: Granulomatose de Wegener, forma limitada grave, Key-words: Wegeneŕs granulomatosis, severe limited form

  17. Total Nasal Skeletal Reconstruction Disfigured by Granulomatosis with Polyangitis (Wegener Granulomatosis

    Directory of Open Access Journals (Sweden)

    Farhad Hafezi, MD

    2015-02-01

    Conclusions: In spite of contamination to the nasal lining and immune-compromising medications, nasal reconstruction is usually successful when considering these factors. Access to strong and abundant fifth or sixth rib cartilage presents an opportunity to reconstruct nasal destruction due to iatrogenic or autoimmune processes.

  18. Necrotizing sarcoid granulomatosis: A distinctive form of pulmonary granulomatous disease.

    Science.gov (United States)

    Karpathiou, Georgia; Batistatou, Anna; Boglou, Panagiotis; Stefanou, Dimitrios; Froudarakis, Marios E

    2017-07-29

    To define the characteristics of necrotizing sarcoid granulomatosis (NGS) a very rare pulmonary disease hardly recognised by pulmonologists and pathologists. PubMed was searched for the term necrotising or necrotizing sarcoid granulomatosis. All cases reported in the English literature were included. NGS is presented at all ages (range 8-68 years) with a median age of 42 years old. It shows female (62%) and Caucasian (80%) predominance. The most frequent symptoms are cough, fever, dyspnoea and chest pain. Extra-pulmonary involvement is found in one third of the cases, with ocular being the most common (12.5%). At imaging, multiple nodules (64.75%) or a solitary mass (20.49%) are found accompanied by mediastinal lymphadenopathy at one third of the cases. It can be clinically mistaken for malignancy as it is tumour-like, increases rapidly in size and it is hyperfixating in PET-SCAN. Histologically, NGS is defined by large areas of necrosis, well-formed granulomas and vascularitis. NGS is a disease often confounded clinically with malignancy or with sarcoidosis even histologically when all criteria are not strictly applied. This review provides NGS' characteristics and discusses its differential diagnosis form sarcoidosis, Wegener granulomatosis and tuberculosis. © 2017 John Wiley & Sons Ltd.

  19. Granulomatosis With Polyangiitis (Wegener’s)

    Science.gov (United States)

    Springer, Jason; Nutter, Benjamin; Langford, Carol A.; Hoffman, Gary S.; Villa-Forte, Alexandra

    2014-01-01

    Abstract To determine outcomes in relation to duration of maintenance therapy in patients with granulomatosis with polyangiitis (Wegener’s) (GPA), we conducted a retrospective chart review of patients with GPA seen at a single vasculitis center from 1992 to 2010. All patients achieved remission defined by a Birmingham Vasculitis Activity Score for Wegener Granulomatosis (BVAS/WG) of 0 with either cyclophosphamide or methotrexate. After achieving remission all patients were started on maintenance therapy with either methotrexate or azathioprine. The study comprised 157 patients with a median follow-up of 3.1 years. Using a univariate model, the continuation of maintenance medications for >18 months showed a 29% reduction in hazard ratio (HR) for relapse (HR, 0.71; 95% confidence interval [CI], 0.42–1.19; p = 0.19). Treatment for >36 months showed a 66% reduction in hazard ratio for relapse (HR, 0.34; 95% CI, 0.15–0.76; p = 0.008). When length of treatment was considered as a continuous factor, longer courses had an inverse relationship with the risk of relapse (HR, 0.70; 95% CI, 0.58–0.84; p < 0.001), which remained significant after adjusting for prednisone dose (HR, 0.59; 95% CI, 0.42–0.83; p = 0.003). Fifty-two percent of relapses occurred while the patients were off maintenance therapy. Among all patients who relapsed on therapy, 52% of those receiving methotrexate were on <15 mg/week, and 67% of those receiving azathioprine were on ≤50 mg/d. There were no differences between the short- and long-term maintenance therapy groups in overall adverse events or GPA-related morbidity. Discontinuation or use of low doses of maintenance therapy is associated with a higher relapse rate. PMID:24646464

  20. Rituximab seems a promising therapeutic option in granulomatosis with polyangiitis with intestinal perforation: a case report and literature review.

    Science.gov (United States)

    Dag, Muhammet Sait; Pehlivan, Yavuz; Tutar, Ediz; Kisacik, Bunyamin

    2013-01-22

    Granulomatosis with polyangiitis (Wegener's) (GPA) is a chronic disease of unknown aetiology that leads to necrotising vasculitis in small and medium-sized vessels characterised by respiratory system and kidney involvement. Intestinal involvement is rare and perforation is even rarer in GPA. In this study, we are presenting a literature review of related cases, and a 29-year-old man referred from the emergency department with a multiple distal ileal perforation that was diagnosed with GPA, and successfully treated with rituximab.

  1. Lymphomatoid granulomatosis with ocular involvement.

    Science.gov (United States)

    Tse, D T; Mandelbaum, S; Chuck, D A; Nichols, P W; Smith, R E

    1985-01-01

    Lymphomatoid granulomatosis is an angiocentric and angiodestructive lymphoproliferative disorder that involves multiple organs including the lung, skin, kidney and central nervous system. A case of lymphomatoid granulomatosis with ocular involvement is reported herein. Bilateral peripheral retinal vasculitis involving both arterioles and veins, with posterior uveitis, is the prominent clinical finding.

  2. Treatment associated Mantle Cell Lymphoma with Cyclophosphamide therapy for Granulomatosis with Polyangiitis.

    Science.gov (United States)

    Sheehy, Robert; Tran, Khoa

    2015-03-01

    We present a case of a 68-year-old woman who developed mantle cell lymphoma in the setting of long-term cyclophosphamide therapy and relapsing granulomatosis with polyangiitis (Wegener's granulomatosis, GPA). Adverse outcomes associated with cyclophosphamide therapy are well documented; however, the development of non-Hodgkin's lymphoma appears rare. Cumulative dose of cyclophosphamide (>36 g) is a significant risk factor in the development of serious long-term adverse outcomes and is particularly relevant to this case in which maintenance cyclophosphamide therapy was continued, following induction, for 2 years total, on account of patient preference. This case study will highlight the patient's initial diagnosis, treatment response, relapse and subsequent complications of therapy.

  3. Necrotizing sarcoid granulomatosis with an uncommon manifestation: clinicopathological features and review of literature.

    Science.gov (United States)

    Giraudo, Chiara; Nannini, Nazarena; Balestro, Elisabetta; Meneghin, Alessia; Lunardi, Francesca; Polverosi, Roberta; Calabrese, Fiorella

    2014-09-01

    We report a rare case of an incidental diagnosis of necrotizing sarcoid granulomatosis (NSG) in a 60-y-old non-smoking male. The patient was admitted to the hospital for sudden back pain. Chest x-ray revealed areas of parenchymal consolidation and high-resolution computed tomography demonstrated a pulmonary nodular pattern with no lymph node enlargement. All laboratory and pulmonary function tests were normal. Bronchoscopy with bronchoalveolar lavage showed no sign of infection or specific inflammation. The diagnosis of NSG was made by histopathological examination of a surgical lung biopsy and by excluding other causes of granulomatous disease. In paucisymptomatic/asymptomatic patients, as in our case, therapy is not necessary, with a good prognosis and complete recovery. NSG is a rare systemic disease similar to sarcoidosis and Wegener's granulomatosis with a benign clinical course and should always be considered for patients with nodular pulmonary lesions even with subclinical or uncommon features. Copyright © 2014 by Daedalus Enterprises.

  4. [Acute coronary syndrom as a cardiac manifestation of granulomatosis with polyangiitis].

    Science.gov (United States)

    Asdonk, T; Tiyerili, V; Dörner, J; Thomas, D; Schaefer, C; Skowasch, D; Nickenig, G; Pabst, S

    2013-02-01

    A 49-year-old patient was admitted to our ward because of a troponin elevation (non ST-elevation myocardial infarction) following a rhinoscopy in an external hospital. The patient complained of typical angina, chronic rhinitis and epistaxis. Analysis of the nasal biopsy had shown the histological finding of granulomatosis with polyangiitis (Wegener's granulomatosis). The consecutively performed catheterization showed a coronary one-vessel disease without significant stenosis. Echocardiography showed diastolic dysfunction as well as hemodynamically not significant pericardial effusion. The MRI scan of the heart revealed multiple myocardial scars located ventricular apical and septal. Extended bilateral pulmonary opacities in the thoracic CT scan, microhematuria, leukocyturia and proteinuria indicated multi-organ involvement of the small vessel disease. The patient's condition improved quickly in response to steroids and cyclophosphamide, followed by attenuation of clinical symptoms and normalizing blood and renal parameters. The prognosis of granulomatosis with polyangiitis is mainly limited by renal and pulmonal involvement. Cardiac involvement is commonly rare and associated with clinical courses refractory to immunosuppressive therapy. Generally, all cardiac structures can be affected, thereby impending serious cardiac events. Normally, granulomatosis with polyangiitis responds quickly to immunosuppressive therapy, associated with a rather good prognosis without higher mortality. © Georg Thieme Verlag KG Stuttgart · New York.

  5. Initial diagnosis of Wegener’s granulomatosis mimicking severe ulcerative colitis: a case report

    Science.gov (United States)

    2013-01-01

    Introduction We describe the case of a woman with an unusual presentation of Wegener’s granulomatosis. Case presentation A 20-year old Caucasian woman presented with the principal feature of a pancolonic, superficial microulceration mimicking severe ulcerative colitis. Our patient was refractory to therapy and had persisting signs of septic shock as well as being at risk of perforation, so we performed a subtotal colectomy and a cholecystectomy due to the incipient necrosis of her gallbladder. Histologic analysis of her colon showed multiple superficial microulcera of the mucosa, lamina propria mucosae and, to a lesser extent, the lamina submucosa. The medium-sized arteries and arterioles of her entire colon, appendix and gallbladder showed acute vasculitic changes with fibrinoid necrosis of the walls and diffuse infiltration with neutrophil granulocytes, accompanied by a strong perivascular histiocyte-rich and partially granulomatous reaction. These findings strongly suggested an autoimmune multisystem disease like Wegener’s granulomatosis or microscopic polyangiitis. A diagnosis of Wegener’s granulomatosis was confirmed by the results of serologic antibody tests: her cytoplasmic antineutrophil cytoplasmic antibody titer was considerably elevated at 1:2560 specific for subclass proteinase 3 (>200kU/L). After the histopathological diagnosis and serological tests, immunosuppression with high doses of corticosteroids and plasmapheresis was started. Conclusion In critically ill patients with severe, therapy-refractory ulcerative colitis, Wegener´s granulomatosis should be considered and serologic antibody testing should be performed. PMID:23718545

  6. [Chronic granulomatosis in childhood].

    Science.gov (United States)

    Fakan, F

    1977-08-01

    In a boy aged 8 years suffering from chronic granulomatosis of childhood necrotizing pneumonie and small pulmonary tuberculoid granulomas containing filaments of moulds were found at autopsy. Necrotizing leucocytic granulomas were present in the liver, spleen and the lymph nodes. All the organs showed aggregates of histiocytes containing yellowish cytoplasmic deposits of lipogment surrounded by a high acid phosphatase activity. The NBT-reduction leucocytic tests was repeatedly negative in vivo. The activities of NADH- and NADPH-tetrazolium reductase and succinate dehydrogenase in the tissues were histochemically normal.

  7. Granulomatose de Wegener – Envolvimento otológico, nasal, laringotraqueal e pulmonar

    Directory of Open Access Journals (Sweden)

    Sandra Figueiredo

    2009-09-01

    Full Text Available Resumo: A granulomatose de Wegener é uma vasculite sistémica rara e idiopática caracterizada pelo atingimento dos pequenos vasos. A doença atinge, preferencial-mente, as vias aérea superior e inferior e os rins, levando à formação de granulomas e necrose destes órgãos. As manifestações clínicas e o envolvimento orgânico variam largamente. O diagnóstico e tratamento precoce podem levar à recuperação total. No entanto, o atraso no diagnóstico pode ser fatal. Os autores apresentam o caso de uma doente de 33 anos com uma forma de apresentação grave e rara da doença, mas com uma evolução favorável após diagnóstico e tratamento adequado.Rev Port Pneumol 2009; XV (5: 929-935 Abstract: Wegener granulomatosis is a rare systemic idiopathic disease characterized by involvement of small vessels – medium and small arteries, venules, arterioles and ocasionally large arteries. This disease has predilection for the upper and lower respiratory tract and the kidney, with granulomatous inflamation and necrosis. Clinical manifestations and organ involvement of the disease vary widely. Early diagnosis and treatment may lead to a full recovery. Without treatment, Wegener’s granulomatosis can be fatal. The authors present a case of a 33 year-old female, with severe disease, but with good outcome, after adequate diagnosis and treatment.Rev Port Pneumol 2009; XV (5: 929-935 Palavras-chave: Granulomatose de Wegener, forma limitada grave, Key-words: Wegener´s granulomatosis, severe limited form

  8. Multiple Intestinal Perforation Due To Wegener?s Granulotamosis: Case Report

    Directory of Open Access Journals (Sweden)

    Nazif Zeybek

    2010-05-01

    Full Text Available Wegener’s granulomatosis is one of the well known vasculitis, which results with necrotizing granulomatous lesions in the upper and lower respiratuary system and glomerulonephritis. Vasculitis is also observed in several other tissues and organ systems including gastrointestinal system. However, gastrointestinal involvement in Wegener’s granulomatosis is extremely rare. This report presents a patient with Wegener’s granulomatosis, wo was diagnosed to have multiple intestinal perforations.

  9. Pituitary Involvement in Granulomatosis With Polyangiitis

    Science.gov (United States)

    De Parisot, Audrey; Puéchal, Xavier; Langrand, Corinne; Raverot, Gerald; Gil, Helder; Perard, Laurent; Le Guenno, Guillaume; Berthier, Sabine; Tschirret, Olivier; Eschard, Jean Paul; Vinzio, Stephane; Guillevin, Loïc; Sève, Pascal

    2015-01-01

    Abstract Pituitary dysfunction is a rare manifestation of granulomatosis with polyangiitis (GPA) (Wegener). The main aim of this multicenter retrospective study was to describe the characteristics and outcomes of pituitary manifestations in patients with GPA included in the French Vasculitis Study Group database. Among the 819 GPA patients included in the database, 9 (1.1%) had pituitary involvement. The median age at diagnosis of GPA and pituitary involvement was 46 and 50.8 years, respectively. Pituitary involvement was present at onset of GPA in 1 case and occurred later in 8 patients after a median follow up of 58.5 months. When pituitary dysfunction occurred, 8 patients had active disease at other sites including ENT (n = 6), eye (n = 4), or central nervous system (n = 3) involvement. The most common hormonal dysfunctions were diabetes insipidus (n = 7) and hypogonadism (n = 7). Magnetic resonance imaging was abnormal in 7 patients. The most common lesions were an enlargement of the pituitary gland, thickening of the pituitary stalk, and loss of posterior hypersignal on T1-weighed images. All patients were treated with corticosteroid therapy and 8 patients received immunosuppressive agents for the pituitary involvement, including cyclophosphamide (n = 3), rituximab (n = 2), and methotrexate (n = 3). After a median follow-up of 9.2 years, GPA was in complete remission in 7 patients, but 8 patients were still under hormone replacement therapy. Among the 5 patients who had a subsequent MRI, 2 had complete resolution of pituitary lesions.By combining our study and the literature review, the frequency of hypogonadism and diabetes insipidus, among the patients with pituitary dysfunction, can be estimated at 78% and 71% respectively. Despite a high rate of systemic disease remission on maintenance therapy, 86% of the patients had persistent pituitary dysfunction. The patients who recovered from pituitary dysfunction had all been

  10. Pulmonary lymphomatoid granulomatosis in a 4-year-old girl

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Subin; Kang, Mi-Jin; Lee, Jihae [Inje University Sanggye Paik Hospital, Department of Radiology, 1342, Dongilro, Nowon-gu, Seoul (Korea, Republic of); Kim, Hyun-Jung [Inje University Sanggye Paik Hospital, Department of Pathology, 1342, Dongilro, Nowon-gu, Seoul (Korea, Republic of); Kim, Hyo-Bin [Inje University Sanggye Paik Hospital, Department of Pediatrics, 1342, Dongilro, Nowon-gu, Seoul (Korea, Republic of)

    2015-07-15

    Lymphomatoid granulomatosis is a rare lymphoproliferative disease associated with the Epstein-Barr virus that commonly affects the lung. There is limited literature on cases of pediatric lymphomatoid granulomatosis. Half of all cases of lymphomatoid granulomatosis develop during the treatment of leukemia. Herein, we describe a case of lymphomatoid granulomatosis in a previously healthy child without leukemia. (orig.)

  11. A Case of Granulomatosis with Polyangiitis Causing Hydroureter and Hydronephrosis

    Directory of Open Access Journals (Sweden)

    Farzin Farpour

    2014-01-01

    Full Text Available Granulomatosis with Polyangiitis (GPA, formerly known as Wegener is a systemic vasculitis characterized by granulomatous involving upper and lower respiratory tract and can also cause necrotizing glomerulonephritis (Umemoto et al. 2012 and Takala et al. 2011. GPA is associated with antineutrophil cytoplasmic autoantibodies (ANCA against serine proteinase 3 (PR3 (Takala et al. 2011, Dufour et al. 2012, and Berthoux et al. 2011. This disease usually starts with involvement of the upper and lower respiratory tracts and also can involve kidney, eyes, skin, central and peripheral nervous systems, and gastrointestinal tract (Umemoto et al. 2012, Takala et al. 2011, and Berthoux et al. 2011. We describe a case of GPA that presented with abdominal pain. Computed tomography (CT scan with contrast showed right sided moderate hydronephrosis and hydroureter, to the level of the right common iliac artery. There was also mural segmental thickening in common iliac artery which was thought to be the cause of the ureteral obstruction and hydronephrosis. Our case shows that mural segmental thickening in common iliac artery happened due to GPA and caused hydronephrosis. In addition, most of the cases with hydronephrosis due to GPA went through urology intervention such as stent placement but in our case hydronephrosis resolved with medical management.

  12. A Case of Granulomatosis with Polyangiitis Causing Hydroureter and Hydronephrosis

    Science.gov (United States)

    Farpour, Farzin; Abrudescu, Adriana

    2014-01-01

    Granulomatosis with Polyangiitis (GPA, formerly known as Wegener) is a systemic vasculitis characterized by granulomatous involving upper and lower respiratory tract and can also cause necrotizing glomerulonephritis (Umemoto et al. 2012 and Takala et al. 2011). GPA is associated with antineutrophil cytoplasmic autoantibodies (ANCA) against serine proteinase 3 (PR3) (Takala et al. 2011, Dufour et al. 2012, and Berthoux et al. 2011). This disease usually starts with involvement of the upper and lower respiratory tracts and also can involve kidney, eyes, skin, central and peripheral nervous systems, and gastrointestinal tract (Umemoto et al. 2012, Takala et al. 2011, and Berthoux et al. 2011). We describe a case of GPA that presented with abdominal pain. Computed tomography (CT) scan with contrast showed right sided moderate hydronephrosis and hydroureter, to the level of the right common iliac artery. There was also mural segmental thickening in common iliac artery which was thought to be the cause of the ureteral obstruction and hydronephrosis. Our case shows that mural segmental thickening in common iliac artery happened due to GPA and caused hydronephrosis. In addition, most of the cases with hydronephrosis due to GPA went through urology intervention such as stent placement but in our case hydronephrosis resolved with medical management. PMID:24551472

  13. Ocular manifestation of granulomatosis with polyangitis

    Directory of Open Access Journals (Sweden)

    Mary Grace

    2015-01-01

    Full Text Available Ocular manifestation of granulomatosis with polyangitis. Proptosis is the most common ocular manifestation of granulomatosis with polyangitis. It is due to inflammation of the extraocular muscles or vasculitis of the vas nervosum. It is a poor prognostic sign.

  14. Wegener’s granulomatosis and pyoderma gangrenosum – rare causes of facial ulcerations

    Directory of Open Access Journals (Sweden)

    Karolina Kędzierska

    2016-03-01

    Full Text Available Background: Pyoderma gangrenosum (PG is caused by immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all PG patients also suffer from autoimmunological diseases, one of which is Wegener granulomatosis (WG. The purpose of this article was to compare cases of patients with WG and PG in terms of their clinical course, histopathology, and applied treatment. In both, histopathological features are not fully distinct. Data from microbiological and immunological evaluation and clinical presentation are required to establish the diagnosis. We also present the case of a patient with WG and deep facial skin lesions not responding to standard treatment. Methods: Systematic review of the literature in PubMed using the search terms “Wegener granulomatosis AND Pyoderma gangrenosum” and case report. Results: The finding of 22 reports in the literature (PubMed suggests that it is a rare phenomenon. This study revealed a similar rate of comorbidity of WG and PG in both genders and an increased incidence of both diseases after the age of 50. Among skin lesions there was a dominance of ulceration, most often deep and painful, covering a large area with the presence of advanced necrosis and destruction of the surrounding tissue. The most common location proved to be the cervical-cephalic area. The most popular treatment included steroids with cyclophosphamide. Discussion: The rarity of the coexistence of these two diseases results in a lack of effective therapy. In such cases sulfone derivatives are still effective and provide an alternative to standard immunosuppression methods. Hyperbaric therapy and plasmapheresis can also play an important complementary role.

  15. Wegener’s granulomatosis and pyoderma gangrenosum – rare causes of facial ulcerations

    Directory of Open Access Journals (Sweden)

    Karolina Kędzierska

    2016-03-01

    Full Text Available Background: Pyoderma gangrenosum (PG is caused by immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all PG patients also suffer from autoimmunological diseases, one of which is Wegener granulomatosis (WG. The purpose of this article was to compare cases of patients with WG and PG in terms of their clinical course, histopathology, and applied treatment. In both, histopathological features are not fully distinct. Data from microbiological and immunological evaluation and clinical presentation are required to establish the diagnosis. We also present the case of a patient with WG and deep facial skin lesions not responding to standard treatment.Methods: Systematic review of the literature in PubMed using the search terms “Wegener granulomatosis AND Pyoderma gangrenosum” and case report.Results: The finding of 22 reports in the literature (PubMed suggests that it is a rare phenomenon. This study revealed a similar rate of comorbidity of WG and PG in both genders and an increased incidence of both diseases after the age of 50. Among skin lesions there was a dominance of ulceration, most often deep and painful, covering a large area with the presence of advanced necrosis and destruction of the surrounding tissue. The most common location proved to be the cervical-cephalic area. The most popular treatment included steroids with cyclophosphamide.Discussion: The rarity of the coexistence of these two diseases results in a lack of effective therapy. In such cases sulfone derivatives are still effective and provide an alternative to standard immunosuppression methods. Hyperbaric therapy and plasmapheresis can also play an important complementary role.

  16. The Many Faces of Granulomatosis With Polyangiitis: A Review of the Head and Neck Imaging Manifestations.

    Science.gov (United States)

    Pakalniskis, Marius G; Berg, Aaron D; Policeni, Bruno A; Gentry, Lindell R; Sato, Yutaka; Moritani, Toshio; Smoker, Wendy R K

    2015-12-01

    Wegener granulomatosis has recently been renamed as granulomatosis with polyangiitis (GPA). In this review, we examine the clinical criteria and pathologic and pathophysiologic mechanisms of GPA, with an emphasis on findings encountered in the realm of head and neck imaging. Particular attention is paid to generating an appropriate differential diagnosis, because many of the imaging features of GPA overlap with those of other diseases, most notably lymphoma and sarcoidosis. Recent therapeutic advancements have underscored the importance of the radiologist in suggesting the diagnosis early, resulting in earlier treatment and decreased patient morbidity. This is particularly true for the head and neck manifestations of GPA; although they are less common, they often herald a refractory disease course that requires aggressive immunosuppressive therapy. Knowledge of common and uncommon imaging findings enables the radiologist to diagnose GPA early enough to start treatment promptly and reduce patient morbidity. Although there are no reliable pathognomonic imaging features for GPA, the present article attempts to identify patterns of disease that are suggestive of the disease. The diagnosis ultimately relies on a constellation of radiographic findings, laboratory values, and accurate clinical history.

  17. Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis

    Directory of Open Access Journals (Sweden)

    Basheer Tashtoush

    2014-01-01

    Full Text Available Granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis (WG, is a rare systemic vasculitis that classically manifests as necrotizing granulomas of the upper and lower respiratory tract, kidneys, and blood vessels; however, it may affect any organ system, including the skin. Cutaneous manifestations occur in up to 45% of patients during the disease course, and are the presenting feature in 9% to 14% of patients. The most common skin lesion specific to GPA is palpable purpura, with the histopathologic correlate of leukocytoclastic vasculitis. However, a wide range of clinical and histologic features may be seen. We herein report a case of a previously healthy 52-year-old Caucasian man who presented with multiple progressively enlarging painful ulcers on his face, upper extremities, back, and abdomen over a two-month period. Skin biopsies revealed pyoderma gangrenosum-like features. Serological tests were positive for PR3/c-ANCA. Six months later, the patient developed recurrent episodes of sinusitis associated with nasal bleeds and eventually nasal septum perforation. Despite aggressive treatment with Cyclophosphamide and steroids over one year, the patient had persistent nonhealing large ulcers and developed multiple lung nodules with cavitary lesions.

  18. OCCURRENCE OF AUTOANTIBODIES TO HUMAN-LEUKOCYTE ELASTASE IN WEGENERS GRANULOMATOSIS AND OTHER INFLAMMATORY DISORDERS

    NARCIS (Netherlands)

    TERVAERT, JWC; MULDER, L; STEGEMAN, C; ELEMA, J; THE, MHH; KALLENBERG, C

    Antineutrophil cytoplasmic antibodies (ANCAs) constitute a new class of autoantibodies that seem to recognise myeloid lysosomal enzymes. The occurrence of ANCAs with specificity for human leucocyte elastase (HLE) was assessed in serum samples that were routinely submitted for ANCA determination.

  19. Granulomatous polyangitis (Wegener granulomatosis): Clinical findings and results of long-term follow-up.

    Science.gov (United States)

    Şen, Nazan; Aydın Tufan, Müge; Yıldız, Reyhan; Ersözlü Bozkırlı, Emine Duygu; Yücel, Eftal

    2016-09-01

    To evaluate long-term outcome of patients with granulomatous polyangitis (GPA) followed up in a tertiary university hospital. We reviewed medical records of 22 patients with GPA diagnosis confirmed by tissue biopsies between 2004 and 2014. The mean time from the onset of symptoms to diagnosis was 7.8 ± 12.3 months [interquartile range (IR)= 4.0]. The most commonly involved organs were the upper respiratory tract (URT) (72.7%), lower respiratory tract (81.8%) and kidneys (72.7%). URT involvement indicated good prognosis (p= 0.046). Survival in the patients with and without URT involvement was 124.6 ± 6.9 months and 59.7 ± 22.9 months, respectively. End-stage renal failure (ESRF) requiring dilaysis and cardiac involvement were associated with mortality (p= 0.022 and p= 0.026, respectively). Of the 12 dialysis-dependent patients at diagnosis, 11 survived > 3 months and seven regained renal function permanently. Dialysis dependency was significantly lower in patients who received plasmapheresis (p= 0.047). Overall mortality rate was 18% (4/22). Mean survival was 55.9 ± 42.8 months (IR= 84.0). Diagnosis of GPA may be delayed by the nonspecific nature of its symptoms. URT involvement was associated with good prognosis, whereas cardiac involvement and ESRF requiring dialysis were associated with poor outcome. Plasmapheresis may increase the rate of renal recovery in the patients with ESRF requiring dialysis.

  20. Idiopathic bronchocentric granulomatosis in an asthmatic adolescent

    Directory of Open Access Journals (Sweden)

    Hiroki Umezawa

    2015-01-01

    Full Text Available Bronchocentric granulomatosis in asthmatic patients has been generally considered to be associated with allergic bronchopulmonary aspergillosis and represent a histopathologic manifestation of fungal hypersensitivity. Here we report a case of an idiopathic bronchocentric granulomatosis in a 17-year-old man with a history of asthma. He was admitted to the hospital with a fever and cough, and a chest CT scan showed peribronchial consolidation in the pulmonary parenchyma, which was unresponsive to antibiotic therapy. The pathological findings obtained by video-assisted thoracoscopic lung biopsy revealed necrotizing granulomatous inflammation centered on bronchi and bronchioles and there was no evidence of fungal colonization, resulting in a diagnosis of idiopathic bronchocentric granulomatosis. Systemic corticosteroid therapy led to clinical and radiological recovery. Physicians should take into account the possibility of the idiopathic process in bronchocentric granulomatosis of asthmatic patients.

  1. Wegener and his Theory of Continental Drift

    Indian Academy of Sciences (India)

    similarities among fauna of India, Australia and Madagascar. ... Madagascar, India, Antarctica and Australia, separated from the western part consisting of Africa and ... islands. Wegener found that longitude measurements on Sabine. Island near Greenland between 1823 and 1907 indicated apparent cumulative westward ...

  2. Granulomatosis with polyangiitis in Tunisia

    Directory of Open Access Journals (Sweden)

    I. Ben Ghorbel

    2017-05-01

    Full Text Available Granulomatosis with polyangiitis (GPA is more frequent in Northern rather than Southern countries. Very few studies have been conducted in Africa. We have performed a retrospective descriptive study including clinical and laboratory profiles of 30 Tunisian GPA patients seen at the department of Internal Medicine of the University Hospital of la Rabta from 2000 to 2014. Mean age at initial GPA diagnosis was 46±12 years, and the average number of months between the onset of symptoms and diagnosis was 25. Seventeen (56% were male, and 13 (44% were female. Ear/nose/throat involvement occurred in 83%. Lung and renal involvement were observed in respectively 70% and 56% followed by mucocutaneous (50%, neurological (50%, ocular (33%, vascular (20%, ureteral (16%, and cardiac involvement in 10%. Cytoplasmic pattern-antineutrophil cytoplasmic antibodies (ANCA was detected in 27 (90% patients. Induction therapy consisted of intravenous cyclophosphamide pulses in 27 patients (90% and oral methotrexate in 3 patients (10%. Trimethoprime-sulfamethoxazole was used in 26 patients (86%. Maintenance therapy consisted of azathioprine in 17 cases and methotrexate in 13 cases. Relapses occurred in 36%. Eighteen patients had favorable outcome and 12 died. Our patients had a distinct phenotype with high prevalence of pleural involvement, lymph node enlargement, sensorimotor neuropathy and ureter stenosis. ENT symptoms were less frequent as inaugural presentation. Overall 2-year survival was 60%.

  3. Mast Cell Quantification in Orofacial Granulomatosis | Nwizu ...

    African Journals Online (AJOL)

    The study points to a possible association between mast cells and orofacial granulomatosis, as oedematous area usually associated with OFG showed less numbers of mast cells. This is probably due to degranulation, releasing mediators of inflammation, which is responsible for oedema formation. Further light needs to be ...

  4. Characteristics of patients with orofacial granulomatosis.

    LENUS (Irish Health Repository)

    McCartan, B E

    2011-10-01

    Orofacial granulomatosis has mostly been described in reports of very small numbers of cases. Few large case groups have been described. The aim of this study was to describe the demographics, symptoms, clinical features and laboratory findings in a large cohort of cases.

  5. Autoantibodies

    Science.gov (United States)

    ... Mosby, Inc., Saint Louis, MO. What is Wegener's Granulomatosis. Wegener's Granulomatosis Association [On-line information]. Available online at ... 20). Anti-Neutrophilic Cytoplasmic Antibodies (ANCA) in Wegener's ... Granulomatosis Association [On-line information]. Available online at ...

  6. Saddle nose deformity and septal perforation in granulomatosis with polyangiitis.

    Science.gov (United States)

    Coordes, A; Loose, S M; Hofmann, V M; Hamilton, G S; Riedel, F; Menger, D J; Albers, A E

    2018-02-01

    Patients who have granulomatosis with polyangiitis (GPA, syn. M. Wegener) often develop an external nose deformity which may have devastating psychological effects. Therefore, reconstruction of nasal deformities by rhinoplasty may become necessary to achieve a normal appearance. The aim of this systematic review was to investigate the efficacy and safety of surgical reconstruction in external nasal deformities and septal perforation in GPA patients. A systematic literature search with defined search terms was performed for scientific articles archived in the MEDLINE-Database up to 10 June 2016 (PubMed Advanced MEDLINE Search), describing management of cases or case series in GPA patients with saddle nose deformity and/or septal perforation. Eleven of 614 publications met the criteria for this analysis including 41 GPA patients undergoing external nasal reconstruction and/or septal reconstruction with a median follow-up of 2.6 years. Overall, saddle nose reconstruction in GPA patients is safe even if an increased rate of revision surgery has to be expected compared with individuals without GPA undergoing septorhinoplasty. Most implanted grafts were autografts of calvarial bone or costal cartilage. For septal perforation reconstruction, few studies were available. Therefore, based on the available data for surgical outcomes, it is impossible to make evidence-based recommendations. All included GPA patients had minimal or no local disease at the time of reconstructive surgery. Therefore, the relationship between disease activity and its impact on surgical outcomes remains unanswered. The potential impact of immune-modulating medications on increased complication rates and the impact of prophylactic antibiotics are unknown. This study systematically reviews the efficacy and safety of surgical reconstruction of external nasal deformities in GPA patients for the first time. Saddle nose reconstruction in GPA patients with minimal or no local disease is a safe procedure

  7. A case of aortic and mitral valve involvement in granulomatosis with polyangiitis.

    Science.gov (United States)

    Espitia, Olivier; Droy, Laure; Pattier, Sabine; Naudin, Frédérique; Mugniot, Antoine; Cavailles, Arnaud; Hamidou, Mohamed; Bruneval, Patrick; Agard, Christian; Toquet, Claire

    2014-01-01

    Granulomatosis with polyangiitis (GPA) (Wegener's) is a necrotizing systemic vasculitis of the small-sized blood vessels, affecting kidneys, lungs, upper respiratory tract and skin. Cardiac valvular involvement is an uncommon manifestation of GPA. We report the case of a 60-year-old woman with arthritis and lung nodules due to GPA without antineutrophil cytoplasmic antibodies (ANCA) at time of diagnosis. Remission was obtained with cyclophosphamide and corticosteroid. Azathioprine was then prescribed for 2years. Four years later, she developed severe inflammatory aortic and mitral valvular involvement characterized by GPA typical histopathological valvular lesions. Search for ANCA was positive at this time (anti-myeloperoxidase). Cardiac valvular involvement is a rare and potentially fatal complication of GPA and may misleadingly suggest infectious endocarditis. A review of literature revealed few cases of histologically well-documented cardiac valvular involvement in GPA. Pathologists should be aware of valvular heart diseases in GPA, which usually comprise valvular necrotic lesions without any microbial agents. Copyright © 2014 Elsevier Inc. All rights reserved.

  8. Orofacial granulomatosis: clinical signs of different pathologies.

    Science.gov (United States)

    Troiano, Giuseppe; Dioguardi, Mario; Giannatempo, Giovanni; Laino, Luigi; Testa, Nunzio Francesco; Cocchi, Roberto; De Lillo, Alfredo; Lo Muzio, Lorenzo

    2015-01-01

    Orofacial granulomatosis (OFG) is an uncommon disease characterized by persistent or recurrent soft tissue enlargement, oral ulceration and a variety of other orofacial features. It could be an oral manifestation of a systemic disease. For a correct differential diagnosis, local and systemic conditions characterized by granulomatous inflammation should be excluded using appropriate clinical and laboratory investigations. In fact, the diagnosis of OFG may be confirmed only by histopathological identification of noncaseating granulomas. The literature from 1943 to 2014 was reviewed with emphasis on the etiology of OFG and on clinical manifestations of systemic pathologies associated with OFG. The precise cause of OFG is still unknown, although several theories have been suggested, such as infection, hereditary factors and allergy. OFG is a disease that has a wide spectrum of presentation, which may include the oral manifestation of a systemic condition such as Crohn's disease, sarcoidosis, granulomatosis with polyangiitis and Melkersson-Rosenthal syndrome. © 2015 S. Karger AG, Basel.

  9. Ervaringen met de resectie-therapie wegens longtuburculose

    NARCIS (Netherlands)

    Dijk, Berto van

    1953-01-01

    In dit onderzoek worden de resultaten besproken, die met de resectietherapie wegens longtuberculose zijn verkregen. Het materiaal omvat 500 resecties, die tussen 20 Mei 7943 en 20 Maart 1952 zijn verricht. ... Zie: Samenvatting.

  10. Pediatric Vasculitis Initiative

    Science.gov (United States)

    2017-05-01

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

  11. Greenland Expeditions by Alfred Wegener - A photographic window to past

    Science.gov (United States)

    Leitner, M.; Tschürtz, S.; Kirchengast, G.; Kranzelbinder, H.; Prügger, B.; Krause, R. A.; Kalliokoski, M.; Thórhallsdóttir, E.

    2012-04-01

    On several expeditions to Greenland, Alfred Wegener (1880-1930) took pictures on glass plates from landscapes and glaciers, the expedition equipment, the people and animals taking part on the expeditions as well as physical phenomena as dust storm, clouds or spherical light phenomena. Chronologically the plates show the Danmark Expedition 1906-1908, the crossing of Greenland expedition with stop in Iceland 1912-1913, and the German Greenland Expedition 1929-1930. Until the tragic end of the expedition in 1930, Wegener was professor at the University of Graz, and such a stock of about 300 glass plates stayed there. The aim of our work is to digitize all plates for further studies. We present a first selection of Wegener's Greenland expedition pictures. For those made at Iceland in 1912 we will present a comparison of the past with pictures from the same viewing point made in 2011.

  12. Allosteric modulation of proteinase 3 activity by anti-neutrophil cytoplasmic antibodies in granulomatosis with polyangiitis.

    Science.gov (United States)

    Hinkofer, Lisa C; Hummel, Amber M; Stone, John H; Hoffman, Gary S; Merkel, Peter A; Spiera, E Robert F; St Clair, William; McCune, Joseph W; Davis, John C; Specks, Ulrich; Jenne, Dieter E

    2015-05-01

    Anti-neutrophil cytoplasmic antibodies (ANCA) with proteinase 3 (PR3) specificity are a useful laboratory biomarker for the diagnosis of Granulomatosis with Polyangiitis (GPA) and are believed to be implicated in the pathogenesis. It has been repeatedly suggested that disease activity of GPA is more closely related to the appearance and rise of PR3-inhibiting ANCA than to an increase of total ANCA. Previous studies on a limited number of patient samples, however, have yielded inconclusive results. To overcome the previous methodological limitations, we established a new ultrasensitive method to quantify the inhibitory capacity of PR3-ANCA using small volumes of plasma from patients with GPA. A large collection of longitudinally-collected samples from the Wegener Granulomatosis Etanercept Trial (WGET) became available to us to determine the functional effects of ANCA on PR3 in comparison to clinical disease manifestations. In these patient samples we not only detected PR3-ANCA with inhibitory capacity, but also PR3-ANCA with enhancing effects on PR3 activity. However no correlation of these activity-modulating PR3-ANCA with disease activity at either the time of enrollment or over the course of disease was found. Only patients with pulmonary involvement, especially patients with nodule formation in the respiratory tract, showed a slight, but not significant, decrease of inhibitory capacity. Epitope mapping of the activity-modulating PR3-ANCA revealed a binding on the active site surface of PR3. Yet these ANCA were able to bind to PR3 with an occupied active site cleft, indicating an allosteric mechanism of inhibition. The recently described signal ratio between the MCPR3-3 and MCPR3-2 capture ELISA was consistent with the binding of activity-modulating ANCA to the active site surface. Evidence for a shared epitope between activity-modulating PR3-ANCA and MCPR3-7, however, was very limited, suggesting that a majority of PR3-ANCA species do not inhibit PR3 by the same

  13. Cytomegalovirus retinitis treated with valganciclovir in Wegener’s granulomatosis

    Directory of Open Access Journals (Sweden)

    Kabata Y

    2012-03-01

    Full Text Available Yoshiaki Kabata1, Genichiro Takahashi1, Hiroshi Tsuneoka21Department of Ophthalmology, Jikei University School of Medicine, Katsushika Medical Center, Katsushika, Tokyo, Japan; 2Department of Ophthalmology, Jikei University School of Medicine, Minato, Tokyo, JapanAbstract: A case of cytomegalovirus (CMV retinitis in a patient with Wegener’s granulomatosis treated with oral valganciclovir as maintenance therapy is reported. A 68-year-old male patient with anti-proteinase-3 ANCA-positive Wegener’s granulomatosis who was receiving immunosuppressive therapy with methylprednisolone, cyclophosphamide, and azathioprine developed CMV retinitis. The patient received intravenous ganciclovir as induction therapy and oral valganciclovir as maintenance therapy. The patient responded to treatment and showed no recurrence for 8 months. There were no serious adverse effects associated with oral valganciclovir. Oral valganciclovir is convenient and effective for the management of CMV retinitis in the patient with Wegener’s granulomatosis.Keywords: cytomegalovirus retinitis, Wegener’s granulomatosis, valganciclovir, ganciclovir, maintenance therapy

  14. Current and future prospects in the management of granulomatosis with polyangiitis (Wegener’s granulomatosis)

    Science.gov (United States)

    Tarzi, Ruth M; Pusey, Charles D

    2014-01-01

    Granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis) is a multisystem autoimmune condition associated with anti-neutrophil cytoplasm antibodies. Management of GPA can be complex, owing to the sometimes fulminant and multisystem nature of the presentation, the age demographics of the affected population, and a significant incidence of disease relapse. In this paper, we discuss how some of the challenges in the management of GPA have been and continue to be addressed including: reducing the toxicity of induction therapy; developing biomarkers to determine who can safely stop maintenance immunosuppression; improving the efficacy of maintenance therapy for relapsing patients; managing localized disease; and management of disease and treatment-associated comorbidity. Consideration is also given to emerging therapeutics in the treatment of GPA. PMID:24790453

  15. Clinic manifestations in granulomatosis with polyangiitis

    Science.gov (United States)

    Greco, A; Marinelli, C; Fusconi, M; Macri, GF; Gallo, A; De Virgilio, A; Zambetti, G; de Vincentiis, M

    2015-01-01

    Granulomatosis with polyangiitis (GPA), formerly Wegener’s granulomatosis (WG), is an uncommon immunologically mediated systemic small-vessel vasculitis that is pathologically characterised by an inflammatory reaction pattern (necrosis, granulomatous inflammation and vasculitis) that occurs in the upper and lower respiratory tracts and kidneys. Although the aetiology of GPA remains largely unknown, it is believed to be autoimmune in origin and triggered by environmental events on a background of genetic susceptibility. In Europe, the prevalence of GPA is five cases per 100,000 population, with greater incidence in Northern Europe. GPA can occur in all racial groups but predominantly affects Caucasians. Both sexes are affected equally. GPA affects a wide age range (age range, 8–99 years). Granulomatosis with polyangiitis is characterised by necrotising granulomatous lesions of the respiratory tract, vasculitis and glomerulonephritis. Classically, the acronym ELK is used to describe the clinical involvement of the ear, nose and throat (ENT); lungs; and kidneys. Because the upper respiratory tract is involved in 70–100% of cases of GPA, classic otorhinolaryngologic symptoms may be the first clinical manifestation of disease. The nasal cavity and the paranasal sinuses are the most common sites of involvement in the head and neck area (85–100%), whereas otological disease is found in approximately 35% (range, 19–61%) of cases. Diagnosis of GPA is achieved through clinical assessment, serological tests for anti-neutrophil cytoplasmic antibodies (ANCA) and histological analysis. The 10-year survival rate is estimated to be 40% when the kidneys are involved and 60–70% when there is no kidney involvement. The standard therapy for GPA is a combination of glucocorticoids and cyclophosphamide. In young patients, cyclophosphamide should be switched to azathioprine in the maintenance phase. A multidisciplinary approach, involving otorhinolaryngologists, oral and

  16. 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

  17. Necrobiotic xanthogranuloma scleritis in a case of granulomatosis with polyangiitis (Wegener’s granulomatosis

    Directory of Open Access Journals (Sweden)

    Keorochana N

    2017-10-01

    Full Text Available Narumon Keorochana,1 Kanaspana Klanarongran,2 Kantang Satayasoontorn,3 Sumapa Chaiamnuay4 1Department of Ophthalmology, Ocular Inflammatory and Uveitis Division, 2Department of Ophthalmology, External Disease and Cornea Division, 3Department of Anatomic Pathology, 4Department of Medicine, Rheumatology Division, Phramongkutklao Hospital, Phramongkutklao College of Medicine, Bangkok, Thailand Abstract: The purpose of this study was to describe a case of necrobiotic xanthogranuloma scleritis in a 53-year-old male with unilateral progressive visual loss, scleritis, prolonged fever, and multiple mononeuropathy. Scleral biopsy showed necrosis with small abscess, and the pathological tissues revealed submucosal infiltration of mononucleated foamy histiocytes (xanthoma cells, hemosiderin-laden macrophages, neutrophils, lymphocytes, plasma cells, and erythrocytes without Touton giant cells or cholesterol clefts. Serum protein electrophoresis showed polyclonal gammopathy. All infectious investigations were negative. Afterward, this patient was diagnosed with granulomatosis with polyangiitis based on granuloma found in scleral tissue, vasculitis seen in sural nerve biopsy and positive serologies (C-ANCA and anti-PR3 antibody. He was treated with high-dose corticosteroid and later with intravenous cyclophosphamide monthly. He responded well to treatment, both eye and systemic conditions. Necrobiotic xanthogranuloma scleritis could be an early presentation of granulomatosis with polyangiitis. Keywords: scleritis, uveitis, ocular inflammation, pathology, autoimmune, xanthoma cells, GCA

  18. Multifocal Langerhans Cell Granulomatosis (Hand-schuller - Christian Disease

    Directory of Open Access Journals (Sweden)

    MPS Sawhney

    1989-01-01

    Full Text Available A 47 year old female developed multifocal Langerhans cell granulomatosis (Hand-Schuller-Christian disease manifesting as papulo-pustular, nodular, crusted and scaly lesions, xanthelasma, ulceration of gingival mucosa with loss of teeth, vaginal granulomatosis, diabetes insipidus, multiple osteolytic bone lesions and honeycombing of the lungs. Skin biopsy confirmed the diagnosis. Treatment with prednisolone 45 mg methotrexate 2.5 mg daily, led to regression of lesions, reduction of bone of partial clearance of lung lesions and osteolytic bone lesions. However, the patient died due to widespread nature of the disease.

  19. Resolution of orofacial granulomatosis with amalgam removal.

    Science.gov (United States)

    Guttman-Yassky, E; Weltfriend, S; Bergman, R

    2003-05-01

    A 61-year-old woman presented with a 2-year history of an abnormal erythematous swelling on the upper lip and cheek. Upon examination there were no other physical findings. Histological examination found discreet sarcoidal granulomas in the lower dermis. Routine laboratory studies, chest radiographs and pulmonary functions were all normal. Clinical presentation and histological findings were, therefore, compatible with the diagnosis of orofacial granulomatosis (OFG). The patient was patch tested with an extended standard series that included metal-salt, dental prosthesis, bakery and corticosteroids series. The patch test was positive (score ++) after 48 and 72 h for mercury in the metal-salt and dental prosthesis series. During the past decade the patient had received amalgam fillings of several dental cavities, including one adjacent to the swollen cheek. The unilateral localization of the soft tissue swelling adjacent to the amalgam tooth fillings, along with the positive patch test for mercury, raised the possibility that the OFG was part of a delayed hypersensitive reaction to the fillings. The patient therefore underwent a total amalgam replacement procedure; complete disappearance of the swelling overlying the right cheek was observed within 7 weeks and the swelling of the upper lip subsided completely within 6 months. We propose that mercury in amalgam tooth fillings is another cause of OFG and suggest appropriate patch testing in patients who do not have an apparent cause of OFG.

  20. Patch testing for food-associated allergies in orofacial granulomatosis.

    LENUS (Irish Health Repository)

    Fitzpatrick, Laura

    2011-01-01

    Food-associated allergies, especially to benzoates and cinnamon-related compounds, have been associated with orofacial granulomatosis and both standard and urticarial patch testing have been used to detect such allergies. Elimination diets have also been shown to be effective in some patients.

  1. Lymphomatoid and Mediastinal Granulomatosis in 2-Year-Old Child

    Directory of Open Access Journals (Sweden)

    S.V. Vesely

    2014-02-01

    24.07.13 the operation was performed: right thoracotomy, removal of the tumor of the upper lobe of right lung. Immunohistochemical examination results were received: lymphomatoid granulomatosis, grade II. The postoperative period was uneventful. Sutures are removed, the wound healed by primary intention. 10.08.13 the child was discharged home. Further treatment in Minsk (Belarus is planned.

  2. "Wegener’s granulomatosis in a patient with Rheumatoid arthritis "

    Directory of Open Access Journals (Sweden)

    "Ahmadi Nejad Z

    2001-06-01

    Full Text Available Wegener’s Granulomatosis is a necrotizing granulomatous vasculitis involving small and medium sized vessels. The syndrome is classically defined as having involvement of kidney, lungs and upper respiratory tract (i.e. sinuses. Wegener’s Granulomatosis may be present in other autoimmune or inflammatory diseases, particulary systemic lupus erythematosis (SLE, but most frequently has been associated with polyarteritis and glumerulonephritis.We present a case of wegener’s Granulomatosis (WG in a middle age lady with Rheumatiod Arthritis (RA; and discuss the implications of these two conditions co-existing in one patient. As for as we are aware, through medline and interent research, this is probably the fourth case with such as association of WG and RA and the fist one in Iran.Presentation of new uncontrollable sing and symptoms, in a previously well controlled RA patient, might suggest a new overlapping syndrome like Wegener’s Granulomatosis besides to flare up of previous disease as differential diagnosis

  3. Gastric presentation (vasculitis) mimics a gastric cancer as initial symptom in granulomatosis with polyangiitis: a case report and review of the literature.

    Science.gov (United States)

    Zheng, Zhaohui; Ding, Jin; Li, Xueyi; Wu, Zhenbiao

    2015-11-01

    Granulomatosis with polyangiitis (GPA), formerly called Wegener's Granulomatosis, is characterized by necrotizing granulomatous inflammation and belongs to the family of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. The main clinical symptoms of GPA are vasculitis primarily involving upper and lower respiratory tracts, as well as kidneys. Gastrointestinal manifestations of GPA are less common (0-20 %), with gastric presentation mimicking a gastric cancer as an initial symptom. This is a descriptive case report of one patient, together with systematic review of the literature. We described a 31-year-old Chinese woman who presented with complaints of abdominal distention, anorexia for 2 months. Gastroscopy was carried out for three times, and stomach cancer was suspected. However, histopathology of gastric biopsy revealed a chronic inflammation with mucosal ulceration, frequent neutrophils and lymphocytes infiltration, and local granulomatous formation, whereas no sign of stomach carcinoma was observed. In view of the positive cANCA test, a diagnosis of GPA was considered. From the onset of the GPA in the patients, no other organs have been involved in the disease. The patient was successfully treated with corticosteroids and cyclophosphamide. As shown in the report, patients who present only with gastrointestinal manifestations represent challenges to diagnosis. ANCA testing can serve as a decisive diagnostic tool. Although uncommon, GI involvement may be a major feature in GPA, sometimes presenting as gastric tumor-like lesions. Diagnosis should be considered in patients presenting with GI symptoms accompanied by evidence of systemic vasculitis, and ANCA test should be used as a diagnostic measurement to clarify differential diagnosis.

  4. Eosinophilic granulomatosis with polyangiitis: an overview

    Directory of Open Access Journals (Sweden)

    Andrea eGioffredi

    2014-11-01

    Full Text Available Eosinophilic granulomatosis with polyangiitis (EGPA is a multisystemic disorder, belonging to the small vessel ANCA-associated vasculitis, defined as a eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium-sized vessels, associated with asthma and eosinophilia. EGPA pathogenesis is not well known: HLA-DRB1*04 and *07, HLA-DRB4 and IL10.2 haplotype of the IL-10 promoter gene are the most studied genetic determinants. Among the acquired pathogenetic factors, the exposure to different allergens, infections, vaccinations, drugs and silica exposure have been involved.Eosinophils are the most characteristic cells in EGPA and different studies have demonstrated their role as effector and immunoregulatory cells.EGPA is considered a disease with a prevalent activation of the Th2 cellular-mediated inflammatory response but also humoral immunity plays an important role. A link between B and T inflammatory responses may explain different disease features. EGPA typically develops into three sequential phases: the allergic phase, distinguished by the occurrence of asthma, allergic rhinitis and sinusitis, the eosinophilic phase, in which the main pathological finding is the eosinophilic organ infiltrations (e.g. lungs, heart and gastrointestinal system and the vasculitic phase, characterized by purpura, peripheral neuropathy and constitutional symptoms.ANCA (especially pANCA anti-MPO are present in 40-60% of the patients. An elevation of IgG4 is frequently found. Corticosteroids and cyclophosphamide are classically used for remission induction, while azathioprine and methotrexate are the therapeutic options for remission maintenance. B-cell depletion with rituximab has shown promising results for remission induction.

  5. Pulmonary manifestations of granulomatosis with polyangiitis.

    Science.gov (United States)

    Gómez-Gómez, Alejandro; Martínez-Martínez, Marco Ulises; Cuevas-Orta, Enrique; Bernal-Blanco, Juan Mario; Cervantes-Ramírez, Daniel; Martínez-Martínez, Raúl; Abud-Mendoza, Carlos

    2014-01-01

    To describe the clinical and laboratory data, with special emphasis on thoracic imaging findings, in 14 patients with a definitive diagnosis of granulomatosis with polyangiitis (GPA). The clinical and tomographic data of 14 patients with a definitive diagnosis of GPA are presented. Patients with thoracic manifestations suggestive of GPA were evaluated in 3 hospitals from 2000 to 2012. All patients had a sputum analysis and bronchoalveolar lavage for bacterial, mycobacterial and fungal stains and cultures; antineutrophil cytoplasmic antibodies, antinuclear-antibodies, rheumatoid factor, and a biopsy of involved organs. A total of 13 patients had at least two organs involved. The most frequent thoracic findings were pulmonary nodules, ground glass opacities and patches of consolidation; other abnormalities were tracheal stenosis, diffuse alveolar hemorrhage, lung masses with organized pneumonia. More than three-quarters (78%) of patients had positive antineutrophil cytoplasmic antibodies (ANCA). Ten patients had respiratory tissue biopsy (8 open lung, one tracheal, and one nasal). In 4 patients the diagnosis was made with the classic organ involvement in GPA, positive ANCA, and renal or skin biopsy, and response to treatment on follow-up. At 6-12 months all patients showed clinical and radiological improvement, with 54% showing a recurrence of disease. The majority of thoracic findings described in GPA are presented in this study. A complete diagnostic approach with invasive diagnostic procedures to rule out other more prevalent respiratory diseases with similar thoracic manifestations must be performed. The positivity of ANCA in this study was high, and the recurrence of the disease was frequent. Copyright © 2013 Elsevier España, S.L.U. All rights reserved.

  6. Pituitary dysfunction in granulomatosis with polyangiitis.

    Science.gov (United States)

    Esposito, Daniela; Trimpou, Penelope; Giugliano, Dario; Dehlin, Mats; Ragnarsson, Oskar

    2017-10-01

    Granulomatosis with polyangiitis (GPA) is a multisystem disease, characterized by necrotizing small-vessel vasculitis, which mainly affects the respiratory tract and the kidneys. Pituitary involvement in GPA is rare, present in about 1% of all cases of GPA. To date, only case reports or small case series have been published. Herein we report clinical features, imaging findings, treatment and outcomes in three patients with GPA-related pituitary dysfunction (PD). A retrospective analysis of three cases of GPA-related PD was conducted, followed by systematic review of the English medical literature using PubMed. The three cases include three women aged between 32 and 37 years. PD was the presenting feature in one and two developed PD in the course of the disease. All patients had a pituitary lesion on MRI. Conventional treatment with high doses of glucocorticoids and cyclophosphamide led to resolution or improvement of the MRI abnormalities, whereas it was not effective in restoring PD. A systematic review identified 51 additional patients, showing that GPA can lead to partial or global PD, either at onset or, during the course of the disease. Secondary hypogonadism is the predominant manifestation, followed by diabetes insipidus (DI). Sellar mass with central cystic lesion is the most frequent radiological finding. GPA should be carefully considered in patients with a sellar mass and unusual clinical presentation with DI and systemic disease. Although conventional induction-remission treatment improves systemic symptoms and radiological pituitary abnormalities, hormonal deficiencies persist in most of the patients. Therefore, follow-up should include both imaging and pituitary function assessment.

  7. Clinical features of childhood granulomatosis with polyangiitis (wegener’s granulomatosis)

    Science.gov (United States)

    2014-01-01

    Background Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis (WG), belongs to the group of ANCA-associated necrotizing vasculitides. This study describes the clinical picture of the disease in a large cohort of GPA paediatric patients. Children with age at diagnosis ≤ 18 years, fulfilling the EULAR/PRINTO/PRES GPA/WG classification criteria were extracted from the PRINTO vasculitis database. The clinical signs/symptoms and laboratory features were analysed before or at the time of diagnosis and at least 3 months thereafter and compared with other paediatric and adult case series (>50 patients) derived from the literature. Findings The 56 children with GPA/WG were predominantly females (68%) and Caucasians (82%) with a median age at disease onset of 11.7 years, and a median delay in diagnosis of 4.2 months. The most frequent organ systems involved before/at the time of diagnosis were ears, nose, throat (91%), constitutional (malaise, fever, weight loss) (89%), respiratory (79%), mucosa and skin (64%), musculoskeletal (59%), and eye (35%), 67% were ANCA-PR3 positive, while haematuria/proteinuria was present in > 50% of the children. In adult series, the frequency of female involvement ranged from 29% to 50% with lower frequencies of constitutional (fever, weight loss), ears, nose, throat (oral/nasal ulceration, otitis/aural discharge), respiratory (tracheal/endobronchial stenosis/obstruction), laboratory involvement and higher frequency of conductive hearing loss than in this paediatric series. Conclusions Paediatric patients compared to adults with GPA/WG have similar pattern of clinical manifestations but different frequencies of organ involvement. PMID:24891844

  8. Multiple pulmonary nodules: a complex case of Wegener’s granulomatosis

    OpenAIRE

    Mariya Apostolova; Mahmoud Shoib; Samer Nasser

    2013-01-01

    Wegener’s granulomatosis is a granulomatous vasculitis that can present with a wide spectrum of clinical manifestations. This disease entity predominantly affects the respiratory tract and the kidneys. Two forms of Wegener’s granulomatosis have been recognized: systemic and limited. It has not been established if the two forms represent separate disease entities or different stages of the same condition. In the limited form of Wegener’s granulomatosis there is no immediate threat to the funct...

  9. Technologies in Computerized Lexicography

    African Journals Online (AJOL)

    scope of this paper (for a historical review of NLP, see Sparck Jones 1995). Here, it is relevant that the automatic analysis of language has become an inter- disciplinary topic of interest, and that some developments may have relevance to corpus analysis and computerized corpus-based lexicography. We particu- larly refer ...

  10. Computerized Mastery Testing.

    Science.gov (United States)

    Lewis, Charles; Sheehan, Kathleen

    1988-01-01

    Introduces a theoretical framework for mastery testing, using Item Response Theory and Bayesian Decision Theory. The idea of sequential testing is developed, with the goal of providing longer or shorter tests as needed, and a computerized application to a hypothetical professional knowledge examination is discussed. (Author/LRW)

  11. Wegener′s granulomatosis disease mimicking pulmonary tuberculosis

    Directory of Open Access Journals (Sweden)

    Naveen Pandhi

    2015-01-01

    Full Text Available Wegener′s granulomatosis (WG is an uncommon autoimmune disease with multi-system involvement that manifests as vasculitis, granulomatosis, and necrosis. While its standard form involves the upper and lower respiratory tracts and kidneys, it may essentially involve any organ. We present a case in a young female patient presenting with symptoms of cough with expectoration, fever, dyspnea and chest discomfort, having cavitating lesion in right upper lobe and nodule in left upper lobe with bilateral paranasal sinus involvement on computed tomography. Allergic rhino-sinusitis with pulmonary tuberculosis was suspected, and patient was investigated further. Upon further investigation, renal involvement was detected, and serology revealed cytoplasmic antineutrophil cytoplasmic antibody. WG was suspected and was proven by histopathology of nasal tissue which revealed necrotizing granulomas.

  12. A Rare Presentation of a Rare Disease: Pulmonary Lymphomatoid Granulomatosis

    Directory of Open Access Journals (Sweden)

    Ghulam Rehman Mohyuddin

    2012-01-01

    Full Text Available A 70-year-old female presented with a 4-week history of dry cough and wheezing. Chest radiograph showed a 10.5 cm mass-like density in the anterior mediastinum which had not been previously visualized. Computed tomography scan (CT of the chest showed a right hilar mass encasing and narrowing right upper lobe bronchus and right mainstem bronchus and secondary atelectatic changes. Biopsy was consistent with a diagnosis of lymphomatoid granulomatosis Grade 3. She responded well clinically and radiologically to therapy. Lymphomatoid granulomatosis is a rare EBV-associated disorder which is considered a lymphoproliferative disease. The most common radiographic feature is multiple lung nodules. An isolated hilar mass is an exceptionally rare presentation of this rare disease.

  13. Computerizing clinical practice guidelines

    DEFF Research Database (Denmark)

    Lyng, Karen Marie

    . The analysis focuses on the emergence of general clinical work practice demands on guidance • An analysis of guidance demands from clinical work practice and business strategy, focusing on implications for the design of computerised CPGs. In my research, I have applied observation studies, interviews......It is well described that hospitals have problems with sustaining high quality of care and expedient introduction of new medical knowledge. Clinical practice guidelines (CPGs) have been promoted as a remedy to deal with these problems. It is, however, also well described that application...... and compliance with CPGs in most areas of clinical practice are deficient. Computerization of CPGs has been brought forward as a method to disseminate and to support application of CPGs. Until now, CPG-computerization has focused on development of formal expressions of CPGs. The developed systems have, however...

  14. Granulomatosis with Polyangiitis Presenting with Coronary Artery and Pericardial Involvement

    Science.gov (United States)

    Dewan, Rohit; Trejo Bittar, Humberto E.; Lacomis, Joan; Ocak, Iclal

    2015-01-01

    Granulomatosis with polyangiitis is a systemic disease resulting in necrotizing vasculitis of small- and medium-sized vessels. Cardiac involvement is rare and when present usually manifests with pericarditis and coronary artery vasculitis. We report here a case of granulomatosis with polyangiitis involving the native coronary arteries, bypass graft, and pericardium with interesting imaging findings on contrast-enhanced CT and MRI. A 57-year-old man with a history of chronic headaches presented to the emergency room with syncope. Contrast-enhanced CT demonstrated extensive soft tissue attenuation around the native coronary arteries and bypass graft. Contrast-enhanced MRI demonstrated enhancing nodular soft tissue surrounding the coronary arteries, bypass graft, and pericardium. Pericardial biopsy revealed a necrotizing granulomatous pericarditis with vasculitis concerning for granulomatosis with polyangiitis. The patient demonstrated MPO-positive and PR-3 negative serologies. After being discharged on rituximab and prednisone, follow-up CT 3 years later showed significant improvement of the soft tissue thickening surrounding the coronary arteries, bypass graft, and pericardium. PMID:26798536

  15. Role of gingival manifestation in diagnosis of granulomatosis with polyangiitis (Wegener’s granulomatosis)

    Science.gov (United States)

    2015-01-01

    Purpose This report describes a case of granulomatosis with polyangiitis (GPA) in which the gingival manifestation was crucial in both making an early diagnosis and possibly in deciding the approach to treatment. Methods A 57-year-old sailor presented to the Department of Dentistry at Ulsan University Hospital complaining of gingival swelling since approximately 2 months. He had orofacial granulomatous lesions and the specific gingival manifestation of strawberry gingivitis. Results The diagnosis of GPA was made on the basis of clinical symptoms and signs, and confirmed by the presence of the anti-neutrophil cytoplasmic antibody and a positive biopsy. The patient was admitted to the hospital and subsequently placed on a disease-modifying therapy regimen that included methotrexate and prednisone. Conclusions Identification of the gingival manifestation of the disease permitted an early diagnosis and prompt therapy in a disease in which time is a crucial factor. Because of its rapid progression and potentially fatal outcome, an early diagnosis of GPA is important. Therefore, dentists should be aware of the oral signs and symptoms of such systemic diseases. PMID:26734495

  16. Cerebral lymphomatoid granulomatosis. A report of two cases, with disseminated necrotizing leukoencephalopathy in one.

    Science.gov (United States)

    Verity, M A; Wolfson, W L

    1976-10-15

    Neuropathologic findings in 2 cases of cerebral lymphomatoid granulomatosis with sequelae are presented. A 30-year old male with macular rash and pulmonary lymphomatoid granulomatosis responded to Prednisone terapy but developed acute intracranial hypertension with coma. A necrotizing hemorrhagic lesion was evident in the left putamen surrounded by diffuse and perivascular atypical lymphoplasmacytic infiltration. An 18-year old girl developed pulmonary lymphomatoid granulomatosis, diplopia, slurred speech and right hemiparesis. Brain scan, angiography and EEG suggested a left fronto-parietal mass assumed to represent lymphomatoid granulomatosis. She responded well to cerebral irradiation, intrathecal methotrexate and cytoxan but relapsed with seizures and increasing respiratory insufficiency. At autopsy, stigmata of cerebral lymphomatoid granulomatosis were absent but a parenchymatous degeneration consistent with disseminated necrotizing leukoencephalopathy following antileukemic therapy in children, was found.

  17. What is Vasculitis?

    Science.gov (United States)

    ... Skin Diseases (NIAMS)) Buerger’s Disease (MedlinePlus) Cryoglobulinemia (MedlinePlus) Granulomatosis With Polyangiitis (Wegener's Granulomatosis ) (National Institute of Allergy and Infectious Diseases) ...

  18. 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

  19. Archive of Geosample Information from the Alfred-Wegener-Institut (AWI) Core Repository

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The Alfred-Wegener-Institut (AWI) made a one-time contribution to the Index to Marine and Lacustrine Geological Samples (IMLGS) database of metadata describing 1820...

  20. Computerized ionospheric tomography

    Energy Technology Data Exchange (ETDEWEB)

    Austen, J.R.; Raymund, T.D.; Klobuchar, J.A.; Stalker, J.; Liu, C.H.

    1990-05-03

    In this paper the background of computerized tomography (CT) and its application to the ionosphere is reviewed. CT techniques, using only total electron content (TEC) data, can be used to reconstruct a two-dimensional image of the electron density in the ionosphere. The limitations of this technique are discussed and examples showing the limitations and capabilities are presented. Simulation results for two applications are presented: imaging the high latitude trough, and the correction of tracking radar range rate errors. Some possible extensions of the technique are presented.

  1. Computerized Library Networking in Canada.

    Science.gov (United States)

    Duchesne, Roderick M.; Islam, Mazharul

    1979-01-01

    Reviews a study which examined computerized bibliographic centers in Canada identifying three types: (1) library processing facility; (2) library network user group; and (3) information retrieval facility. The study also reported on ways to promote a computerized library network with emphasis on national location service. (CWM)

  2. Lymphomatoid granulomatosis: case report and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Ng, P.; Dwyer, R.; Hughes, A.; Despas, P. [Westmead Hospital, Westmead, NSW (Australia), Depts of Radiology, Neurology and Respiratory Medicine

    1997-02-01

    Lymphomatoid granulomatosis (LG) is an uncommon disease now regarded by most authors as an unusual form of lymphoma with the ability to affect any bodily system. The disease and its complications may result in a spectrum of radiological appearances. A case illustrating how cerebral lesions may mimic demyelinating plague, neoplasm or infection and how pulmonary lesions may lead to pneumopericardium is presented. The difficulty in establishing a definitive diagnosis, due to the non-specificity of symptoms, pathological investigations and radiological appearances of the disease, is emphasized. 36 refs., 5 figs.

  3. Computerizing natural history collections.

    Science.gov (United States)

    Sunderland, Mary E

    2013-09-01

    Computers are ubiquitous in the life sciences and are associated with many of the practical and conceptual changes that characterize biology's twentieth-century transformation. Yet comparatively little has been written about how scientists use computers. Despite this relative lack of scholarly attention, the claim that computers revolutionized the life sciences by making the impossible possible is widespread, and relatively unchallenged. How did the introduction of computers into research programs shape scientific practice? The Museum of Vertebrate Zoology (MVZ) at the University of California, Berkeley provides a tractable way into this under-examined question because it is possible to follow the computerization of data in the context of long-term research programs. Copyright © 2013 Elsevier Ltd. All rights reserved.

  4. Lung involvement in childhood onset granulomatosis with polyangiitis.

    Science.gov (United States)

    Filocamo, Giovanni; Torreggiani, Sofia; Agostoni, Carlo; Esposito, Susanna

    2017-04-14

    Granulomatosis with polyangiitis is an ANCA-associated systemic vasculitis with a low incidence in the pediatric population. Lung involvement is a common manifestation in children affected by granulomatosis with polyangiitis, both at disease's onset and during flares. Its severity is variable, ranging from asymptomatic pulmonary lesions to dramatic life-threatening clinical presentations such as diffuse alveolar haemorrhage. Several radiologic findings have been described, but the most frequent abnormalities detected are nodular lesions and fixed infiltrates. Interstitial involvement, pleural disease and pulmonary embolism are less common. Histology may show necrotizing or granulomatous vasculitis of small arteries and veins of the lung, but since typical features may be patchy, the site for lung biopsy should be carefully chosen with the help of imaging techniques such as computed tomography. Bronchoalveolar lavage is helpful to confirm the diagnosis of alveolar haemorrhage. Pulmonary function tests are frequently altered, showing a reduction in the diffusion capacity for carbon monoxide, which can be associated with obstructive abnormalities related to airway stenosis. Nodular lung lesions tend to regress with immunosuppressive therapy, but lung disease may also require second line treatments such as plasmapheresis. In cases of massive diffuse alveolar haemorrhage, ventilator support is crucial in the management of the patient.

  5. Cytomegalovirus retinitis presenting as vasculitis in a patient with Wegener’s granulomatosis

    Directory of Open Access Journals (Sweden)

    Paris G Tranos

    2008-08-01

    Full Text Available Paris G Tranos1, Ilias Georgalas2, Panagiota Founti1, Ioannis Ladas21Papageorgiou University Hospital, Thessaloniki, Greece; 2Department of Ophthalmology, “G.Genimatas” Hospital, Athens, GreecePurpose: To present an unusual case of cytomegalovirus (CMV retinitis in a patient with Wegener’s granulomatosis.Results: A 54-year-old lady with Wegener’s granulomatosis presented with decreased vision in her left eye. Wegener’s retinal vasculitis was diagnosed initially and the patient received treatment with oral steroids. Three days later the patient developed typical CMV retinitis.Conclusion: The likelihood of CMV retinitis in patients with Wegener’s granulomatosis should not be overlooked. Increased awareness in such cases is very important since CMV retinitis may present with less typical manifestations, which makes the correct diagnosis more challenging.Keywords: cytomegalovirus retinitis, Wegener’s granulomatosis, ganciclovir

  6. Renal Solid Mass as a Rare Presentation of Wagener's Granulomatosis: A Case Report

    Directory of Open Access Journals (Sweden)

    Mehrdad Mohammadi Sichani

    2012-01-01

    Full Text Available Wagener's granulomatosis (WG is a rheumatologic disease with unknown etiology which renal and pulmonary involvement is commonly seen. Renal involvement in Wagener's granulomatosis represents as a segmental necrotizing glomerulonephritis which is not visible with imaging techniques and usually presents with proteinuria, microhematuria, and hypertension. A rare presentation of the disease is a renal mass which can be mistaken as renal tumors, abscess, or lymphoma. We report a 22-year-old female with flank pain and fever who was admitted in our hospital. The patient underwent renal tumor biopsy and diagnosed with Wagener's granulomatosis in pathologic staining. The aim of this work is introduction of Wagener's granulomatosis as a differential diagnosis of renal tumors, to prevent unnecessary interventions and delayed treatment.

  7. Lymphomatoid granulomatosis: clinical and histopathological report of a patient presenting with spinal cord involvement

    NARCIS (Netherlands)

    Herderscheê, D.; Troost, D.; de Visser, M.; Neve, A. J.

    1988-01-01

    An unusual mode of presentation of lymphomatoid granulomatosis is reported. A 19-year-old man developed spinal cord symptoms, and magnetic resonance imaging of the spinal cord disclosed a compatible lesion. Despite medical therapy the outcome was fatal

  8. Estudio histopatologico retrospectivo de nueve casos de Granulomatosis Micotica en el tracto digestivo de caninos

    National Research Council Canada - National Science Library

    Emiro Vale-Echeto, Oswaldo; Oviedo de Vale, Maria Guadalupe; Simoes, David

    2013-01-01

    .... Sin embargo, cada dia aparecen nuevos agentes infecciosos de variada naturaleza, entre los cuales se deben mencionar los hongos, que producen las llamadas enfermedades micoticas o la Granulomatosis Micotica (GM...

  9. Validation and correction of rainfall data from the WegenerNet high density network in southeast Austria

    Science.gov (United States)

    O, Sungmin; Foelsche, U.; Kirchengast, G.; Fuchsberger, J.

    2018-01-01

    Eight years of daily rainfall data from WegenerNet were analyzed by comparison with data from Austrian national weather stations. WegenerNet includes 153 ground level weather stations in an area of about 15 km × 20 km in the Feldbach region in southeast Austria. Rainfall has been measured by tipping bucket gauges at 150 stations of the network since the beginning of 2007. Since rain gauge measurements are considered close to true rainfall, there are increasing needs for WegenerNet data for the validation of rainfall data products such as remote sensing based estimates or model outputs. Serving these needs, this paper aims at providing a clearer interpretation on WegenerNet rainfall data for users in hydro-meteorological communities. Five clusters - a cluster consists of one national weather station and its four closest WegenerNet stations - allowed us close comparison of datasets between the stations. Linear regression analysis and error estimation with statistical indices were conducted to quantitatively evaluate the WegenerNet daily rainfall data. It was found that rainfall data between the stations show good linear relationships with an average correlation coefficient (r) of 0.97 , while WegenerNet sensors tend to underestimate rainfall according to the regression slope (0.87). For the five clusters investigated, the bias and relative bias were - 0.97 mm d-1 and - 11.5 % on average (except data from new sensors). The average of bias and relative bias, however, could be reduced by about 80 % through a simple linear regression-slope correction, with the assumption that the underestimation in WegenerNet data was caused by systematic errors. The results from the study have been employed to improve WegenerNet data for user applications so that a new version of the data (v5) is now available at the WegenerNet data portal (www.wegenernet.org).

  10. HLA-DR4, DR13(6) and the ancestral haplotype A1B8DR3 are associated with ANCA-associated vasculitis and Wegeners granulomatosis

    NARCIS (Netherlands)

    Stassen, Patricia M.; Cohen-Tervaert, Jan W.; Lems, Simon P. M.; Hepkema, Bouke G.; Kallenberg, Cees G. M.; Stegeman, Coen A.

    Objectives. As the HLA system is involved in recognition of self and non-self, an association with the development of ANCA-associated vasculitis (AAV) seems probable. In this study, the relation between HLA antigens and AAV and its severity were investigated. Methods. Consecutive patients diagnosed

  11. Myeloperoxidase-Antineutrophil Cytoplasmic Antibody (ANCA)-Positive and ANCA-Negative Patients With Granulomatosis With Polyangiitis (Wegener's) Distinct Patient Subsets

    NARCIS (Netherlands)

    Miloslavsky, Eli M.; Lu, Na; Unizony, Sebastian; Choi, Hyon K.; Merkel, Peter A.; Seo, Philip; Spiera, Robert; Langford, Carol A.; Hoffman, Gary S.; Kallenberg, Cees G. M.; St Clair, E. William; Tchao, Nadia K.; Fervenza, Fernando; Monach, Paul A.; Specks, Ulrich; Stone, John H.

    2016-01-01

    Objective. To examine the relationship of antineutrophil cytoplasmic antibody (ANCA) type and ANCA-associated vasculitis (AAV) diagnosis with demographic features, disease manifestations, and clinical outcomes. We focused on patients who account for the differences between ANCA type and disease type

  12. Granulomatosis with polyangiitis confined to lacrimal gland, a case report.

    Science.gov (United States)

    Zuazo, F; González, M; Abdala, A; Olvera-Morales, O; Monroy, M H; Rodríguez-Reyes, A; Tovilla-Canales, J L; Nava-Castañeda, Á

    2016-12-22

    A 43 year-old woman consulted due to 2 months of swelling on the superolateral side of the left orbit, with pain and erythema. An excisional biopsy was performed that revealed vasculitis with polyangiitis of the lacrimal gland. A systemic study showed that no other system was compromised. Orbital involvement occurs in up to 60% of patients with granulomatosis with polyangiitis. The involvement of the lacrimal gland is rare and often unilateral. Serological tests are generally negative, both in initial stages, as in localized forms of the disease. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  13. [Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)].

    Science.gov (United States)

    Guillevin, Loïc

    2012-10-01

    Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss), is a rare necrotizing vasculitis of small-sized vessels, associated to antimyeloperoxydase ANCA in 40% of patients. EGPA occurs in patients with asthma. Asthma is sever, associated with eosinophilia and extrapulmonary symptoms. Among them, mononeuritis multiplex is the most frequent symptom. When cardiac involvement is present, prognosis is poor. Despite a good overall prognosis, deaths are caused by vasculitis activity, gastrointestinal and cardiac involvement. Treatment is well codified based on steroids, which are quickly effective. Immunosuppressants combined with corticosteroids are compulsory to treat the most sever forms, mainly when cardiac and gastrointestinal or renal symptoms are present. Copyright © 2012. Published by Elsevier Masson SAS.

  14. Urologic and male genital manifestations of granulomatosis with polyangiitis.

    Science.gov (United States)

    Alba, Marco A; Moreno-Palacios, Jorge; Beça, Sara; Cid, María C

    2015-10-01

    Granulomatosis with polyangiitis (GPA) is a systemic necrotizing granulomatous vasculitis, which predominantly affects small-sized blood vessels. Major organ involvement includes the upper/lower respiratory tract and kidneys. In contrast, genitourinary disease is rare in GPA patients, reported in <1% of cases in large cohorts. Manifestations at this level include prostatitis, destructive urethritis, genital ulcers, orchitis and renal masses. Also, high-dose cyclophosphamide, one of the main immunosuppressive drugs used for GPA treatment, is associated with bladder toxicity, i.e., hemorrhagic cystitis and cancer. Here, we review the main urogenital symptoms associated with this ANCA-associated vasculitis. In addition, cyclophosphamide-induced urologic complications are detailed. Copyright © 2015 Elsevier B.V. All rights reserved.

  15. Tuberculosis, granulomatosis with polyangiitis, or both? A case report.

    Science.gov (United States)

    Nava-Castañeda, Á; Martín, F; Voorduin, S; Zuazo, F

    2017-07-27

    Tuberculosis (TB) is a chronic granulomatose infection, and granulomatosis with polyangiitis (GP) is a small vessel vasculitis, both of which affect the lungs. The combination of these diseases is rare. Both have similar clinical features, making the differential diagnosis difficult. It concerns a 37 year-old female undergoing treatment for pulmonary TB, who presented with left ocular proptosis, eyelid and conjunctival edema and erythema. Orbital biopsy revealed GP. C-Anti-neutrophil cytoplasmic antibodies were elevated. The patient responded well to immunosuppressive treatment. TB and GP can associate. Diagnosis should include not only C-anti-neutrophil cytoplasmic antibodies, but also a biopsy, in order to select the appropriate treatment. Copyright © 2017 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  16. Complete Heart Block due to Limited Wegener’s Granulomatosis: A Case Report

    Directory of Open Access Journals (Sweden)

    SA Jahed

    2011-03-01

    Full Text Available Complete heart block is a rare manifestation of various cardiac involvementsseen in Wegener’s granulomatosis. To our knowledge there have only been twoprevious cases of complete heart block reported in patients with limited form ofWegener’s granulomatosis in English literature. We describe a 62-year-oldIranian woman presenting with complete heart block who was candidate forpacemaker installation. Limited Wegener’s granulomatosis was suspected becauseof bloody nasal discharge, fatigue, fever, arthralgia, dacrocystitis, andhistory of recurrent subglutic stenosis, but no renal involvement. The diagnosiswas subsequently confirmed by presence of granuloma in nasal mucosal biopsy andalso high level of serum anti-proteinase 3. Prednisolone and cyclophosphamidetherapy were initiated and her electrocardiography returned to normal sinusrhythm without any pacemaker installation. Awareness of unusual presentation ofWegener’s granulomatosis is important in proper diagnosis and management of thepotentially fatal cardiac involvement of the disease. Complete heart block maybe reversed by appropriate medical therapy of Wegener’s granulomatosis.

  17. Computerized sociometric assessment for preschool children

    NARCIS (Netherlands)

    Endedijk, Hinke M.; Cillessen, Antonius H. N.

    2015-01-01

    In preschool classes, sociometric peer ratings are used to measure children's peer relationships. The current study examined a computerized version of preschool sociometric ratings. The psychometric properties were compared of computerized sociometric ratings and traditional peer ratings for

  18. Technologies in computerized lexicography | Kruyt | Lexikos

    African Journals Online (AJOL)

    Although the topic of this paper is technology, focus is on functional rather than technical aspects of computerized lexicography. Keywords: computerized lexicography, electronic dictionary, electronic text corpus, lexicographer's workbench, integrated language database, automatic linguistic analysis, information retrieval, ...

  19. Computerized Proof Techniques for Undergraduates

    Science.gov (United States)

    Smith, Christopher J.; Tefera, Akalu; Zeleke, Aklilu

    2012-01-01

    The use of computer algebra systems such as Maple and Mathematica is becoming increasingly important and widespread in mathematics learning, teaching and research. In this article, we present computerized proof techniques of Gosper, Wilf-Zeilberger and Zeilberger that can be used for enhancing the teaching and learning of topics in discrete…

  20. Computerizing a Department of Psychiatry.

    Science.gov (United States)

    Lombroso, Paul; Eng, Lenny

    1987-01-01

    Describes the process by which a department of psychiatry computerized its clinical services under a single communication network. Presents the program in detail, demonstrating the recording of various clinical, administrative and demographic data. Emphasizes the value of such information for returning patients, immediate updating of information,…

  1. Perspectives on Computerized Psychological Assessment.

    Science.gov (United States)

    Butcher, James N.

    1985-01-01

    Psychological assessment, a subspecialty of psychology has served as one of the earliest proving grounds of automated technology. This Special Series highlights some of the advances in computerized psychological assessment methods and discusses some of the lingering issues and contemporary problems with automated psychological assessment…

  2. Wegener’s granulomatosis mimicking inflammatory bowel disease and presenting with chronic enteritis

    Directory of Open Access Journals (Sweden)

    Shahedi K

    2013-10-01

    Full Text Available Kamyar Shahedi,1,2 Ramy Magdy Hanna,1,2 Oleg Melamed,1,2 James Wilson2,31Department of Medicine Olive-View UCLA Medical Center, Sylmar, CA, 2David Geffen School of Medicine at UCLA, Los Angeles, CA, 3UCLA Medical Center-UCLA Stone Center, Los Angeles, CA, USAAbstract: Wegener’s granulomatosis, also known as anti-neutrophil cytoplasmic antibody (ANCA-associated vasculitis, is a small vessel vasculitis with primarily pulmonary, renal, and sinus disease manifestations. The prevalence of Wegener’s granulomatosis is three cases per 100,000 patients. Cardiovascular, neurologic, cutaneous, and joint manifestations have been reported in many case reports and case series. Gastrointestinal manifestations are less noted in Wegener’s granulomatosis, although they have been previously reported in the form of intestinal perforation and intestinal ischemia. Additionally, there are characteristic findings of vasculitis that are noted with active Wegener’s granulomatosis of the small bowel. We report a case of an elderly patient who presented with weight loss, diarrhea, and hematochezia. His symptoms were chronic and had lasted for more than 1 year before diagnosis. Inflammatory bowel disease or chronic enteritis due to Salmonella arizonae because of reptile exposure originally were suspected as etiologies of his presentation. The findings of proteinuria, renal failure, and pauci-immune glomerulonephritis on renal biopsy, in conjunction with an elevated c-ANCA titer, confirmed the diagnosis of Wegener’s granulomatosis with associated intestinal vasculitis. This case demonstrates an atypical presentation of chronic duodenitis and jejunitis secondary to Wegener’s granulomatosis, which mimicked inflammatory bowel disease.Keywords: ANCA-associated vasculitis, Wegener’s syndrome, pauci-immune glomerulonephritis, Salmonella arizonae, inflammatory bowel disease

  3. Chronic active EBV infection with features of granulomatosis with polyangiitis.

    Science.gov (United States)

    Shimomura, Masaki; Morishita, Hideaki; Meguro, Takaaki; Seto, Shiro; Kimura, Mitsuaki; Hamazaki, Minoru; Hashimoto, Ayako; Sugiyama, Yuka; Kimura, Hiroshi

    2016-07-01

    Herein, we report the case of a 13-year-old boy with multiple recurrent ulcers on his legs. He developed severe sinusitis at 10 years of age and had significant weight loss (6 kg) in the 2 months prior to admission. Histology of tissue biopsied from the ulcer indicated small vessel vasculitis and granulomatous inflammation. Given that these findings met the diagnostic criteria for granulomatosis with polyangiitis (GPA), he was treated with immunosuppressive agents. Further pathology, however, indicated Epstein-Barr virus (EBV)-encoded RNA (EBER) in most lymphocytes in the same sample. The EBER-positive lymphocytes were mainly CD4-positive T cells. The EBV-DNA load in the peripheral blood was also abnormally increased (1.0 × 10(4) copies/μg DNA). Thus, the diagnosis was established as chronic active EBV infection (CAEBV). This case illustrates the necessity of careful differential diagnosis of CAEBV owing to its clinical resemblance and pathological overlap with GPA. © 2016 Japan Pediatric Society.

  4. Lymphomatoid Granulomatosis: CT and FDG-PET Findings

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Jonathan H. [National Jewish Health, Denver (United States); Wu, Carol C.; Gilman, Matthew D.; Palmer, Edwin L.; Hasserjian, Robert P.; Sphepar, Jo-Anne O. [Massachusetts General Hospital, Boston (United States)

    2011-11-15

    Lymphomatoid granulomatosis (LG) is a rare, aggressive extranodal Epstein-Barr virus (EBV)-positive B-cell lymphoproliferative disease. The purpose of our study was to analyze the CT and fluorodeoxyglucose positron emission tomography (FDG-PET) findings of pulmonary LG. Between 2000 and 2009, four patients with pathologically proven pulmonary LG and chest CT were identified. Two of these patients also had FDG-PET. Imaging features of LG on CT and PET were reviewed. Pulmonary nodules or masses with peribronchovascular, subpleural, and lower lung zonal preponderance were present in all patients. Central low attenuation (4 of 4 patients), ground-glass halo (3 of 4 patients), and peripheral enhancement (4 of 4 patients) were observed in these nodules and masses. An air-bronchogram and cavitation were seen in three of four patients. FDG-PET scans demonstrated avid FDG uptake in the pulmonary nodules and masses. Pulmonary LG presents with nodules and masses with a lymphatic distribution, as would be expected for a lymphoproliferative disease. However, central low attenuation, ground-glass halo and peripheral enhancement of the nodules/masses are likely related to the angioinvasive nature of this disease. Peripheral enhancement and ground-glass halo, in particular, are valuable characteristic not previously reported that can help radiologists suggest the diagnosis of pulmonary LG.

  5. Juxta-vertebral lesions in granulomatosis with polyangiitis.

    Science.gov (United States)

    Ramirez, Giuseppe A; Della-Torre, Emanuel; Campochiaro, Corrado; Bozzolo, Enrica; Berti, Alvise; Praderio, Luisa; Dagna, Lorenzo; Sabbadini, Maria Grazia

    2016-12-01

    To describe the clinical, pathological, serological, and radiological characteristics of juxta-vertebral masses occurring in patients with granulomatosis with polyangiitis (GPA). We analyzed the clinical records of patients with juxta-vertebral lesions from our GPA study cohort and reviewed the English literature for other cases of GPA with juxta-vertebral localization. Out of 74 patients in our GPA study cohort, six (8%) had juxta-vertebral lesions. We found 10 cases of juxta-vertebral GPA described in the English literature. Overall, juxta-vertebral lesions were detected at GPA onset in 11/16 (69%) patients, and preferentially occurred on the right side of the spine (12/15 patients, 80%). Fifteen patients (94%) with juxta-vertebral lesions had systemic GPA. Juxta-vertebral lesions were associated with back pain at GPA onset in 8/16 (50%) patients. In all of them juxta-vertebral lesions resolved or improved after treatment. Preference for the right-anterior side of the spine, increased 18 FDG uptake on PET scan, low or absent invasiveness of the surrounding tissues, and occurrence in the context of systemic disease were the main features of juxta-vertebral GPA. Symptomatic lesions showed a better response to immunosuppressive therapies. Copyright © 2016 Elsevier Inc. All rights reserved.

  6. Characteristics of granulomatosis with polyangiitis patients in Japan.

    Science.gov (United States)

    Tsuchida, Yumi; Shibuya, Mihoko; Shoda, Hirofumi; Sumitomo, Shuji; Kubo, Kanae; Setoguchi, Keigo; Fujio, Keishi; Yamamoto, Kazuhiko

    2015-03-01

    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a disease with significant ethnic differences. Reports on characteristics of Japanese granulomatosis with polyangiitis (GPA) patients are limited, and this study was undertaken to determine the characteristics of Japanese GPA patients. This was a retrospective chart study of 24 Japanese GPA patients. GPA was defined according to the European Medicines Agency algorithm. The percentage of MPO-ANCA-positive patients was 33.3%, higher than the percentages reported in studies from Western countries. MPO-ANCA-positive GPA patients differed from PR3-ANCA-positive GPA patients in organs involved at diagnosis with MPO-ANCA-positive patients having nose and sinus involvement less frequently compared to PR3-ANCA-positive patients. Interstitial lung infiltrates were more common among MPO-ANCA-positive GPA patients compared to PR3-ANCA-positive GPA patients. Among Japanese GPA patients, the proportion of MPO-ANCA-positive patients is higher compared to reports from Western countries, and those patients are often different from the classical picture of GPA.

  7. Review article: cinnamon- and benzoate-free diet as a primary treatment for orofacial granulomatosis.

    Science.gov (United States)

    Campbell, H E; Escudier, M P; Patel, P; Challacombe, S J; Sanderson, J D; Lomer, M C E

    2011-10-01

    Orofacial granulomatosis is a rare chronic granulomatous inflammatory disease of the lips, face and mouth. The aetiology remains unclear but may involve an allergic component. Improvements have been reported with cinnamon- and benzoate-free diets. To explore the prevalence of compound and food sensitivity and examine the dietary treatments used in orofacial granulomatosis. A comprehensive literature search was carried out and relevant studies from January 1933 to January 2010 were identified using the electronic database search engines; AGRIS 1991-2008, AMED 1985-2008, British Nursing and Index archive 1985-2008, EMBASE 1980-2008, evidence based medicine review databases (e.g. Cochrane DSR), International Pharmaceutical and Medline 1950-2008. Common sensitivities identified, predominantly through patch testing, were to benzoic acid (36%) food additives (33%), perfumes and flavourings (28%), cinnamaldehyde (27%), cinnamon (17%), benzoates (17%) and chocolate (11%). The cinnamon- and benzoate-free diet has been shown to provide benefit in 54-78% of patients with 23% requiring no adjunctive therapies. A negative or positive patch test result to cinnamaldehyde, and benzoates did not predict dietary outcome. The most concentrated source of benzoate exposure is from food preservatives. Use of liquid enteral formulas can offer a further dietary therapy, particularly in children with orofacial granulomatosis. Management of orofacial granulomatosis is challenging but cinnamon- and benzoate-free diets appear to have a definite role to play. © 2011 Blackwell Publishing Ltd.

  8. Wegener′s granulomatosis of urinary tract presenting as bladder outlet obstruction

    Directory of Open Access Journals (Sweden)

    V Suryaprakash

    2006-01-01

    Full Text Available We report a rare case of Wegener′s granulomatosis involving the prostate gland in a 45-year-old male who presented with acute urinary retention. Treatment was initiated with oral cyclophosphamide and steroids. The prostate size regressed in four weeks and patient voided well after removal of catheter.

  9. Limited wegener′s granulomatosis presenting as multiple retinal vascular occlusions

    Directory of Open Access Journals (Sweden)

    Shenoy Radha

    2002-01-01

    Full Text Available In the early stages of Wegener′s granulomatosis, and in the atypical forms, the classic clinicopathologic features of the disease are often absent, delaying diagnosis. Recognition of the disease and its variant is aided by serological markers

  10. Ano-genital Granulomatosis and Crohn's Disease: A Case Series of Males Presenting with Genital Lymphoedema.

    Science.gov (United States)

    Alexakis, Christopher; Gordon, Kristiana; Mellor, Russell; Chong, Heung; Mortimer, Peter; Pollok, Richard

    2017-04-01

    Ano-genital granulomatosis is a rare chronic granulomatous condition of the skin that causes lymphoedema of the external genitalia. There is a reported association with Crohn's disease. Mechanisms of disease and optimal methods of treatment are poorly understood. A retrospective casenote review of 25 male patients with ano-genital granulomatosis presenting with genital lymphoedema was performed to determine the clinical and histopathological features of this condition and its relationship to intestinal Crohn's disease. A combination of penile and scrotal oedema was reported at presentation in 80% of patients; 40% of patients had associated intestinal Crohn's disease. The average time from symptom onset to diagnosis was 52.7 months. Half of cutaneous biopsies contained non-caseating granulomas and 14% contained intralymphatic granulomas. In all, 72% of patients responded to oral steroids initially but recurrence was common. Complete or partial response was achieved in 60% of patients treated with azathioprine. Three of six patients responded to anti-tumour necrosis factor [TNF] therapy. A small proportion of patients required circumcision or de-bulking surgery for more debilitating disease. Ano-genital granulomatosis is a rare condition that presents with genital lymphoedema, and there is frequently a protracted delay in diagnosis. There is a very strong association with intestinal Crohn's disease. Genital lymphoedema associated with gastrointestinal symptoms should prompt careful evaluation to exclude both ano-genital granulomatosis and Crohn's disease.

  11. [Disseminated histoplamosis in adolescent mimicking granulomatosis with polyangiitis].

    Science.gov (United States)

    van Weelden, Marlon; Viola, Gabriela R; Kozu, Katia T; Aikawa, Nadia E; Ivo, Claudia M; Silva, Clovis A

    2015-03-04

    Systemic histoplasmosis is an invasive fungal infection that may mimic primary vasculitis, particularly granulomatosis with polyangiitis (GPA), and was rarely described in adult patients. We reported an immunocompetent patient with disseminated histoplasmosis mimicking GPA who fulfilled European League Against Rheumatism (EULAR)/Pediatric Rheumatology International Trials Organisation (PRINTO)/Pediatric Rheumatology European Society (PRES) validated classification criteria. A 6-year old boy presented acute migratory polyarthritis with spontaneous improvement, sinus inflammation, fever, headache and abdominal pain. Serologic test for hepatitis, cytomegalovirus, human immunodeficiency virus, Epstein-Barr virus, toxoplasmosis, dengue virus and antistreptolysin O were all negative. Magnetic resonance imaging (MRI) showed moderate ascites in pelvis and pansinusitis. Antineutrophil cytoplasmic antibodies (c-ANCA) were positive. He had spontaneous remission of the symptoms including fever. At the age of 11 years and 11 months, he had sinusitis, pneumonia and epididymitis. A month later, he was hospitalized and MRI showed left eye proptosis. Cerebrospinal fluid was normal and indirect tests of fungi were negative. Two months later, he had lumbar pain and computer tomography showed a mass in the right kidney and pulmonary nodule in the right lung. He fulfilled EULAR/PRINTO/PRES criteria for GPA, however the renal biopsy showed a focal granulomatous interstitial nephritis with yeast fungal cells compatible with Histoplasma sp. He was treated with liposomal amphotericin B and itraconazole with improvement of signs and symptoms. We reported a progressive disseminated histoplasmosis case mimicking GPA. Histoplasmosis infection should be considered in immunocompetent subjects with uncommon clinical manifestations, such as arthritis, nephritis and epididymitis. Copyright © 2015 Elsevier Editora Ltda. All rights reserved.

  12. Manifestation of eosinophilic granulomatosis with polyangiitis in head and neck.

    Science.gov (United States)

    Petersen, Hannes; Götz, Paul; Both, Marcus; Hey, Matthias; Ambrosch, Petra; Bremer, Jan Phillip; Holle, Julia; Moosig, Frank; Laudien, Martin

    2015-09-01

    Besides an obvious clinical involvement of the ear, nose and throat (ENT)-region in Eosinophilic Granulomatosis with Polyangiitis (EGPA), systematic data is sparse. Only a few case series and case reports are available that particularly describe rhinological, otological or other manifestations of EGPA in the ENT-region. Therefore, the objective of this study is to systematically describe data on ENT-region involvement in a large series of EGPA patients. EGPA patients examined in the Department of Otorhinolaryngology of the Christian-Albrechts-University of Kiel between 1990 and 2010 were included in the study. Criteria for ENT-manifestation were assigned to five subgroups (history, ENT examination, audiological and rhinological diagnostic findings and cranial MRI) and documented cumulatively. EGPA patients were examined in a standardized way based on the validated Ear Nose and Throat Activity Score (ENTAS) or its precursor, including audiological and rhinological diagnostic findings. MRI scans were analysed to further evaluate ENT involvement. A total of 95 EGPA patients were included in the study. In approximately 80% of them, ENT-involvement was documented and the assumption of a frequent rhinological manifestation in patients with EGPA was confirmed. Moreover, the data reveals remarkable evidence for an otological manifestation. A missing correlation between the rhinological and the otological manifestation indicates an independent autoimmune-inflammatory process for this manifestation. The data of the largest monocentric study presented here confirms the hypothesis of a frequent ENT involvement in EGPA patients, in whom rhinological and otological manifestations are most common. Therefore, treatment should include long term follow-up and should be managed interdisciplinary.

  13. Applying Computerized Adaptive Testing in Schools.

    Science.gov (United States)

    Olsen, James B.

    1990-01-01

    Presents two studies applying computerized adaptive testing (CAT) in schools. Compared paper-administered, computer-administered, and CAT modes for administering school achievement and assessment tests. Then compared computerized adaptive aptitude test results with individually administered Weschler Intelligence Scale for Children-Revised. Found…

  14. Computerizing primary schools in rural kenya

    DEFF Research Database (Denmark)

    Ogembo, J.G.; Ngugi, B.; Pelowski, Matthew John

    2012-01-01

    This paper investigates the outstanding challenges facing primary schools' computerization in rural Kenya. Computerization of schools is often envisaged as a 'magic', or at least a particularly efficient, solution to many of the problems that developing countries face in improving primary school...... education. However, while a great deal of consideration is given to the technical issues surrounding computer implementation, government policy makers, administrators, aid organizations and individuals participating in school computerization programs often have not carefully considered the contextual...... questions surrounding this endeavour. Specifically: 1.) what problems do rural schools actually want to solve with computerization; 2.) is computerization the most important priority for rural schools; 3.) are schools ready, in terms of infrastructure, for a computer in the classroom; or 4.) might...

  15. Voriconazole-induced periostitis causing arthralgias mimicking a flare of granulomatosis with polyangiitis.

    Science.gov (United States)

    Gladue, Heather S; Fox, David A

    2013-12-01

    We describe a case of voriconazole-induced periostitis that occurred in a 68-year-old woman with granulomatosis with polyangiitis. Our patient presented with months of severe hip pain limiting her daily activities, which was initially felt to be a flare of her granulomatosis with polyangiitis. However, upon further review, she had an elevated alkaline phosphatase and periostitis on her hip radiograph; voriconazole was held, and within 2 days she had marked improvement in her pain. Although this clinical syndrome is well documented in transplant patients, it is a rare complication in patients with autoimmune disorders. However, it is important because it may cause severe arthralgias that can mimic a flare of rheumatic diseases.

  16. Chronic meningitis with multiple cranial neuropathies: A rare initial presentation of Wegener′s granulomatosis

    Directory of Open Access Journals (Sweden)

    Vikas Gupta

    2013-01-01

    Full Text Available Wegener′s granulomatosis (WG is a systemic necrotizing vasculitis that affects the small blood vessels. It mainly affects the upper and lower respiratory tract and kidneys. Central nervous system (CNS involvement is rare, and has been reported only in about 8% of cases during the course of illness. Initial presentation with neurologic affection, particularly chronic hypertrophic meningitis is very unusual. We report the case of a 34 year old male who presented with chronic hypertrophic meningitis and multiple cranial nerve involvement as the initial manifestation, without respiratory and renal symptoms. This case highlights the difficulties in diagnosing a rare disease with rarer presentation, and at the same time illustrates that Wegener′s granulomatosis should be considered in the differential diagnosis of chronic meningitis.

  17. Unifocal orofacial granulomatosis in retromolar mucosa: surgical treatment with Er,Cr:YSGG laser

    OpenAIRE

    Castelo, Pablo; Seoane Romero, Juan M.; García Caballero, Lucía; Suárez Peñaranda, J. M.; Romero Méndez, María Amparo; Varela Centelles, Pablo Ignacio

    2014-01-01

    Orofacial granulomatosis is defined by permanent or recurrent swelling of orofacial tissues with different multiform and multifocal clinical patterns. An 11-year old boy presented with a 2-month history of mucosa enlargement. Intraoral examination revealed an erythematous, polylobulated, exophytic lesion with a smooth surface located in retromolar mucosa, non-tender and non-infiltratated to palpation. The diagnosis was inflammatory lesion compatible with pyogenic granuloma and laser excision ...

  18. Cytoplasmic-anti-neutrophil cytoplasmic antibodies targeting myeloperoxidase in Wegener′s granulomatosis: A rare phenomenon

    Directory of Open Access Journals (Sweden)

    Bhavana M Venkatesh

    2014-01-01

    Full Text Available Wegener′s granulomatosis (WG patients can rarely have antineutrophil cytoplasmic antibodies (ANCAs directed against myeloperoxidase (MPO, producing a cytoplasmic pattern on indirect immunofluorescence (IIF. This has important implications in the diagnosis and pathophysiology of the disease. We present to you a report of three cases of WG, demonstrating a cytoplasmic-ANCA pattern on indirect IIF, but directed against MPO. It is necessary to diagnose a patient taking into account both the autoimmune test results and the clinical features.

  19. Otologic Manifestations and Progression in Patients with Wegener’s granulomatosis: A Survey in 55 Patients

    Science.gov (United States)

    Safavi Naini, Ali; Ghorbani, Jahangir; Montazer Lotfe Elahi, Sima; Beigomi, Mohsen

    2017-01-01

    Introduction: Granulomatosis with polyangiitis (GPA; also known as Wegener’s granulomatosis) is a primary systemic vasculitis involving the ear, nose and throat system (ENT) and lower respiratory tract. Because of the lack of knowledge regarding the clinical findings of GPA due to the limited number of studies, the current study was designed to investigate the prevalence and nature of the otology manifestations in the disease course. Materials and Methods: In the current prospective study, patients with a diagnosis of GPA from 2012–2016 were included. A definitive diagnosis was made based on the history, physical examination (otomicroscopy, Rinne and Weber test), audiometry, tympanometry, cytoplasmic and perinuclear anti-neutrophil cytoplasmic antibody (C-ANCA and P-ANCA) investigations, and pathologic studies. Results: Twenty-seven male and 28 female patients aged 41.6±15.3 years were enrolled. Ear involvement was found in 20 patients (36.3%), and the most prevalent symptom was loss of hearing followed by otalgia and tinnitus. Tinnitus improved in none of the patients. The most prevalent sign was otitis serous followed by mastoiditis and external otitis. The most important audiometry finding was sensorineural hearing loss. Pathological studies using pulmonary samples were more useful for diagnosis. Conclusions: Precise clinical examination is crucial for the early diagnosis of GPA. Otological manifestations are common, especially loss of hearing and otitis serous, and can be the first sign of this disease. Early diagnosis can lead to better treatment of Wegener’s granulomatosis. PMID:29383313

  20. Computerizing marine biota: a rational approach

    Digital Repository Service at National Institute of Oceanography (India)

    Chavan, V.S.; Chandramohan, D.; Parulekar, A.H.

    Data on marine biota while being extensive are also patchy and scattered; thus making retrieval and dissemination of information time consuming. This emphasise the need for computerizing information on marine biota with the objective to collate...

  1. Computerizing a house organ: recharting familiar territory

    Energy Technology Data Exchange (ETDEWEB)

    None

    1982-01-01

    Computerization can offer great advantages. But one publication ideally suited to computerization was slow to take advantage of the new technology. The main reason was reluctance to try an unfamiliar way of doing things. Having now switched to computerization, the publication has reaped many benefits. Among them: production time is faster; costs are lower; errors are fewer. Computerization has not been without minor problems. The most obvious is vulnerability to the rarity of a system failure. Others include the technology's potential reinforcement of overediting and of excessive reliance on extremely rapid response. Such problems, however, do not indicate weaknesses in the technology itself; rather, they reflect an incomplete adaption to it and the need for more realistic expectations. An unwarranted reluctance to innovate can slow advances in communication. Technical communicators must be willing to rechart their own familiar territory.

  2. HUMAN RELIABILITY ANALYSIS FOR COMPUTERIZED PROCEDURES

    Energy Technology Data Exchange (ETDEWEB)

    Ronald L. Boring; David I. Gertman; Katya Le Blanc

    2011-09-01

    This paper provides a characterization of human reliability analysis (HRA) issues for computerized procedures in nuclear power plant control rooms. It is beyond the scope of this paper to propose a new HRA approach or to recommend specific methods or refinements to those methods. Rather, this paper provides a review of HRA as applied to traditional paper-based procedures, followed by a discussion of what specific factors should additionally be considered in HRAs for computerized procedures. Performance shaping factors and failure modes unique to computerized procedures are highlighted. Since there is no definitive guide to HRA for paper-based procedures, this paper also serves to clarify the existing guidance on paper-based procedures before delving into the unique aspects of computerized procedures.

  3. 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

  4. 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...

  5. VCRC Tissue Repository

    Science.gov (United States)

    2018-01-18

    Aortitis; Cutaneous Vasculitis; Eosinophilic Granulomatosis With Polyangiitis; Giant Cell Arteritis; Granulomatosis With Polyangiitis (Wegener's); Henoch-Schonlein Purpura; IgA Vasculitis; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu Arteritis; Churg-Strauss Syndrome

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

    DEFF Research Database (Denmark)

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

    2017-01-01

    diagnosed before their eighteenth birthday as having granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or ANCA-positive pauci-immune glomerulonephritis. The primary outcome measure was achievement of disease remission...

  7. Genetically Distinct Subsets within ANCA-Associated Vasculitis

    NARCIS (Netherlands)

    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; Harper, Lorraine; Hruskova, 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, Mrten; Stegeman, Coen A.; Tesar, Vladimir; Vaglio, Augusto; Wieczorek, Stefan; Wilde, Benjamin; Zwerina, Jochen; Rees, Andrew J.; Clayton, David G.; Smith, Kenneth G. C.

    2012-01-01

    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

  8. Advances in Pathogenesis and Treatment of ANCA-associated Vasculitis

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.

    2014-01-01

    Anti-neutrophil cytoplasmic autoantibodies (ANCA) directed to proteinase 3 (PR3-ANCA) and myeloperoxidase (MPO-ANCA) are sensitive and specific markers for their associated diseases, granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) and microscopic polyangiitis (MPA),

  9. Wegner′s granulomatosis developing for the first time in a patient eight years after starting maintenance hemodialysis

    Directory of Open Access Journals (Sweden)

    Ahmad Ramadan Ali

    2015-01-01

    Full Text Available Wegener′s granulomatosis is a serious autoimmune disorder characterized by necrotizing small-vessel vasculitis. It is a multisystem disease that primarily affects the lung and kidneys. Previous studies indicated few relapses of vasculitis after hemodialysis due to uremic immunosuppression. Our case report describes an end-stage renal failure patient who had developed non-caseating lung granulomata with giant cell formation and fibrinoid necrosis of arterial media that is consistent with Wegner′s granulomatosis for the first time and eight years after initiation of maintenance hemodialysis. We believe that such a phenomenon has rarely been reported.

  10. Educational Needs of Patients With Systemic Vasculitis

    Science.gov (United States)

    2014-07-11

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

  11. [C-ANCA positive necrotising scleritis and multiple sclerosis compatible with ocular Wegener: treatment with rituximab].

    Science.gov (United States)

    Aldasoro-Cáceres, V; Aldasoro-Cáceres, I; Pérez-Moreiras, J V; Murié-Fernández, M; Ibáñez-Bosch, R

    2014-01-01

    A patient diagnosed with necrotizing scleritis, c-ANCA+ an orbital pseudotumour, and possible multiple sclerosis in 2003 was treated with oral cyclophosphamide and steroids with partial response. Between 2005-2010 she suffered self-limited episodes. In 2010 a first scleral transplant was performed with poor outcome. She was treated with rituximab, and a second graft was performed with good results. At 12 months there was no change in magnetic resonance and the second graft healed. Wegener's disease with limited involvement of the orbit and/or the eye is a rare condition. The histopathology, blood analysis, symptoms and good response to treatment are the key to its diagnosis. Copyright © 2011 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.

  12. Computerized occlusal analysis in bruxism

    Directory of Open Access Journals (Sweden)

    Lazić Vojkan

    2006-01-01

    Full Text Available Introduction. Sleep bruxism as nocturnal parafunction, also known as tooth grinding, is the most common parasomnia (sleep disorder. Most tooth grinding occurs during rapid eye movement - REM sleep. Sleep bruxism is an oral habit characterized by rhythmic activity of the masticatory muscles (m. masseter that causes forced contact between dental surfaces during sleep. Sleep bruxism has been associated with craniomandibular disorders including temporomandibular joint discomfort, pulpalgia, premature loss of teeth due to excessive attrition and mobility, headache, muscle ache, sleep interruption of an individual and problems with removable and fixed denture. Basically, two groups of etiological factors can be distinguished, viz., peripheral (occlusal factors and central (pathophysiological and psychological factors. The role of occlusion (occlusal discrepancies as the causative factor is not enough mentioned in relation to bruxism. Objective. The main objective of this paper was to evaluate the connection between occlusal factors and nocturnal parafunctional activities (occlusal disharmonies and bruxism. Method. Two groups were formed- experimental of 15 persons with signs and symptoms of nocturnal parafunctional activity of mandible (mean age 26.6 years and control of 42 persons with no signs and symptoms of bruxism (mean age 26.3 yrs.. The computerized occlusal analyses were performed using the T-Scan II system (Tekscan, Boston, USA. 2D occlusograms were analyzed showing the occlusal force, the center of the occlusal force with the trajectory and the number of antagonistic tooth contacts. Results. Statistically significant difference of force distribution was found between the left and the right side of the arch (L%-R% (t=2.773; p<0.02 in the group with bruxism. The difference of the centre of occlusal force - COF trajectory between the experimental and control group was not significant, but the trajectory of COF was longer in the group of

  13. Successful application of extracorporeal membrane oxygenation due to pulmonary hemorrhage secondary to granulomatosis with polyangiitis

    Directory of Open Access Journals (Sweden)

    Hohenforst-Schmidt W

    2013-07-01

    Full Text Available Wolfgang Hohenforst-Schmidt,1 Arndt Petermann,2 Aikaterini Visouli,3 Paul Zarogoulidis,4 Kaid Darwiche,5 Ioanna Kougioumtzi,6 Kosmas Tsakiridis,3 Nikolaos Machairiotis,6 Markus Ketteler,2 Konstantinos Zarogoulidis,4 Johannes Brachmann11II Medical Clinic, Coburg Clinic, University of Wuerzburg, Coburg, Germany; 2Division of Nephrology, Coburg Clinic, University of Wuerzburg, Coburg, Germany; 3Cardiothoracic Surgery Department, “Saint Luke” Private Hospital, Thessaloniki, Greece; 4Pulmonary Department, “G Papanikolaou” General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece; 5Department of Interventional Pneumology, Ruhrlandklinik, West German Lung Center, University Hospital, University of Duisburg-Essen, Essen, Germany; 6Surgery Department (National Health System, University General Hospital of Alexandroupolis, Alexandroupolis, GreeceAbstract: Extracorporeal membrane oxygenation (ECMO is increasingly applied in adults with acute refractory respiratory failure that is deemed reversible. Bleeding is the most frequent complication during ECMO support. Severe pre-existing bleeding has been considered a contraindication to ECMO application. Nevertheless, there are cases of successful ECMO application in patients with multiple trauma and hemorrhagic shock or head trauma and intracranial hemorrhage. ECMO has proved to be life-saving in several cases of life-threatening respiratory failure associated with pulmonary hemorrhage of various causes, including granulomatosis with polyangiitis (Wegener’s disease. We successfully applied ECMO in a 65-year-old woman with acute life-threatening respiratory failure due to diffuse massive pulmonary hemorrhage secondary to granulomatosis with polyangiitis, manifested as severe pulmonary-renal syndrome. ECMO sustained life and allowed disease control, together with plasmapheresis, cyclophosphamide, corticoids, and renal replacement therapy. The patient was successfully weaned from ECMO

  14. Focal segmental glomerulosclerosis as the sole renal lesion in Wegener′s granulomatosis

    Directory of Open Access Journals (Sweden)

    Ibrahim Salwa

    2010-01-01

    Full Text Available Wegener′s granulomatosis usually presents with focal necrotizing glomerulonephritis with crescents. We present here a 45-year old man who was treated for tuberculosis and later pre-sented with bilateral ankle swelling. His serum creatinine was 2.4 mg/dL and urine analysis revealed hematuria and proteinuria. His 24-hour urine protein excretion was 1.9 g. Anti neutrophil cytoplasmic antibodies (ANCA test was positive with cytoplasmic florescence pattern. Renal biopsy revealed focal segmental sclerosis with no active vasculitis and lung biopsy revealed extensive breakdown with cavitations and scattered granulomas.

  15. Spinal cord compression from Wegener’s granulomatosis: an unusual presentation

    Science.gov (United States)

    Roy, Deb; Phan, Kevin; Mobbs, Ralph J.; Selby, Michael

    2016-01-01

    Wegener’s granulomatosis (WG) causing spinal cord compression is very rare with only few cases reported in literature. We present a case report with review of literature. A 55-year-old lady with known WG presented with acute on chronic spinal cord compression. MRI scan revealed spinal cord compression anteriorly and posteriorly at T2–T5 level. Patient underwent urgent surgical decompression with excision of the posterior dural lesion with synthetic duraplasty. Patient made good neurological recovery. Histopathology revealed features consistent with WG. A rare case of spinal cord compression from WG is presented. Urgent surgical decompression with duraplasty resulted in good neurological outcome. PMID:28097250

  16. Dacryoadenitis with Ptosis and Diplopia as the Initial Presentation of Granulomatosis with Polyangiitis.

    Science.gov (United States)

    Hibino, Makoto; Kondo, Tetsuri

    2017-10-01

    A 77-year-old Japanese woman presented to an ophthalmologist with an erythematous swollen upper eyelid and diplopia which was initially diagnosed to be idiopathic dacryoadenitis on the basis of a histological evaluation of an orbital mass that was in remission following a 3.5-month period of systemic corticosteroid therapy. She subsequently developed respiratory symptoms, and was finally diagnosed with systemic granulomatosis with polyangiitis (GPA) based on the clinical and histological features. She was successfully treated with corticosteroids and azathioprine. Dacryoadenitis in the form of an orbital inflammatory pseudotumor may be an initial presenting feature of GPA, sometimes as the limited phenotype, and occasionally progressing to systemic disease.

  17. Acute Myocarditis in a Patient with Newly Diagnosed Granulomatosis with Polyangiitis

    DEFF Research Database (Denmark)

    Munch, Anne; Sundbøll, Jens; Høyer, Søren

    2015-01-01

    A 22-year-old woman recently diagnosed with granulomatosis with polyangiitis (GPA) was admitted to the department of cardiology due to chest pain and shortness of breath. The ECG showed widespread mild PR-segment depression, upwardly convex ST-segment elevation, and T-wave inversion. The troponin T...... level was elevated at 550 ng/L. Transthoracic echocardiography showed basal inferoseptal thinning and hypokinesis, mild pericardial effusion, and an overall preserved left ventricular ejection fraction of 55%. Global longitudinal strain, however, was clearly reduced. Cardiac magnetic resonance imaging...

  18. Acute Myocarditis in a Patient with Newly Diagnosed Granulomatosis with Polyangiitis

    DEFF Research Database (Denmark)

    Munch, Anne; Sundbøll, Jens; Høyer, Søren

    2015-01-01

    level was elevated at 550 ng/L. Transthoracic echocardiography showed basal inferoseptal thinning and hypokinesis, mild pericardial effusion, and an overall preserved left ventricular ejection fraction of 55%. Global longitudinal strain, however, was clearly reduced. Cardiac magnetic resonance imaging......A 22-year-old woman recently diagnosed with granulomatosis with polyangiitis (GPA) was admitted to the department of cardiology due to chest pain and shortness of breath. The ECG showed widespread mild PR-segment depression, upwardly convex ST-segment elevation, and T-wave inversion. The troponin T...

  19. Eosinophilic granulomatosis with polyangiitis with severe pulmonary hemorrhage treated with rituximab

    Directory of Open Access Journals (Sweden)

    S Baikunje

    2016-01-01

    Full Text Available Eosinophilic granulomatosis with polyangiitis (EGPA is characterized by systemic vasculitis, asthma and eosinophilia. Severe pulmonary hemorrhage is rare. Renal involvement is seen in approximately 25% and can vary from isolated urinary abnormality to rapidly progressive glomerulonephritis. There is limited evidence to support the use of rituximab in this condition. We present a patient with EGPA who had severe pulmonary hemorrhage and rapidly progressive glomerulonephritis. He responded to standard treatment including prednisolone, cyclophosphamide, and plasma exchange. He subsequently had a relapse of pulmonary hemorrhage that was treated successfully with rituximab.

  20. A Rare Manifestation of Crohn's Disease: Sinonasal Granulomatosis. Report of a Case and Review of Literature

    Directory of Open Access Journals (Sweden)

    Lilia Baili

    2014-10-01

    Full Text Available Crohn’s disease is a granulomatous inflammatory bowel disease. Its pathologic findings include non-contiguous chronic inflammation and non-caseating granulomas, sometimes with extra-intestinal localizations. Sinonasal manifestations of Crohn’s disease are quite rare and only a few cases have been reported up to date in the worldwide literature. They are characterized by chronic mucosal inflammation, obstruction, bleeding and occasionally septal perforation. We report a case of sinonasal granulomatosis revealing Crohn’s disease in a 22-year-old woman and go over the available literature on sinonasal involvement in Crohn’s disease.

  1. Chronic skin lichenification as unusual presentation of eosinophilic granulomatosis with polyangitis: case report and literature review

    Directory of Open Access Journals (Sweden)

    Francesco Sbrana

    2016-04-01

    Full Text Available Eosinophilic granulomatosis with polyangitis (EGPA is an uncommon ANCA-associated systemic small-vessel necrotizing vasculitis. At times, EGPA presenting manifestations can be very different from the usually recognized disease patterns. We report a 52-year-old female patient with 3 years history of itching. During the time occurred a chronic skin lichenification on her legs and gradually developed a full-blown ANCA-MPO positive EGPA in combination with blood hypereosinophilia, eosinophilic vasculitis at skin biopsy, subclinical asthma and chronic rhinosinusitis.

  2. A rare case of primary orofacial granulomatosis of gingiva during pregnancy

    Science.gov (United States)

    Joshipura, Vaibhavi; Mahantesha, S; Subbaiah, Shobha Krishna; Lakkasetty, Yogesh T

    2015-01-01

    Orofacial granulomatosis (OFG) comprises a group of diseases characterized by noncaseating granulomatous inflammation affecting the soft tissues of the oral and maxillofacial region. It is important to establish the diagnosis accurately because this condition is sometimes a manifestation of many systemic conditions like Crohn's disease or sarcoidosis. The clinical outcome of OFG patients continues to be unpredictable. Current therapies remain unsatisfactory. This article reports a rare case of isolated OFG with langhans type giant cells and inflammatory infiltrate without any systemic involvement, in which the condition was a manifestation of pregnancy. The diagnostic approach to and the treatment of OFG are reviewed. PMID:26980981

  3. Radiation exposure from musculoskeletal computerized tomographic scans.

    Science.gov (United States)

    Biswas, Debdut; Bible, Jesse E; Bohan, Michael; Simpson, Andrew K; Whang, Peter G; Grauer, Jonathan N

    2009-08-01

    Computerized tomographic scans are routinely obtained to evaluate a number of musculoskeletal conditions. However, since computerized tomographic scans expose patients to the greatest amounts of radiation of all imaging modalities, the physician must be cognizant of the effective doses of radiation that are administered. This investigation was performed to quantify the effective doses of computerized tomographic scans that are performed for various musculoskeletal applications. The digital imaging archive of a single institution was retrospectively reviewed to identify helical computerized tomographic scans that were completed to visualize the extremities or spine. Imaging parameters were recorded for each examination, and dosimetry calculator software was used to calculate the effective dose values according to a modified protocol derived from publication SR250 of the National Radiological Protection Board of the United Kingdom. Computerized tomographic scans of the chest, abdomen, and pelvis were also collected, and the effective doses were compared with those reported by prior groups in order to validate the results of the current study. The mean effective doses for computerized tomographic scans of the chest, abdomen, and pelvis (5.27, 4.95, and 4.85 mSv, respectively) were consistent with those of previous investigations. The highest mean effective doses were recorded for studies evaluating the spine (4.36, 17.99, and 19.15 mSv for the cervical, thoracic, and lumbar spines, respectively). In the upper extremity, the effective dose of a computerized tomographic scan of the shoulder (2.06 mSv) was higher than those of the elbow (0.14 mSv) and wrist (0.03 mSv). Similarly, the effective dose of a hip scan (3.09 mSv) was significantly higher than those observed with knee (0.16 mSv) and ankle (0.07 mSv) scans. Computerized tomographic scans of the axial and appendicular skeleton are associated with substantially elevated radiation exposures, but the effective dose

  4. Orofacial granulomatosis in children can be the initial manifestation of systemic disease: a presentation of two cases

    DEFF Research Database (Denmark)

    Simonsen, Anne Birgitte; Deleuran, Mette

    2014-01-01

    Orofacial granulomatosis is a chronic granulomatous condition characterized by relapsing and remitting lip swelling and oral involvement that may include deep ulcers, tags and cobblestone formation. It occurs as an independent entity but also in conjunction with systemic diseases such as tubercul...

  5. Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis

    Directory of Open Access Journals (Sweden)

    Robert Ali

    2015-01-01

    Full Text Available Granulomatosis with polyangiitis (GPA, previously termed Wegener’s Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched.

  6. The Diagnostic Value of Alpha-1-Antitrypsin Phenotype in Patients with Granulomatosis with Polyangiitis

    Directory of Open Access Journals (Sweden)

    M. Y. Pervakova

    2016-01-01

    Full Text Available The deficiency of alpha-1 protease inhibitor, or alpha-1-antitrypsin (A1AT, predisposes to chronic lung diseases and extrapulmonary pathology. Besides classical manifestations, such as pulmonary emphysema and liver disease, alpha-1-antitrypsin deficiency (A1ATD is also known to be associated with granulomatosis with polyangiitis (GPA or Wegener’s granulomatosis. The aim of our study was to evaluate the frequency of allelic isoforms of A1AT and their clinical significance among GPA patients. Detailed clinical information, including Birmingham Vasculitis Activity Score (BVAS, incidence of lung involvement, anti-proteinase 3 (PR3 antibodies concentrations, and other laboratory data were collected in 38 GPA patients. We also studied serum samples obtained from 46 healthy donors. In all collected samples A1AT phenotyping by isoelectrofocusing (IEF and turbidimetric A1AT measurement were performed. Abnormal A1AT variants were found in 18.4% (7/38 of cases: 1 ZZ, 4 MZ, 2 MF, and only 1 MZ in control group (2%. The mean A1AT concentration in samples with atypical A1AT phenotypes was significantly lower (P=0.0038 than in normal A1AT phenotype. We found that patients with abnormal A1AT phenotypes had significantly higher vasculitis activity (BVAS as well as anti-PR3 antibodies concentration. We conclude that A1AT deficiency should be considered in all patients with GPA.

  7. Pulmonary talc granulomatosis mimicking malignant disease 30 years after last exposure: a case report

    Directory of Open Access Journals (Sweden)

    Krimsky William S

    2008-07-01

    Full Text Available Abstract Introduction Pulmonary talc granulomatosis is a rare disorder characterized by the development of foreign body granuloma secondary to talc exposure. Previous case reports have documented the illness in current intravenous drug users who inject medications intended for oral use. We present a rare case of the disease in a patient with a distant history of heroin abuse who presented initially with history and imaging findings highly suggestive of malignancy. Case presentation A 53-year-old man reported a 4-month history of increasing dyspnea and weight loss. He had a long history of smoking and admission chest X-ray revealed a density in the right hemithorax. Computed tomography confirmed a probable mass with further speculated opacities in both lung fields suspicious for malignant spread. Biopsies obtained using endobronchial ultrasound-guided aspiration returned negative for malignancy and showed bronchial epithelial cells with foreign body giant cell reaction and polarizable birefringent talc crystals. Conclusion This case demonstrates a rare presentation of talc granulomatosis three decades after the last likely exposure. The history and imaging findings in a chronic smoker were initially strongly suggestive of malignant disease, and we recommend that talc-induced lung disease is considered in any patient with multiple scattered pulmonary lesions and a history of intravenous drug use. Confirmation of the disease by biopsy is essential, but unfortunately there are few successful proven management options for patients with worsening disease.

  8. Successful endonasal dacryocystorhinostomy in a patient with Wegener’s granulomatosis

    Directory of Open Access Journals (Sweden)

    P Eloy

    2009-11-01

    Full Text Available P Eloy, E Leruth, B Bertrand, Ph RombauxENT and HNS department, Cliniques Universitaires de Mont-Godinne, Université Catholique de Louvain, 5530, Yvoir, BelgiumAbstract: Wegener’s granulomatosis (WG is one form of idiopathic autoimmune vasculitis. The disease has a predilection for the upper and lower respiratory tracts (lungs, nose, sinus, and kidneys. WG may be systemic, severe, and potentially lethal, but it may also be limited to the otolaryngological area or to the eyes and the orbits. Obstruction of the lacrimal pathway is a possible complication of the disease that affects approximately 7% of patients with WG. It usually occurs as a direct extension of sinonasal disease and typically is a late manifestation. Management of such a condition is generally viewed as difficult. We report the case of a patient with a quiescent WG limited to the otolaryngological area. This patient presented a bilateral obstruction of the nasolacrimal ducts caused by bilateral extensive adhesions in the nasal cavity. Because she had several episodes of left-side acute dacryocystitis which necessitated several courses of broad-spectrum antibiotics, she successfully underwent an endonasal endoscopic dacryocystorhinostomy using a diode laser and powered instrumentation. The authors describe the clinical case, the surgical technique, and review the literature.Keywords: Wegener’s granulomatosis, recurrent dacryocystitis, endonasal DCR, diode, laser, powered instrumentation

  9. Oxidative Stress and Free-Radical Oxidation in BCG Granulomatosis Development

    Directory of Open Access Journals (Sweden)

    Elena Menshchikova

    2013-01-01

    Full Text Available Background. Little is known about the role of free-radical and oxidative stress signaling in granuloma maturation and resolution. We aimed to study the activity of free-radical oxidation processes in the dynamics of BCG-induced generalized granulomatosis in mice. Methods. Chronic granulomatous inflammation was induced in male BALB/c mice by intravenously injecting the BCG vaccine, and the production of oxidative stress (activity of free-radical oxidation processes and histological changes in the lungs, liver, and peritoneal exudate were measured 3, 30, 60, and 90 days after infection. Results. The tuberculous granuloma numerical density and diameter continuously increased from day 30 to day 90, and the macrophage content within the granulomas progressively diminished with a concomitant elevation in the number of epithelioid cells. The activity of the free-radical oxidation processes in the liver (i.e., the intensity of the homogenate chemiluminescence reached a maximum at postinfection day 60 and subsequently began to decrease. The peak generation of reactive oxygen species by phagocytes in the peritoneal exudate (measured using flow cytometry was also shifted in time and fell on day 30. Conclusions. The rise in the steady-state concentration of H2O2 in the liver of mice with BCG-induced granulomatosis is not related to local H2O2 production by phagocytes, and a decrease in the severity of generalized inflammation precedes the resolution of local inflammation.

  10. The EORTC emotional functioning computerized adaptive test

    DEFF Research Database (Denmark)

    Gamper, Eva-Maria; Grønvold, Mogens; Petersen, Morten Aa

    2014-01-01

    The European Organisation for Research and Treatment of Cancer (EORTC) Quality of Life Group is currently developing computerized adaptive testing measures for the Quality of Life Questionnaire Core-30 (QLQ-C30) scales. The work presented here describes the development of an EORTC item bank...

  11. Computerized management information systems and organizational structures

    Science.gov (United States)

    Zannetos, Z. S.; Sertel, M. R.

    1970-01-01

    The computerized management of information systems and organizational structures is discussed. The subjects presented are: (1) critical factors favoring centralization and decentralization of organizations, (2) classification of organizations by relative structure, (3) attempts to measure change in organization structure, and (4) impact of information technology developments on organizational structure changes.

  12. Computerized management support for swine breeding farms

    NARCIS (Netherlands)

    Huirne, R.B.M.

    1990-01-01

    1. INTRODUCTION

    The investigations described in this thesis have been directed towards computerized management support for swine breeding farms, focused on sow productivity and profitability. The study is composed of three basic parts: (1) basic description and

  13. Computerizing Maintenance Management Improves School Processes.

    Science.gov (United States)

    Conroy, Pat

    2002-01-01

    Describes how a Computerized Maintenance Management System (CMMS), a centralized maintenance operations database that facilitates work order procedures and staff directives, can help individual school campuses and school districts to manage maintenance. Presents the benefits of CMMS and things to consider in CMMS selection. (EV)

  14. Implementation of a Computerized Maintenance Management System

    Science.gov (United States)

    Shen, Yong-Hong; Askari, Bruce

    1994-01-01

    A primer Computerized Maintenance Management System (CMMS) has been established for NASA Ames pressure component certification program. The CMMS takes full advantage of the latest computer technology and SQL relational database to perform periodic services for vital pressure components. The Ames certification program is briefly described and the aspects of the CMMS implementation are discussed as they are related to the certification objectives.

  15. Computerized Facilities Layout Design | Mulugeta | Zede Journal

    African Journals Online (AJOL)

    Facilities are crucial as they usually represent the largest and the most expensive assets of an organization. Determining location of machines, workstations, and other facilities are layout problems in a manufacturing plant. Different computerized algorithms have been developed to optimize the flow of materials within a ...

  16. Violations of ignorability in computerized adaptive testing

    NARCIS (Netherlands)

    Glas, Cornelis A.W.

    Using auxiliary information and allowing item review in computerized adaptive testing produces a violation of the ignorability principle for missing data (Rubin, 1976) that may bias parameter estimates in IRT models. However, the violation of ignorability does not automatically lead to bias. In this

  17. Computerized technique for recording board defect data

    Science.gov (United States)

    R. Bruce Anderson; R. Edward Thomas; Charles J. Gatchell; Neal D. Bennett; Neal D. Bennett

    1993-01-01

    A computerized technique for recording board defect data has been developed that is faster and more accurate than manual techniques. The lumber database generated by this technique is a necessary input to computer simulation models that estimate potential cutting yields from various lumber breakdown sequences. The technique allows collection of detailed information...

  18. [Computerization of hospital blood banks in France].

    Science.gov (United States)

    Daurat, G; Py, J-Y

    2012-11-01

    In France, most blood products are delivered by the établissement francais du sang, directly to the recipients, and hospital blood banks deliver a minor part, but are independent from it. However that may be, hospital blood banks are hazardous activities regarding to recipients, blood products, blood supply of the hospital and regional blood supply. Because of the high risk level, a computerized information system is compulsory for all hospital blood banks, except for those only devoted to vital emergency transfusion. On the field, the integration of computerization in the different processes is very heterogeneous. So, it has been decided to publish guidelines for computerizing hospital blood banks information systems and production management. They have been built according to risk assessment and are intended to minimize those risks. The principle is that all acquisition and processing of data about recipients or blood products and tracking, must be fully computerized and that the result of all manual processes must be checked by computer before proceeding to the next step. The guidelines list the different processes and, for each of them, the functions the software must play. All together, they form the basic level all hospital blood banks should reach. Optional functions are listed. Moreover, the guidelines are also aimed to be a common tool for regional health authorities who supervise hospital blood banks. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  19. Robinson's Computerized Strabismus Model Comes of Age

    NARCIS (Netherlands)

    H.J. Simonsz (Huib); H. Spekreijse (Henk)

    1996-01-01

    textabstractIn this article we review our further development of D.A. Robinson's computerized strabismus model. First, an extensive literature study has been carried out to get more accurate data on the anatomy of the average eye and the eye muscles, and about how these vary with age and with

  20. Neumayer III and Kohnen Station in Antarctica operated by the Alfred Wegener Institute

    Directory of Open Access Journals (Sweden)

    Christine Wesche

    2016-08-01

    Full Text Available The Alfred Wegener Institute operates two stations in Dronning Maud Land, Antarctica. The German overwintering station Neumayer III is located on the Ekström Ice Shelf at 70°40’S and 08°16’W and is the logistics base for three long-term observatories (meteorology, air chemistry and geophysics and nearby research activities. Due to the vicinity to the coast (ca. 20 km from the ice shelf edge, the Neumayer III Station is the junction for many German Antarctic expeditions, especially as the starting point for the supply traverse for the second German station Kohnen. The summer station Kohnen is located about 600 km from the coast and 750 km from Neumayer III Station on the Antarctic plateau at 75°S and 00°04’E. It was erected as the base for the deep-drilling ice core project, which took place between 2001 and 2006. Since then Kohnen Station is used as a logistics base for different research projects.

  1. [Recurrence of rectal cancer in computerized tomography].

    Science.gov (United States)

    Kuckein, D

    1981-04-01

    In contrast to conventional methods of x-ray diagnosis, recurrence of a rectum carcinoma may be visualized directly by means of computerized tomography. CT criteria of a tumor relapse are discussed. Based on own cases, it is shown that the method is usually employed too late. CT as follow-up examination should be used as a routine procedure in order to establish an early diagnosis of tumor recurrence.

  2. Computerized flow monitors detect small kicks

    Energy Technology Data Exchange (ETDEWEB)

    McCann, D.; White, D. (Sedco Forex, Paris (FR))

    1992-02-24

    This paper reports on a smart alarm system installed on a number of offshore rigs and one land rig which can detect kicks more quickly than conventional systems. This rapid kick detection improves rig safety because the smaller the detected influx, the easier it is to control the well. The extensive computerized monitoring system helps drilling personnel detect fluid influxes and fluid losses before the changes in flow would normally be apparent.

  3. Computerized models for strategic planning and marketing.

    Science.gov (United States)

    Coffey, R J

    1985-03-01

    In the rapidly changing health care industry, planning and marketing based primarily on historical information is no longer sufficient. The use of computerized models to evaluate alternatives is proposed as a key part of strategic planning and marketing. The general concept, approach, components, and uses of such models are described in general, followed by an example model including the inputs, formats, and outputs, Finally, the use and interpretation of such models are discussed.

  4. Computerized implant-dentistry: Advances toward automation

    Directory of Open Access Journals (Sweden)

    Minkle Gulati

    2015-01-01

    Full Text Available Advancements in the field of implantology such as three-dimensional imaging, implant-planning software, computer-aided-design/computer-aided-manufacturing (CAD/CAM technology, computer-guided, and navigated implant surgery have led to the computerization of implant-dentistry. This three-dimensional computer-generated implant-planning and surgery has not only enabled accurate preoperative evaluation of the anatomic limitations but has also facilitated preoperative planning of implant positions along with virtual implant placement and subsequently transferring the virtual treatment plans onto the surgical phase via static (guided or dynamic (navigated systems aided by CAD/CAM technology. Computerized-implant-dentistry being highly predictable and minimally invasive in nature has also allowed implant placement in patients with medical comorbidities (e.g. radiation therapy, blood dyscrasias, in patients with complex problems following a significant alteration of the bony anatomy as a result of benign or malignant pathology of the jaws or trauma and in patients with other physical and emotional problems. With significant achievements accomplished in the field of computerized implant-dentistry, attempts are now been made toward complete automation of implant-dentistry.

  5. Computerized implant-dentistry: Advances toward automation.

    Science.gov (United States)

    Gulati, Minkle; Anand, Vishal; Salaria, Sanjeev Kumar; Jain, Nikil; Gupta, Shilpi

    2015-01-01

    Advancements in the field of implantology such as three-dimensional imaging, implant-planning software, computer-aided-design/computer-aided-manufacturing (CAD/CAM) technology, computer-guided, and navigated implant surgery have led to the computerization of implant-dentistry. This three-dimensional computer-generated implant-planning and surgery has not only enabled accurate preoperative evaluation of the anatomic limitations but has also facilitated preoperative planning of implant positions along with virtual implant placement and subsequently transferring the virtual treatment plans onto the surgical phase via static (guided) or dynamic (navigated) systems aided by CAD/CAM technology. Computerized-implant-dentistry being highly predictable and minimally invasive in nature has also allowed implant placement in patients with medical comorbidities (e.g. radiation therapy, blood dyscrasias), in patients with complex problems following a significant alteration of the bony anatomy as a result of benign or malignant pathology of the jaws or trauma and in patients with other physical and emotional problems. With significant achievements accomplished in the field of computerized implant-dentistry, attempts are now been made toward complete automation of implant-dentistry.

  6. Granulomatosis de células de Langerhans: Presentación de un caso

    Directory of Open Access Journals (Sweden)

    . Caridad Nazco Ríos

    1998-12-01

    Full Text Available Se reporta un inusual caso de granulomatosis de células de Langerhans localizado en maxilares en un paciente de 30 meses de edad, quien presentaba además de las lesiones líticas óseas, lesiones de aspecto tumoral en encía palatina y lingual de ambas arcadas dentarias. El examen óseo no arrojaba compromiso de otros huesos. El diagnóstico histológico fue corroborado por el Centro Nacional de Referencia en Anatomía Patológica.A unusual case of Langerhans´ cells is reported, located in maxillae of a 30 months old patients, who, apart from bone lithic injuries, showed injuries of tumoral type in lingual and palatine gingiva of both dental archs. According to bone examination, there weren´t involvement of other bones. Histologic diagnosis was corroborated by National Reference Center in Pathologic Anatomy.

  7. Infectious and Noninfectious Granulomatosis in Patient with Multiple Sclerosis: Diagnostic Dilemmas and Followup

    Directory of Open Access Journals (Sweden)

    Jelena Paovic

    2014-01-01

    Full Text Available Patient was followed up over the course of 30 years. In 1978, after severe systemic infection followed by fever, pulmonary edema, and numerous neurological manifestations, patient was differentially diagnosed with apoplectic form of multiple sclerosis (MS, which was confirmed a year later via neurological and MRI findings. Approximately 20 years following the initial attack, sarcoidosis was diagnosed during the regular preoperative procedures required for cataract surgery. As consequence of lower immune system, infectious granulomatosis in form of pulmonary tuberculosis developed. Ophthalmological findings revealed bilateral retrobulbar neuritis (RBN approximately six years after initial attack. This developed into total uveitis with retinal periphlebitis and anterior granulomatous uveitis—all of which are clinically similar in both MS and sarcoidosis.

  8. Suppurative dacroadenitis causing ocular sicca syndrome in classic Wegener′s granulomatosis

    Directory of Open Access Journals (Sweden)

    Khanna Dhanita

    2011-01-01

    Full Text Available Wegener′s granulomatosis (WG is a multisystem vasculitic disorder which can commonly afflict various components of the eye. Here we describe some unusual ocular manifestations of the disease in one patient. A young male with history of upper respiratory tract symptoms including epistaxis, nasal stuffiness and maxillary sinus pain presented with bilateral lacrimal gland abscess and ptosis. Lacrimal gland biopsy revealed granulomatous vasculitis. Lung cavities, positive cytoplasmic-antineutrophil cytoplasmic antibodies and high titers of serine proteinase-3 antibodies confirmed the diagnosis of WG. The patient developed dry eyes after a month of first presentation. There was no dryness of mouth, suggesting the absence of salivary gland involvement, and antinuclear antibodies as well as antibodies against Ro and La antigens classical of primary Sjogren′s syndrome were absent. Granulomatous vasculitis of lacrimal gland leading to abscess formation and dryness of eyes has not been described in WG and reflects the aggressive nature of inflammatory process in this disease.

  9. Successful treatment of granulomatosis with polyangiitis with hydropneumothorax using corticosteroids and immunosuppressant.

    Science.gov (United States)

    Shi, Xu-Hua; Zhang, Yong-Feng; Lu, Yue-Wu

    2017-06-01

    Pneumothorax and pleural effusion is a rare and serious complication of granulomatosis with polyangiitis (GPA). The present study reported a case with a history of sinusitis for 20 years, dry cough for three years and exacerbated purulent nasal discharge and recurrent skin ulcers for two years. The patient experienced sudden difficulty in breathing two months prior to presentation. Lung computed tomography (CT) showed multiple bilateral lung nodules and cavitary nodules as well as right hydropneumothorax. Paranasal sinus CT showed soft tissue infiltration. The cytoplasmic pattern of anti-neutrophil cytoplasmic antibody (c-ANCA) was positive and anti-proteinase 3 (PR3) antibodies, erythrocyte sedimentation rate and C-reactive protein were elevated. After pleural drainage and methylprednisolone pulse treatment, followed by cyclophosphamide and cyclosporine, the patient's symptoms were ameliorated, lungs were re-expanded, and c-ANCA, PR3 and inflammatory markers returned to normal levels.

  10. Granulomatosis with polyangiitis and facial palsy: Literature review and insight in the autoimmune pathogenesis.

    Science.gov (United States)

    Iannella, Giannicola; Greco, Antonio; Granata, Guido; Manno, Alessandra; Pasquariello, Benedetta; Angeletti, Diletta; Didona, Dario; Magliulo, Giuseppe

    2016-07-01

    Granulomatosis with polyangiitis (GPA) is an autoimmune systemic necrotizing small-vessel vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Oto-neurological manifestations of ANCA-associated vasculitis according to PR3-ANCA positivity and MPO-ANCA positivity are usually reported. Facial nerve palsy is usually reported during the clinical course of the disease but it might appear as the presenting sign of GPA. Necrotizing vasculitis of the facial nerve 'vasa nervorum' is nowadays the most widely accepted etiopathogenetic theory to explain facial damage in GPA patients. A central role for PR3-ANCA in the pathophysiology of vasculitis in GPA patients with oto-neurological manifestation is reported. GPA requires prompt, effective management of the acute and chronic manifestations. Once the diagnosis of GPA has been established, clinicians should devise an appropriate treatment strategy for each individual patient, based on current clinical evidence, treatment guidelines and recommendations. Copyright © 2016. Published by Elsevier B.V.

  11. Gingival hyperplasia being the first sign of Wegener’s granulomatosis

    Science.gov (United States)

    Aravena, Víctor; Beltrán, Víctor; Cantín, Mario; Fuentes, Ramón

    2014-01-01

    Wegener’s granulomatosis (GW) is a multisystemic granulomatous vasculitis that predominantly affects the airways and the kidneys, but may affect any organ. Otorhinolaryngological manifestations may be gingival swelling, oral ulcer or septal perforations that can cause saddle nose deformities, rhinitis, sinusitis and hearing loss. We report a case of WG that was first diagnosed on oral gingival mucosa. A 54-year old woman was referred to a specialized dentist because of consistent irritative buccal gingival hyperplasia that did not react to conservative and microbial treatment. The lesion was biopsied and the diagnosis was suggestive for WG. Patient was further referred to the Unit of Rheumatology and the diagnose of WG was confirmed and treated. This case emphasizes the importance to recognize the oral manifestation of WG to get proper medication as soon as possible and avoid serious systemic tissue damage. PMID:25232440

  12. Prolonged risk of specific malignancies following cyclophosphamide therapy among patients with granulomatosis with polyangiitis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Mellemkjaer, Lene; Voss, Anne

    2015-01-01

    OBJECTIVE: The long-term cancer risk for patients treated for granulomatosis with polyangiitis (GPA) is not well characterized. We assessed the risk of early and late-occurring cancers among 293 patients diagnosed with GPA from 1973 to 1999 and followed throughout 2010. METHODS: Cancer incidence......-melanoma skin cancers (NMSCs) and 11 were bladder carcinomas. A high occurrence of NMSC was observed from the second year of follow-up onwards, with a SIR of 7.0 (95% CI 2.3, 16) for cases diagnosed ≥20 years after GPA. The incidence of bladder cancer increased after 5-9, 10-14 and 15-19 years of follow...... was observed among patients exposed to cumulative CYC doses >36 g, while the only malignancy type observed in excess among those treated with lower CYC doses was NMSC. The cancer risk among CYC-naive patients was not significantly increased. CONCLUSION: GPA patients experience a greater than expected number...

  13. Relapsing granulomatosis with polyangiitis with severe lung and upper respiratory tract involvement successfully treated with rituximab.

    Science.gov (United States)

    Masiak, Anna; Zdrojewski, Zbigniew

    2017-01-01

    Granulomatosis with polyangiitis (GPA) is a chronic, relapsing, primary systemic small vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies. The disease is characterised by the involvement of various organs. The relapse rate rises from about 20% at 12 months to about 60% at 5 years. The combination of glucocorticoids and cyclophosphamide remains the standard therapy for patients with generalised GPA; nevertheless, some patients do not respond satisfactorily to this treatment. According to EULAR-EDTA recommendations for the management of ANCA-associated vasculitis, RTX should be considered for remission-induction of new-onset as well as major relapse of organ-threatening or life-threatening GPA. Here we present a 35-year-old patient with relapsing GPA successfully treated with RTX. We also highlight the infectious complications of immunosuppressive treatment.

  14. Eosinophilic Granulomatosis with Polyangiitis Presenting as Acute Polyneuropathy Mimicking Guillain-Barre Syndrome

    Science.gov (United States)

    Camara-Lemarroy, Carlos R.; Infante-Valenzuela, Adrian; Villareal-Montemayor, Hector J.; Soto-Rincon, Carlos A.; Davila-Olalde, Javier A.; Villareal-Velazquez, Hector J.

    2015-01-01

    Eosinophilic granulomatosis with polyangiitis (EGPA) is a small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) which commonly affects the peripheral nervous system. A 38-year-old female with a history of asthma presented with a 2-week history of bilateral lower extremity paresthesias that progressed to symmetric ascending paralysis. Nerve conduction studies could not rule out Guillain-Barre syndrome (GBS) and plasmapheresis was considered. Her blood work revealed marked eosinophilia (>50%), she had purpuric lesions in her legs, and a head magnetic resonance image showed evidence of pansinusitis. Coupled with a history of asthma we suspected EGPA-associated neuropathy and started steroid treatment. The patient showed rapid and significant improvement. ANCAs were later reported positive. ANCA-associated vasculitides present most often as mononeuritis multiplex, but they can mimic GBS and should always be considered in the differential diagnosis, since the treatment strategies for these conditions are radically different. PMID:26199772

  15. Eosinophilic Granulomatosis with Polyangiitis Presenting as Acute Polyneuropathy Mimicking Guillain-Barre Syndrome

    Directory of Open Access Journals (Sweden)

    Carlos R. Camara-Lemarroy

    2015-01-01

    Full Text Available Eosinophilic granulomatosis with polyangiitis (EGPA is a small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs which commonly affects the peripheral nervous system. A 38-year-old female with a history of asthma presented with a 2-week history of bilateral lower extremity paresthesias that progressed to symmetric ascending paralysis. Nerve conduction studies could not rule out Guillain-Barre syndrome (GBS and plasmapheresis was considered. Her blood work revealed marked eosinophilia (>50%, she had purpuric lesions in her legs, and a head magnetic resonance image showed evidence of pansinusitis. Coupled with a history of asthma we suspected EGPA-associated neuropathy and started steroid treatment. The patient showed rapid and significant improvement. ANCAs were later reported positive. ANCA-associated vasculitides present most often as mononeuritis multiplex, but they can mimic GBS and should always be considered in the differential diagnosis, since the treatment strategies for these conditions are radically different.

  16. Granulomatosis with polyangitis with mononeuritis multiplex-immunosuppressives playing a double-edged sword

    Directory of Open Access Journals (Sweden)

    Subhasis Mukherjee

    2014-01-01

    Full Text Available A 52-year-old female was diagnosed with rheumatoid arthritis and was on methotrexate and prednisolone. She developed fever, cough, hemoptysis, and cavitary lesion on chest skiagram. She was put on antitubercular therapy without any improvement, meanwhile she developed painful right foot drop. Clinicoradiology and C-ANCA study confirmed the diagnosis of granulomatosis with polyangitis (GPA. She was started on cyclophosphamide, corticosteroid, and co-trimoxazole. While her treatment was being continued she showed significant improvement of pulmonary manifestations. About 1 year later, there was reappearance of fever, cough, and radiological opacity with oropharyngeal candidiasis. She became very ill with disseminated intravascular coagulation (DIC-like features. Immunological markers were negative but bronchoalveolar lavage fluid study showed growth of Aspergillus spp. The patient was promptly put on intravenous voriconazole but unfortunately she succumbed to her illness.

  17. Altered Liver Proteoglycan/Glycosaminoglycan Structure as a Manifestation of Extracellular Matrix Remodeling upon BCG-induced Granulomatosis in Mice.

    Science.gov (United States)

    Kim, L B; Shkurupy, V A; Putyatina, A N

    2017-01-01

    Experimental BCG-induced granulomatosis in mice was used to study changes in the dynamics of individual liver proteoglycan components reflecting phasic extracellular matrix remodeling, determined by the host-parasite interaction and associated with granuloma development. In the early BCG-granulomatosis period, the increase in individual proteoglycan components promotes granuloma formation, providing conditions for mycobacteria adhesion to host cells, migration of phagocytic cells from circulation, and cell-cell interaction leading to granuloma development and fibrosis. Later, reduced reserve capacity of the extracellular matrix, development of interstitial fibrosis and granuloma fibrosis can lead to trophic shortage for cells within the granulomas, migration of macrophages out of them, and development of spontaneous necrosis and apoptosis typical of tuberculosis.

  18. Primary diffuse large B-cell lymphoma or lymphomatoid granulomatosis grade 3: a still-puzzling diagnosis in autopsy

    Directory of Open Access Journals (Sweden)

    Fernando Peixoto Ferraz de Campos

    2013-12-01

    Full Text Available Primary lung lymphoma is a rare entity accounting for approximately 0.3% of all primary neoplasia of the lung and includes diffuse large B-cell lymphoma (DLBL and lymphomatoid granulomatosis (LYG. Considering that clinical features may be similar, whereas epidemiology, morphology, and radiological features are different, the authors report a case of a middle-aged man who presented multiple pulmonary nodules in the lower lobes and ground-glass opacities scattered bilaterally on computed tomography. Clinically, he presented a consumptive syndrome with respiratory failure and pleurisy, which progressed until death. The autopsy findings were consistent with lymphomatoid granulomatosis (LYG grade 3/ diffuse large B-cell lymphoma (DLBL. The authors call attention to the difficulty of establishing an accurate diagnosis, mainly when the demonstration of EBV-infected atypical B-cells fails.

  19. Case Report

    African Journals Online (AJOL)

    Memiş L. An unusual renal presentation of Wegener's granulomatosis. Tuberk Toraks. 2003;51(2):193-6. 8. Banerjee A, McKane W, Thiru S, Farrington. K. Wegener's granulomatosis presenting as acute suppurative interstitial nephritis. J Clin Pathol. 2001. Oct;54(10):787-9. 9. Lee L, Saunders J, Zhou XJ, Maalouf NM. Acute.

  20. 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.

  1. Computerized adaptive control weld skate with CCTV weld guidance project

    Science.gov (United States)

    Wall, W. A.

    1976-01-01

    This report summarizes progress of the automatic computerized weld skate development portion of the Computerized Weld Skate with Closed Circuit Television (CCTV) Arc Guidance Project. The main goal of the project is to develop an automatic welding skate demonstration model equipped with CCTV weld guidance. The three main goals of the overall project are to: (1) develop a demonstration model computerized weld skate system, (2) develop a demonstration model automatic CCTV guidance system, and (3) integrate the two systems into a demonstration model of computerized weld skate with CCTV weld guidance for welding contoured parts.

  2. Visualization in the age of computerization

    CERN Document Server

    Carusi, Annamaria; Webmoor, Timothy; Woolgar, Steve

    2014-01-01

    Digitalization and computerization are now pervasive in science. This has deep consequences for our understanding of scientific knowledge and of the scientific process, and challenges longstanding assumptions and traditional frameworks of thinking of scientific knowledge. Digital media and computational processes challenge our conception of the way in which perception and cognition work in science, of the objectivity of science, and the nature of scientific objects. They bring about new relationships between science, art and other visual media, and new ways of practicing science and organizing

  3. Computerized documentation systems: blessings or curse?

    Science.gov (United States)

    Vlasses, F R

    1993-01-01

    This article considers the possibility that computerized documentation systems will negatively impact knowledge development in nursing. Ideas from three vantage points is presented. First, systems are being developed from theoretical frameworks that are not necessarily grounded in nursing, and these systems, in turn, influence the nurses's ability to process and conceptualize information. Second, computer systems may support the retrieval of empirical data to the elimination of other types of data necessary to the development of nursing knowledge. Third, computers may decrease opportunities for collegial dialogue. These factors together create an atmosphere of "technologic determinism" (Robinson & Robinson, 1990), which can inhibit the development of new ideas in nursing.

  4. Drug administration error related to computerized prescribing.

    Science.gov (United States)

    Le Garlantezec, P; Aupée, O; Alméras, D; Lefeuvre, L; Souleau, B; Sgarioto, A; Bohand, X

    2010-12-01

    One of the main reasons for the implementation of computer-based prescribing was to reduce medication errors. However, the risk has not fallen to zero and new kinds of errors have been detected. the following case relates one of these medication errors involving a preparation of vincristine. This antineoplastic drug was injected to a patient via a subcutaneous route of administration instead of an intravenous bolus injection. consequently, a cutaneous erythema appeared. This incident resulted from an error in the programming of the administration route of the protocol operated by a pharmacist and a physician. The pharmacist, who was responsible for the validation of the computerized medical order and then for the compounding and the dispensing of the drug, did not detect the error. this case highlights the need of improved and irreproachable therapeutic protocols. Recorded in a database, they must be validated pharmaceutically and medicinally to secure computer-based prescribing, drug handling, dispensing, and administering of the antineoplastic drugs. Even if the pharmaceutical analysis of prescriptions is made easier with computerization, we encourage the training of nurses and the evaluation of their knowledge as well as the necessity for pharmacists to learn to detect new kinds of errors and to verify periodically protocols.

  5. Pulmonary foreign body granulomatosis in a chronic user of powder cocaine.

    Science.gov (United States)

    Khurana, Shruti; Chhoda, Ankit; Sahay, Sandeep; Pathania, Priyanka

    2017-08-03

    We describe the case of a 33-year-old man, a chronic user of powder cocaine, who presented with dyspnea, fever, night sweats, and significant weight loss. Chest HRCT revealed centrilobular nodules, giving an initial impression of miliary tuberculosis. Therefore, he was started on an empirical, four-drug antituberculosis treatment regimen. Four weeks later, despite the tuberculosis treatment, he continued to have the same symptoms. We then performed transbronchial lung biopsy. Histopathological analysis of the biopsy sample revealed birefringent foreign body granuloma. A corroborative history of cocaine snorting, the presence of centrilobular nodules, and the foreign body-related histopathological findings led to a diagnosis of pulmonary foreign body granulomatosis. This report underscores the fact that pulmonary foreign body granulomatosis should be included in the differential diagnosis of clinical profiles resembling tuberculosis. RESUMO Descrevemos o caso de um homem de 33 anos de idade, usuário crônico de cocaína em pó, que apresentava dispneia, febre, sudorese noturna e perda de peso significativa. A TCAR de tórax revelou nódulos centrolobulares, dando uma impressão inicial de tuberculose miliar. Por isso, o paciente passou a receber tratamento empírico com quatro tuberculostáticos. Quatro semanas depois, apesar do tratamento antituberculose, o paciente continuou a apresentar os mesmos sintomas. Foi então realizada a biópsia pulmonar transbrônquica. A análise histopatológica da amostra obtida revelou granuloma de corpo estranho birrefringente. A história de uso de cocaína por inalação, a presença de nódulos centrolobulares e os achados histopatológicos de corpos estranhos confirmaram o diagnóstico de granulomatose pulmonar de corpo estranho. Este relato destaca o fato de que a granulomatose pulmonar de corpo estranho deve ser incluída no diagnóstico diferencial de perfis clínicos que se assemelham a tuberculose.

  6. Heart conduction system defects and sustained ventricular tachycardia complications in a patient with granulomatosis with polyangiitis. A case report and literature review.

    Science.gov (United States)

    Santos, Laryssa Passos Sarmento; Bomfim, Victor Guerreiro; Bezerra, Camila Fagundes; Costa, Natália Vieira; Carvalho, Rafael Barreto Paes de; Carvalho, Ricardo Sobral de; Passos, Rogério da Hora; Boaventura, Olivia Carla Bomfim; Gobatto, André Luiz Nunes

    2017-01-01

    Granulomatosis with polyangiitis is a rare systemic inflammatory disorder characterized by vasculitis of the small arteries, the arterioles and the capillaries together with necrotizing granulomatous lesions. This case reports on a young female patient, previously diagnosed with granulomatosis with polyangiitis, who was admitted to the intensive care unit with seizures and hemodynamic instability due to a complete atrioventricular heart block. The event was associated with multiple episodes of sustained ventricular tachycardia without any structural heart changes or electrolyte disturbances. In the intensive care unit, the patient was fitted with a provisory pacemaker, followed by immunosuppression with corticosteroids and immunobiological therapy, resulting in a total hemodynamic improvement. Severe conduction disorders in patients presenting granulomatosis with polyangiitis are rare but can contribute to increased morbidity. Early detection and specific intervention can prevent unfavorable outcomes, specifically in the intensive care unit.

  7. Participatory design for computerization of clinical practice guidelines

    DEFF Research Database (Denmark)

    Lyng, Karen Marie; Pedersen, B. S.

    2011-01-01

    There have been made many attempts on computerization of clinical practice guidelines (CPGs), none have, however achieved any general application in clinical work practice. The objective of this paper is: (1) to raise awareness about the impact the design method used for computerization of CPGs...

  8. Computerization of academic libraries: a case study of Hezekiah ...

    African Journals Online (AJOL)

    The paper discussed the computerization of the Hezekiah Oluwasanmi Library, Obafemi Awolowo University, Ile-Ife. The history, possible design options and the level of computerization were given. The Library's network architecture and the chosen design option used were explained with reasons. The state of the art of the ...

  9. FEASIBILITY OF COMPUTERIZED PSYCHOLOGICAL-TESTING WITH PSYCHIATRIC OUTPATIENTS

    NARCIS (Netherlands)

    SPINHOVEN, P; LABBE, MR; ROMBOUTS, R

    The feasibility of computerized psychological testing was investigated in a sample of 452 consecutive psychiatric outpatients. Forty-six percent of the solicited patients agreed to participate in the computerized assessment. Tested patients were significantly younger and better educated than those

  10. Developing of a Computerized Brain Diagnosing System for Case ...

    African Journals Online (AJOL)

    The main purpose of this project is to design a computerized brain diagnosing system that would be used in carrying out the daily diagnosing activity in the clinic. The developed computerized system has numerous advantages over manual operation which is very tedious and time consuming. As part of the research method ...

  11. Vasculitis Pregnancy Registry

    Science.gov (United States)

    2017-04-27

    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

  12. Granulomatosis with Polyangiitis Presenting as Pauci-Immune Crescentic Glomerulonephritis in Pregnancy

    Directory of Open Access Journals (Sweden)

    Ryan Kunjal

    2016-01-01

    Full Text Available Antineutrophil cytoplasmic antibody (ANCA associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophilia. During her course she also developed one episode of small volume hemoptysis with right upper lobe infiltrates on CT Chest. There were no cutaneous manifestations of vasculitis or upper respiratory symptoms. Renal biopsy revealed a pauci-immune crescentic glomerulonephritis (PICGN. The diagnosis was consistent with granulomatosis with polyangiitis (GPA. Management initially comprised teratogen sparing agents; steroids, intravenous immunoglobulin; and plasma exchange. The response was suboptimal and she became dependent on daily renal replacement therapy. Ultimately the pregnancy was terminated allowing for traditional treatment approaches with dramatic effect. This is the first case of GPA presenting as PICGN in pregnancy and highlights the challenges of its management.

  13. Impact of pre-existing co-morbidities on mortality in granulomatosis with polyangiitis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Ahlström, Magnus Glindvad; Lindhardsen, Jesper

    2016-01-01

    OBJECTIVE: To assess the impact of pre-existing co-morbidities on mortality among patients affected by granulomatosis with polyangiitis (GPA). METHODS: By means of the Danish National Hospital Register, we identified a cohort of patients hospitalized for GPA during 1994-2010 (n = 308). The burden...... throughout 2010. Cox regression analyses were used to calculate mortality rate ratios (MRRs). RESULTS: The median duration of follow-up in the GPA cohort was 5.8 years (interquartile range 2.3-10.0). Compared with their matched population controls, the MRR for patients presenting with a CCI score of 0 (n...... found to have significantly higher mortality than GPA patients with a CCI score of 0 during years 0-2 [adjusted MRR 3.4 (95% CI 1.6, 7.0)] but not after >2 years of follow-up [adjusted MRR 1.3 (95% CI 0.7, 2.6)]. CONCLUSION: During early follow-up periods, the mortality among GPA patients with pre...

  14. Immune deposits in cutaneous lesions of Wegener′s granulomatosis: Predictor of an active disease

    Directory of Open Access Journals (Sweden)

    Seema Chhabra

    2011-01-01

    Full Text Available A retrospective analysis was conducted of eight cases of Wegener′s granulomatosis (WG, who presented with cutaneous lesions. The clinical, immunopathologic and histopathologic features of the cutaneous lesions were reviewed. Antineutrophil cytoplasmic antibody (ANCA status of the patients was established. When possible, a comparison of immunofluorescence findings of skin biopsies was made with those of renal biopsies taken at the same time. In all except one, systemic and cutaneous disease developed concurrently. On histopathology, leukocytoclastic vasculitis was noted in five patients and features of lupus erythematosus and pyoderma gangrenosum in one case each. Four patients showed immunoglobulin deposits in subepidermal blood vessel walls, while one patient showed granular immune deposits at dermo-epidermal junction only. Immunoglobulin G was the most common immunoreactant detected. C-ANCA/proteinase 3 (PR3-ANCA was positive in six patients, P-ANCA/myeloperoxidase (MPO-ANCA in one patient, while one patient did not show ANCA positivity on indirect immunofluorescence. All four renal biopsies showed pauci-immune glomerulonephritis, irrespective of the presence (n=3 or absence (n=1 of immune deposits in the skin biopsy. Skin manifestations are encountered in nearly half of the patients with WG, thus it is important to be familiar with cutaneous histopathologic as well as immunofluorescence findings in WG patients.

  15. Cutaneous manifestations of pediatric granulomatosis with polyangiitis: a clinicopathologic and immunopathologic analysis.

    Science.gov (United States)

    Wright, Adam C; Gibson, Lawrence E; Davis, Dawn Marie R

    2015-05-01

    Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis associated with variable cutaneous manifestations and histopathologic findings. It is less frequent in children than adults and is often positive for cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or proteinase 3-ANCA. We sought to better define and correlate the clinical, histopathologic, and immunopathologic characteristics of cutaneous GPA in pediatric patients. We retrospectively reviewed clinical records and cutaneous histopathologic specimens of patients 17 years or younger with cutaneous manifestations of GPA who were seen at our institution from 1990 to 2013. Of the 52 patients identified with GPA, cutaneous involvement occurred in 36.5% and was the initial manifestation of disease in 7.7%. Of the 19 patients with cutaneous involvement, 26.3% developed acneiform and folliculitis-like papules; 84.2% were cytoplasmic ANCA positive; and 78.9% were proteinase 3-ANCA positive. Histopathologic features included leukocytoclastic vasculitis, granulomatous inflammation, acneiform and perifollicular inflammation, granulomatous vasculitis, and palisading granulomas. Our study was limited because of its retrospective design. Pediatric patients with cutaneous GPA most commonly have palpable purpura, leukocytoclastic vasculitis, and positive cytoplasmic ANCA or proteinase 3-ANCA serologic results. Cutaneous manifestations and histopathologic findings vary, but acneiform lesions may be a cutaneous manifestation of the disease unique to this age group. Copyright © 2015 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  16. Concomitant Granulomatosis with Polyangiitis and C3 Glomerulonephritis Causing Renal Failure.

    Science.gov (United States)

    Chaudhuri, Aadel A; Davis, Jason T

    2016-02-05

    A 75-year-old male with no prior history of vasculitis or renal deficiency presented with a history of 36 hours of anterior epistaxis, one month of worsening shortness of breath, severe anemia requiring transfusion, thrombocytopenia, coagulopathy with INR 4.9, and renal failure requiring hemodialysis. A peripheral blood smear revealed no evidence of microangiopathic hemolytic anemia. We performed an autoimmune workup, which revealed high levels of serum serine protease 3 antibody (C-ANCA), elevated rheumatoid factor, low serum C3, and normal levels of serum C4. We performed a renal biopsy and then performed light microscopy, immunofluorescence, and electron microscopy on the resulting samples. This revealed that approximately half of the sampled glomeruli were globally sclerotic, consistent with severe renal disease. Among the non-sclerotic glomeruli, several demonstrated diffuse granular mesangial staining for C3, while other glomeruli had small crescents, consistent with a mixed picture of C3 glomerulonephritis (C3GN) and crescentic glomerulonephritis. The patient responded well to treatment with cyclophosphamide and prednisone, with a resolution of his acute issues, significant improvement in kidney function, and was eventually weaned from routine hemodialysis. In summary, this is a unique case of a patient presenting with features of both granulomatosis with polyangiitis (GPA) and C3GN.

  17. Granulomatosis With Polyangiitis in Otolaryngologist Practice: A Review of Current Knowledge.

    Science.gov (United States)

    Wojciechowska, Joanna; Krajewski, Wojciech; Krajewski, Piotr; Kręcicki, Tomasz

    2016-03-01

    Granulomatosis with polyangiitis (GPA) is an idiopathic vasculitis of medium and small arteries, characterized by necrotizing granulomatous inflammation. GPA typically affects upper and lower respiratory tract with coexisting glomerulonephritis. This disease is generally characterized by antineutrophil cytoplasm antibodies (ANCA), nevertheless, there are rare cases with negative ANCA. GPA affects people at any age, with predominance of the sixth and seventh decade of life. In 80%-95% of the patients the first symptoms of GPA are otorhinolaryngological manifestations of head and neck including nose/sinuses, ears, eyes, larynx/trachea, oral cavity, and salivary glands. Diagnosis of GPA is based on Criteria of the American College of Rheumatology. In clinical practice diagnosis, the presence of distinctive ANCA antibodies and biopsy of affected organ are crucial. GPA must be differentiated from neoplastic, infectious or inflammatory ulcerative lesions of the head and neck. The standard treatment procedure is divided into two essential phases, induction and maintenance. The induction phase is based on combination of systemic corticosteroid and immunosuppressant therapy, whereas the maintenance phase comprises corticosteroids and azathioprine/methotrexate supplementation. Surgical treatment ought to be considered for patients who are not responding to pharmacotherapy.

  18. Serum biomarkers in patients with relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss.

    Directory of Open Access Journals (Sweden)

    Christian Dejaco

    Full Text Available Previous studies suggest a role for eotaxin-3, TARC/CCL17 and IgG4 in newly-diagnosed patients with eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss with highly active disease. The role of these biomarkers in relapsing disease is unclear.Serum levels of TARC/CCL17, eotaxin-3, IgG4, and IgG4/IgG ratio were determined in serum samples from a longitudinal cohort of patients with EGPA (105 visits of 25 patients. Epidemiological, clinical and laboratory data were available for all visits.At the first visit, 80% of patients were using glucocorticoids and 68% additional immunosuppressive drugs. Disease flares were seen at 18 visits. The median BVAS and BVAS/WG scores at time of relapse were 4 and 2, respectively. None of the biomarkers tested were useful to discriminate between active disease and remission. Patients treated with prednisone had lower eotaxin-3 and eosinophil levels compared to patients not taking glucocorticoids irrespective of disease activity. Use of immunosuppressive agents was not associated with biomarker levels.Serum levels of TARC/CCL17, eotaxin-3, IgG4, and IgG4/IgG ratio do not clearly differentiate active and inactive disease in established EGPA. Defining biomarkers in EGPA remains a challenge especially during times of glucocorticoid use.

  19. A case of exorbitism in association with Wegener′s granulomatosis with renal involvement

    Directory of Open Access Journals (Sweden)

    S Beji

    2012-01-01

    Full Text Available Wegener′s granulomatosis (WG is a necrotizing granulomatous vasculitis invol-ving the nose, paranasal sinuses, lungs, and kidneys. Ocular involvement can occur in about 50% of cases. There are very few reports of WG with orbital inflammation and exorbitism. We report a case of a female patient who presented with exorbitism related to orbital inflammation secondary to WG, with renal involvement. A 29-year-old woman with a previous history of recurrent pan-sinusitis presented with bilateral exophthalmos and renal failure with rapidly progressive glo-merulonephritis. Computed tomography showed extensive bilateral soft tissue in the retro-orbital area. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies and renal biopsy revealed pauci immune crescentic glomerulonephritis. The patient was treated with corticosteroids and pulses of cyclophosphamide followed by azathioprine and trimethoprim-sulfamethoxazole. After a follow-up of 10 months, the renal outcome was favorable with improvement of renal function but there was persistence of exorbitism and loss of visual function. Our case suggests that WG should be considered in the differential diagnosis of persistent bila-teral exophthalmos. Prompt recognition of this early manifestation is important for the institution of early treatment.

  20. Association between orofacial granulomatosis and Crohn's disease in children: systematic review.

    Science.gov (United States)

    Lazzerini, Marzia; Bramuzzo, Matteo; Ventura, Alessandro

    2014-06-21

    To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn's disease. We conducted a systematic review according to the PRISMA guidelines. We searched Medline, LILACS, Virtual Health Library, and Web of Knowledge in September 2013 for cases of OFG in the pediatric age range (Crohn's disease. In about half (51.4%) of the cases the onset of OFG anticipated the diagnosis of Crohn's disease, with a mean time between the two diagnoses of 13.1 ± 11.6 mo (range: 3-36 mo). Overall, 21/173 (12.1%) of the children with OFG had perianal disease, while 11/173 (6.4%) had a family history of Crohn's disease. Both perianal disease and a family history of Crohn's disease were significantly associated with a higher risk of Crohn's disease diagnosis in children with OFG [relative risk (RR) = 3.10, 95% confidence interval (CI): 2.46-3.90; RR = 2.74, 95%CI: 2.24-3.36, P Crohn's disease in children with OFG suggests that OFG may be a subtype of Crohn's disease.

  1. DOCK 8 Deficiency, EBV+ Lymphomatoid Granulomatosis, and Intrafamilial Variation in Presentation.

    Science.gov (United States)

    Dimitriades, Victoria R; Devlin, Vincent; Pittaluga, Stefania; Su, Helen C; Holland, Steven M; Wilson, Wyndham; Dunleavy, Kieron; Shah, Nirali N; Freeman, Alexandra F

    2017-01-01

    Dedicator of cytokinesis 8 (DOCK8) deficiency is an autosomal recessive, combined immunodeficiency within the spectrum of hyper-IgE syndromes. Epstein-Barr virus-positive lymphomatoid granulomatosis (LYG) (EBV + LYG) is a rare diagnosis and a previously unreported presentation of DOCK8 deficiency. A 10-year-old girl was initially evaluated for mild eczema and recurrent sinopulmonary infections. She had normal immunoglobulins with elevated IgE, poor polysaccharide response with low switched memory B cells, low CD4 count, and normal mitogen and antigen responses. Despite clinical improvement following immunoglobulin replacement, a prolonged cough prompted a CT scan, which showed nodules. Biopsy identified a Grade 2 EBV + LYG. Due to an inadequate response with chemotherapy, further workup for primary immunodeficiency was performed. With her symptoms of eczema and IgE elevation, along with her brother's history of recurrent sinopulmonary infections and warts, targeted sequencing of DOCK8 was performed revealing compound heterozygous mutations for the two siblings. Both patients were successfully transplanted with resolution of the LYG and warts, respectively. This is the first reported case of LYG in DOCK8 deficiency. The EBV-driven lymphoproliferative disease along with the infection history in the brother led to the diagnosis of DOCK8 deficiency and curative hematopoietic stem cell transplants.

  2. Efficiency of using rituximab in a patient with generalized granulomatosis with polyangiitis: A case report

    Directory of Open Access Journals (Sweden)

    Gulazyk Malikovna Koilubaeva

    2014-01-01

    Full Text Available Systemic vasculitides (SVs are characterized by inflammation of the blood vessels wall; the spectrum of their clinical manifestations depends on the type, extent, and location of affected vessels and the activity of systemic inflammation. The etiology of most primary SVs is unknown. Antineutrophil cytoplasmic antibodies (ANCAs are implicated in its pathogenesis. The presence of ANCAa in patients' serum and the correlation of their level with the severity of clinical manifestations served as a basis for identifying a subgroup of systemic necrotizing vasculitides associated with ANCA synthesis: granulomatosis with polyangiitis (GPA, microscopic polyangiitis, and Churg – Strauss syndrome. GPA is characterized by systemic granulomatous necrotizing vasculitis involving the small vessels of the upper respiratory tract, lung, and kidney.The paper describes a case of difficult diagnosis and successful rituximab (RTM treatment of generalized GPA in a 45-year-old female patients. The disease occurred with local damage to the upper respiratory tract, granulomatous inflammation of the pulmonary vessels to form multiple infiltrates with lung tissue destruction elements and necrotizing glomerulonephritis. Despite intensive immunosuppressive treatment, there was a rapid GPA progression with the further development of respiratory failure, which had been induced by stenotic laryngitis subglottica leading to tracheostoma. Damage to the organ of vision could lead to severe complications, including amaurosis. RMT was shown to be effective in treating generalized GPA with a poor prognosis.

  3. Computerized color perimetry in multiple sclerosis.

    Science.gov (United States)

    Accornero, N; Rinalduzzi, S; Capozza, M; Millefiorini, E; Filligoi, G C; Capitanio, L

    1998-04-01

    Color visual field analysis has proven highly sensitive for early visual impairments diagnosis in MS, yet it has never attained widespread popularity usually because the procedure is difficult to standardize, the devices are costly, and the test is fatiguing. We propose a computerized procedure running on standard PC, cost effective, clonable, and easy handled. Two hundred and sixty-four colored patches subtending 1 degree angle vision, with selected hues and low saturation levels are sequentially and randomly displayed on gray equiluminous background of the PC screen subtending 24 degrees x 40 degrees angle of vision. The subject is requested to press a switch at the perception of the stimulus. The output provides colored maps with quantitative information. Comparison between normals and a selected population of MS patients with no actual luminance visual field defects, showed high statistical difference.

  4. Computerized tomographic evaluation of cerebral cysticercosis

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Bo Young; Lee, Mi Sook; Jeon, Doo Sung; Kim, Hong Soo; Rhee, Hak Song [Precbyterian Medical Center, Chonju (Korea, Republic of)

    1988-08-15

    Cerebral cysticercosis, unfortunately frequent in Korea, is a parastic disease in which man serve as the intermediate host of taenia solium. The larvae have a predilection for the central nervous system and can cause a variety of neurologic symptoms. The authors reviewed 19 cases of surgically proven cerebral cysticercosis and following results were obtained. 1. The most frequent age distribution was 5th and 6th decade and male to female ratio was 14:5. 2. The most frevalent involving site was cerebral parenchyme and following by ventricles. 3. Clinical manifestations were symtom and sign of increased ICP, seizure and focal neurological dificit. 4. It was assumed that computerized tomography was the procedure of choice for the diagnosis of these parasitic brain disease.

  5. Computerization of a telescope at secondary education

    Science.gov (United States)

    García Santiago, A.; Martos Jumillas, J.

    2017-03-01

    The work we are presenting in this paper is the computerization of a refractor telescope on an EQ3 type equatorial mount through Arduino. The control of the mount is done via three different interfaces: Stellarium, an Android interface for mobile phones and a second interface for PC made with Processing. The aforementioned work was done by the authors with a double purpose: presenting the interest in astronomy in the Mathematics department, and the development of applications within the subject of Technology in 4th ESO. So, it is a collaborative project between both departments. Except for the telescope and the mount, all the resources we have used can be found in any high school: free software (Guadalinex v9), App Inventor and Processing.The project was carried out under the principle of reducing all possible costs given the economic possibilities of the institution.

  6. A COMPUTERIZED OPERATOR SUPPORT SYSTEM PROTOTYPE

    Energy Technology Data Exchange (ETDEWEB)

    Thomas A. Ulrich; Roger Lew; Ronald L. Boring; Ken Thomas

    2015-03-01

    A computerized operator support system (COSS) is proposed for use in nuclear power plants to assist control room operators in addressing time-critical plant upsets. A COSS is a collection of technologies to assist operators in monitoring overall plant performance and making timely, informed decisions on appropriate control actions for the projected plant condition. A prototype COSS was developed in order to demonstrate the concept and provide a test bed for further research. The prototype is based on four underlying elements consisting of a digital alarm system, computer-based procedures, piping and instrumentation diagram system representations, and a recommender module for mitigation actions. The initial version of the prototype is now operational at the Idaho National Laboratory using the Human System Simulation Laboratory.

  7. Termination Criteria for Computerized Classification Testing

    Directory of Open Access Journals (Sweden)

    Nathan A. Thompson

    2011-02-01

    Full Text Available Computerized classification testing (CCT is an approach to designing tests with intelligent algorithms, similar to adaptive testing, but specifically designed for the purpose of classifying examinees into categories such as - pass- and - fail.- Like adaptive testing for point estimation of ability, the key component is the termination criterion, namely the algorithm that decides whether to classify the examinee and end the test or to continue and administer another item. This paper applies a newly suggested termination criterion, the generalized likelihood ratio (GLR, to CCT. It also explores the role of the indifference region in the specification of likelihood-ratio based termination criteria, comparing the GLR to the sequential probability ratio test. Results from simulation studies suggest that the GLR is always at least as efficient as existing methods.

  8. Economic Evaluation of Computerized Structural Analysis

    Science.gov (United States)

    Fortin, P. E.

    1985-01-01

    This completed effort involved a technical and economic study of the capabilities of computer programs in the area of structural analysis. The applicability of the programs to NASA projects and to other users was studied. The applications in other industries was explored including both research and development and applied areas. The costs of several alternative analysis programs were compared. A literature search covered applicable technical literature including journals, trade publications and books. In addition to the literature search, several commercial companies that have developed computerized structural analysis programs were contacted and their technical brochures reviewed. These programs include SDRC I-DEAS, MSC/NASTRAN, SCADA, SUPERSAP, NISA/DISPLAY, STAAD-III, MICAS, GTSTRUDL, and STARS. These programs were briefly reviewed as applicable to NASA projects.

  9. MRI features of Wegener's granuloma in the head. Magnetresonanztomographische Kennzeichen der Wegenerschen Granulomatose im Kopfbereich

    Energy Technology Data Exchange (ETDEWEB)

    Asmus, R.; Muhle, C.; Koltze, H.; Spielmann, R.P. (Kiel Univ., Klinik fuer Radiologische Diagnostik (Germany)); Gross, W.L. (Medizinische Univ. Luebeck, Poliklinik fuer Rheumatologie (Germany) Rheumaklinik Bad Bramstedt GmbH (Germany)); Duncker, G.; Noelle, B. (Kiel Univ., Klinik fuer Ophthalmologie (Germany)); Beigel, A. (Kiel Univ., Hals-, Nasen- und Ohrenklinik (Germany))

    1992-07-01

    MRI of the head was performed in 25 patients suffering from Wegener's granuloma. 23 patients showed evidence of mucosal thickening in the paranasal sinuses, the middle and inner ears and in the mastoid cells; these were characterised by low signal intensity of T[sub 1]-weighted and high signal intensity of T[sub 2]-weighted images. In 10 patients there were granulomas in the paranasal sinuses and in the orbits which showed low signal intensity of both T[sub 1]-weighted and T[sub 2]-weighted images. In 4 patients, additional images were obtained after the intravenous injection of Gd-DTPA. In 2 patients this resulted in non-homogeneous contrast accumulation in the granuloma. In 7 patients there were signal changes in the brain which were typical of infarcts. The complete extent of bone destruction in the facial skeleton was visible only by CT. (orig.).

  10. Successful Use of Plasma Exchange in the Treatment of Corticosteroid-Refractory Eosinophilic Granulomatosis with Polyangiitis Associated with Gastrointestinal Manifestations

    Directory of Open Access Journals (Sweden)

    Kohei Tsujimoto

    2016-01-01

    Full Text Available We describe the case of a 33-year-old woman having corticosteroid-refractory eosinophilic granulomatosis with polyangiitis (EGPA who presented with abdominal pain and responded dramatically to plasma exchange. Eosinophilia, asthma history, neuropathy, pulmonary infiltrates, and paranasal sinus abnormalities confirmed the diagnosis of EGPA. Treatment was initiated with 1 g/day of methylprednisolone pulse therapy for 3 days followed by 60 mg/day of intravenous prednisolone without relieving abdominal pain. Then, plasma exchange was performed thrice. Abdominal pain disappeared after the first plasma exchange. Indication of plasma exchange for EGPA remains controversial; however, it may represent a valid option in cases with gastrointestinal involvement.

  11. The new WegenerNet climate station network web portal - A gateway to over 10 years of high-resolution precipitation data

    Science.gov (United States)

    Fuchsberger, Jürgen; Kirchengast, Gottfried; Bichler, Christoph; Kabas, Thomas; Lenz, Gunther; Leuprecht, Armin

    2017-04-01

    The Feldbach region in southeast Austria, characteristic for experiencing a rich variety of weather and climate patterns, has been selected as the focus area for a pioneering weather and climate observation network at very high resolution: The WegenerNet comprises 153 meteorological stations measuring temperature, humidity, precipitation, and other parameters, in a tightly spaced grid within an area of about 20 km × 15 km centered near the city of Feldbach (46.93°N, 15.90°E). With its stations about every 2 km2, each with 5-min time sampling, the network provides regular measurements since January 2007. Detailed information is available in the recent description by Kirchengast et al. (2014) and via www.wegcenter.at/wegenernet. As a smaller "sister network" of the WegenerNet Feldbach region, the WegenerNet Johnsbachtal consists of eleven meteorological stations (complemented by one hydrographic station at the Johnsbach creek), measuring temperature, humidity, precipitation, radiation, wind, and other parameters in an alpine setting at altitudes ranging from below 700 m to over 2100 m. Data are available partly since 2007, partly since more recent dates and have a temporal resolution of 10 minutes. The networks are set to serve as a long-term monitoring and validation facility for weather and climate research and applications. Uses include validation of nonhydrostatic models operated at 1-km-scale resolution and of statistical downscaling techniques (in particular for precipitation), validation of radar and satellite data, study of orography-climate relationships, and many others. Quality-controlled station time series and gridded field data (spacing 200 m × 200 m) are available in near-real time (data latency less than 1-2 h) for visualization and download via a data portal (www.wegenernet.org). This data portal has been undergoing a complete renewal over the last year, and now serves as a modern gateway to the WegenerNet's more than 10 years of high

  12. Generation of high-resolution wind fields from the dense meteorological station network WegenerNet in South-Eastern Austria

    Science.gov (United States)

    Schlager, Christoph; Kirchengast, Gottfried; Fuchsberger, Jürgen

    2016-04-01

    To investigate weather and climate on a local scale as well as for evaluating regional climate models (RCMs) the Wegener Center at the University of Graz established the long-term field experiment WegenerNet Feldbach region, a dense grid of 153 meteorological stations. The observations of these stations are managed by an automatic WegenerNet Processing system. This system includes a quality check of collected observations and a Data Product Generator (DPG), among other subsystems. Products already implemented in the DPG are gridded weather and climate products, generated from the main parameters temperature, precipitation and relative humidity (Kirchengast et. al., Bull. Amer. Meteor. Soc., 95, 227-242, 2014). Missing elements are gridded wind fields from wind observations. Wind is considered as one of the most difficult meteorological variables to model and depends on many different parameters such as topography and surface roughness. Therefore a simple interpolation can only be performed in case of uniform characteristics of landscape. The presentation introduces our method of generation of wind fields from near real-time observations of the WegenerNet. Purpose of this work is to provide a database with 3D wind fields in a high spatial and time resolution as addition to the existing products, for evaluating convection permitting climate models as well as investigating weather and climate on a local scale. Core of the application is the diagnostic California Meteorological Model (CALMET). This model computes 3D wind fields based on meteorological observational data, a digital elevation model and land use categories. The application generates the required input files from meteorological stations of the WegenerNet Feldbach region and triggers the start of the CALMET model with these input files. In a next step the modeled wind fields are stored automatically every 30 minutes with a spatial resolution of 100 x 100 m in the WegenerNet database. To verify the

  13. Research on accounting transition from computerization to informationization

    Directory of Open Access Journals (Sweden)

    Shu Chen

    2017-11-01

    Full Text Available The application for computer technology, digitalization technology and network technology in the accounting field has promoted the development of accounting informationization. Accounting informationization is a product integrated with traditional accounting theory and modern information technology, which is an inevitable trend of continuous development of modern accounting. This paper discusses the basic concepts and characteristics of accounting computerization and informationization based on the normative research method and literature data method, analyzes the feasibility of accounting transition from computerization to informationization, and finally puts forward the specific approaches and ultimate goals of accounting transition from computerization to informationization.

  14. Pronounced Risk of Fractures among Elderly Men Affected by Granulomatosis with Polyangiitis.

    Science.gov (United States)

    Faurschou, Mikkel; Baslund, Bo; Obel, Niels

    2015-09-01

    It is unknown whether patients affected by granulomatosis with polyangiitis (GPA) are at increased risk of fractures, and whether the fracture risk in GPA varies with age and sex. The aim of the present study was to compare the fracture risk among patients with GPA with that among age- and sex-matched population controls. We established a monocentric cohort of patients treated for GPA at a Danish tertiary care center from 1995 to 2010 (n = 159) and a register-derived GPA cohort identified from the Danish National Hospital Register (n = 402). Each patient was matched with 7 population controls. The occurrence of fractures among patients was compared with that among controls by calculation of incidence rate ratios (IRR). In the monocentric cohort, an increased fracture risk was observed among men aged ≥ 55 years at the time of first hospitalization for GPA (IRR 3.5, 95% CI 1.6-7.6), but not among men < 55 years (IRR 0.3, 95% CI 0.04-2.1) or women (IRR women ≥ 55 yrs: 1.0, 95% CI 0.4-2.7 and IRR for women < 55 yrs: 0.7, 95% CI 0.2-2.4). In the register-derived cohort, an increased fracture risk was also observed among men aged ≥ 55 years at study baseline (IRR 2.0, 95% CI 1.2-3.5), whereas the incidence rate of fractures was not significantly increased among younger men or women (IRR for men < 55 yrs: 1.0, 95% CI 0.4-2.3; IRR for women ≥ 55 yrs: 0.9, 95% CI 0.5-1.5; IRR for women < 55 yrs: 1.6, 95% CI 0.7-3.6). Elderly male patients with GPA have a pronounced risk of developing fractures. This finding is of relevance for the clinical management of patients with GPA.

  15. Retro-orbital granuloma associated with granulomatosis with polyangiitis: a series of nine cases.

    Science.gov (United States)

    Bitik, Berivan; Kılıç, Levent; Küçükşahin, Orhan; Şahin, Kubilay; Tufan, Abdurrahman; Karadağ, Ömer; Pay, Salih; Ateş, Aşkın; Ucar, Murat; Tutar, Hakan; Karaaslan, Yaşar; Yilmaz, Sedat; Ertenli, A Ihsan; Konuk, Onur; Turgay, Murat; Goker, Berna

    2015-06-01

    Retro-orbital granuloma is a rare and devastating component of granulomatosis with polyangiitis (GPA). Current medical treatment protocols are falling short, and outcomes are poor. The aim of the study was to investigate the frequency, clinical features, and treatment outcomes of retro-orbital granuloma in patients with GPA. This is a retrospective, multi-centre study, which involves GPA cohorts from five different clinics. Data were extracted from patient charts including history, physical examination, radiological-laboratory-histological findings, and treatment protocols. Major clinical outcome measures were changes in the volume of the granuloma on comparative MRI, and visual acuity on repeated ophthalmologic examinations. Among 141 GPA patients, nine (five females and four males) were diagnosed with a retro-orbital granuloma. Median duration of disease was 8 years. Proptosis and diplopia were the dominant presenting symptoms (77%), followed by orbital pain (55%). Three out of nine patients had isolated retro-orbital granulomas, without other organ involvement of GPA. Five patients received conventional pulse steroid and pulse cyclophosphamide (CYC) as the first-line remission induction therapy. Four of these patients had progressive disease, and a regression in granuloma size was observed in one patient using this regimen. Two patients were already receiving immunosuppressants when they were diagnosed with retro-orbital granuloma. Six patients had been treated with RTX as the second-line remission induction therapy. None of these patients had progression following RTX therapy. Three patients underwent orbital decompression surgery. The indication for the decision for surgery was either progressive loss of vision or intractable pain. Standard first-line chemotherapy (CYC and steroids) was ineffective against retro-orbital granuloma associated with GPA. RTX could be an alternative in these cases. Surgical intervention may help to decrease the morbidity. Further

  16. Development of a computerized handbook of architectural plans

    NARCIS (Netherlands)

    Koutamanis, A.

    1990-01-01

    The dissertation investigates an approach to the development of visual / spatial computer representations for architectural purposes through the development of the computerized handbook of architectural plans (chap), a knowledge-based computer system capable of recognizing the metric properties of

  17. Survey of methods for improving operator acceptance of computerized aids

    Energy Technology Data Exchange (ETDEWEB)

    Frey, P. R.; Kisner, R. A.

    1982-04-01

    The success of current attempts to improve the operational performance and safety of nuclear power plants by installing computerized operational aids in the control rooms is dependent, in part, on the operator's attitude toward the aid. Utility experience with process computer systems indicates that problems may already exist with operator acceptance of computerized aids. The growth of the role that computers have in nuclear power plants makes user acceptance of computer technology an important issue for the nuclear industry. The purpose of this report is to draw from the literature factors related to user acceptance of computerized equipment that may also be applicable to the acceptance of computerized aids used in the nuclear power plant control room.

  18. Significance of computerized tomography and nasal cytology in the ...

    African Journals Online (AJOL)

    Significance of computerized tomography and nasal cytology in the diagnosis of rhinosinusitis among asthmatic children. Karima Abdel Khalik, Laila AG Hegazy, Hassan A Wahba, Laila A Abdurrahman, Sahar SA Zaki ...

  19. Computerized Point of Sale = Faster Service + Better Accountability.

    Science.gov (United States)

    Pannell, Dorothy V.

    1991-01-01

    Describes selecting and installing a computerized point of sale for a district food service program; the equipment needed and preferred; and the training of trainers, managers, and cashiers. Also discusses the direct benefits and side benefits of the system. (MLF)

  20. Radation distribution in head & thorax computerized tomography

    Directory of Open Access Journals (Sweden)

    Mohammad Mahdavi

    2014-05-01

    Full Text Available Background: Determination of the exposure levels in the computerized tomography (CT practices is necessary to define the respected national reference levels, quality control of CT centers and the risk assessment for radiation induced cancers. Material and Methods: On the basis of this necessity, the radiation exposure distribution due to common CT practices has been investigated at Razi Hospital CT center in Rasht using tissue-equivalent phantoms and the thermoluminescent dosimeters (TLD. The Head and Thorax phantoms were used with the standard dimensions incorporating holes at the center and edges for TLD placement. Dosimetry was carried out using LiF Mg, Cu, P small chips due to their relatively tissue equivalence, high sensitivity convenient annealing procedure and the non-complex glow curve. Results: Results showed that CTDI for Head is 52.85 mGy and for Body is 68.15mGy. CTDLW for Head is 13.67 mGy and for Body is 16.94mGy . Conclusion: In comparison with other radiographical procedures, patient absorbed doses in CT imaging are usually very high. Content of dose is increased by increase of mAs.

  1. Computerized Interactive Gaming via Supporting Vector Machines

    Directory of Open Access Journals (Sweden)

    Y. Jiang

    2008-01-01

    Full Text Available Computerized interactive gaming requires automatic processing of large volume of random data produced by players on spot, such as shooting, football kicking, and boxing. This paper describes a supporting vector machine-based artificial intelligence algorithm as one of the possible solutions to the problem of random data processing and the provision of interactive indication for further actions. In comparison with existing techniques, such as rule-based and neural networks, and so forth, our SVM-based interactive gaming algorithm has the features of (i high-speed processing, providing instant response to the players, (ii winner selection and control by one parameter, which can be predesigned and adjusted according to the needs of interaction and game design or specific level of difficulties, and (iii detection of interaction points is adaptive to the input changes, and no labelled training data is required. Experiments on numerical simulation support that the proposed algorithm is robust to random noise, accurate in picking up winning data, and convenient for all interactive gaming designs.

  2. Study on forefoot by computerized tomography

    Energy Technology Data Exchange (ETDEWEB)

    Machida, Eiichi (Nihon Univ., Tokyo. School of Medicine)

    1983-10-01

    Computerized tomography (CT) was used to study coronary sections of the forefoot in both normal and abnormal human feet. CT images of the transverse arches at the metatarsal head, middle and base of the shaft were classified into five patterns. In the pattern most commonly found in normal feet, the second metatarsus appeared elevated above the other metatarsal bones at all points, and there was a gradual and even reduction in elevation from the second to the fifth metatarsal. In cases of hallux valgus, however, a variety of deformities were noted in the arc of the second to fifth metatarsals, particularly at the head. The rotation of the first metatarsus and shift of the sesamoids were measured from CT images at the head of the first metatarsus. In hallux valgus, both the rotation and the sesamoid shift appeared to have a wider angle than in the case of normal feet. In normal feet, the differences between the rotation of the first metatarsus and shift of the sesamoids were relatively small, whereas in hallux valgus there was a much greater degree of variation. Furthermore, while normal feet the variation in rotation of the first metatarsus and sesamoid shift both tended to be either great or small, in hallux valgus a large degree of sesamoid shift was sometimes found in combination with a small degree of rotation of the first metatarsus.

  3. Computerized adaptive testing--ready for ambulatory monitoring?

    DEFF Research Database (Denmark)

    Rose, Matthias; Bjørner, Jakob; Fischer, Felix

    2012-01-01

    Computerized adaptive tests (CATs) have abundant theoretical advantages over established static instruments, which could improve ambulatory monitoring of patient-reported outcomes (PROs). However, an empirical demonstration of their practical benefits is warranted.......Computerized adaptive tests (CATs) have abundant theoretical advantages over established static instruments, which could improve ambulatory monitoring of patient-reported outcomes (PROs). However, an empirical demonstration of their practical benefits is warranted....

  4. A Computerized Test of Design Fluency.

    Directory of Open Access Journals (Sweden)

    David L Woods

    Full Text Available Tests of design fluency (DF assess a participant's ability to generate geometric patterns and are thought to measure executive functions involving the non-dominant frontal lobe. Here, we describe the properties of a rapidly administered computerized design-fluency (C-DF test that measures response times, and is automatically scored. In Experiment 1, we found that the number of unique patterns produced over 90 s by 180 control participants (ages 18 to 82 years correlated with age, education, and daily computer-use. Each line in the continuous 4-line patterns required approximately 1.0 s to draw. The rate of pattern production and the incidence of repeated patterns both increased over the 90 s test. Unique pattern z-scores (corrected for age and computer-use correlated with the results of other neuropsychological tests performed on the same day. Experiment 2 analyzed C-DF test-retest reliability in 55 participants in three test sessions at weekly intervals and found high z-score intraclass correlation coefficients (ICC = 0.79. Z-scores in the first session did not differ significantly from those of Experiment 1, but performance improved significantly over repeated tests. Experiment 3 investigated the performance of Experiment 2 participants when instructed to simulate malingering. Z-scores were significantly reduced and pattern repetitions increased, but there was considerable overlap with the performance of the control population. Experiment 4 examined performance in veteran patients tested more than one year after traumatic brain injury (TBI. Patients with mild TBI performed within the normal range, but patients with severe TBI showed reduced z-scores. The C-DF test reliably measures visuospatial pattern generation ability and reveals performance deficits in patients with severe TBI.

  5. Orbital socket contracture: a complication of inflammatory orbital disease in patients with Wegener’s granulomatosis

    Science.gov (United States)

    Talar-Williams, C; Sneller, M C; Langford, C A; Smith, J A; Cox, T A; Robinson, M R

    2005-01-01

    Aim: To describe the clinical characteristics of orbital socket contracture in patients with Wegener’s granulomatosis (WG). Methods: A retrospective cohort study The medical records of 256 patients with WG examined at the National Institutes of Health from 1967 to 2004 were reviewed to identify patients with orbital socket contracture. Details of the orbital disease including Hertel exophthalmometry readings, radiological findings, and results of eye examinations were recorded. Orbital socket contracture was defined as orbital inflammation with proptosis followed by the development of enophthalmos and radiographic evidence of residual fibrotic changes in the orbit. To examine for risk factors in the development of a contracted orbit, patients with orbital socket contracture were compared to patients without contracture with respect to multiple variables including history of orbital surgery, orbital disease severity, and major organ system involvement. The main outcome measures were the clinical characteristics of orbital socket contracture associated with inflammatory orbital disease in patients with WG. Results: Inflammatory orbital disease occurred in 34 of 256 (13%) patients and detailed clinical data on 18 patients were available and examined. Orbital socket contracture occurred during the clinical course in six patients; the features included restrictive ophthalmopathy (five), chronic orbital pain (three), and ischaemic optic nerve disease (two) resulting in blindness (no light perception) in one patient. The orbital socket contracture occurred within 3 months of treatment with immunosuppressive medications for inflammatory orbital disease in five patients and was not responsive to immunosuppressive medications. The median degree of enophthalmos in the contracted orbit compared with the fellow eye was 2.8 mm (range 1.5–3.5 mm) by Hertel exophthalmometry. There were no risk factors that predicted development of orbital socket contracture. Conclusions: In

  6. Aortic {sup 18}F-FDG uptake in patients suffering from granulomatosis with polyangiitis

    Energy Technology Data Exchange (ETDEWEB)

    Kemna, Michael J. [Maastricht University Medical Center, Department of Nephrology and Clinical Immunology, Maastricht (Netherlands); Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Bucerius, Jan [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Maastricht University Medical Center, Department of Nuclear Medicine, Maastricht (Netherlands); University Hospital RWTH Aachen, Department of Nuclear Medicine, Aachen (Germany); Drent, Marjolein [Maastricht University, Department of Pharmacology and Toxicology, Maastricht (Netherlands); Voeoe, Stefan [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Maastricht University Medical Center, Department of Nuclear Medicine, Maastricht (Netherlands); Veenman, Martine [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Paassen, Pieter van [Maastricht University Medical Center, Department of Nephrology and Clinical Immunology, Maastricht (Netherlands); Tervaert, Jan Willem Cohen [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Sint Franciscus Gasthuis, Noordoever Academy, Rotterdam (Netherlands); Kroonenburgh, Marinus J.P.G. van [Maastricht University Medical Center, Department of Nuclear Medicine, Maastricht (Netherlands)

    2015-08-15

    The objective of the study was to systematically assess aortic inflammation in patients with granulomatosis with polyangiitis (GPA) using {sup 18}F-2-deoxy-2-[{sup 18}F]fluoro-D-glucose (FDG) positron emission tomography (PET)/CT. Aortic inflammation was studied in PET/CT scans obtained from 21 patients with GPA; 14 patients with sarcoidosis were included as disease controls, 7 patients with stage I or II head and neck carcinoma ascertained during routine clinical practice were used as healthy controls (HC) and 5 patients with large vessel vasculitis (LVV) were used as positive controls. Aortic {sup 18}F-FDG uptake was expressed as the blood-normalized maximum standardized uptake value (SUV{sub max}), known as the target to background ratio (mean TBR{sub max}). The mean TBR{sub max} (interquartile range) of the aorta in patients with GPA, sarcoidosis, HC and LVV were 1.75 (1.32-2.05), 1.62 (1.54-1.74), 1.29 (1.22-1.52) and 2.03 (1.67-2.45), respectively. The mean TBR{sub max} was significantly higher in patients suffering from GPA or LVV compared to HC (p < 0.05 and p < 0.005, respectively) and tended to be higher in patients suffering from sarcoidosis, but this did not reach statistical significance (p = 0.098). The mean TBR{sub max} of the most diseased segment was significantly higher compared to HC [1.57 (1.39-1.81)] in LVV patients [2.55 (2.22-2.82), p < 0.005], GPA patients [2.17 (1.89-2.83), p < 0.005] and patients suffering from sarcoidosis [2.04 (1.88-2.20), p < 0.05]. In GPA patients, the mean TBR{sub max} of the aorta was significantly higher in patients with previous renal involvement [2.01 (1.69-2.53)] compared to patients without renal involvement in the past [1.60 (1.51-1.80), p < 0.05]. Interrater reproducibility with a second reader was high (all intraclass correlation coefficients >0.9). Patients suffering from GPA show marked aortic FDG uptake. (orig.)

  7. Computerized videodefecography versus defecography: do we need radiographs?

    Directory of Open Access Journals (Sweden)

    Carlos Walter Sobrado

    Full Text Available CONTEXT AND OBJECTIVE: Defecography has been recognized as a valuable method for evaluating patients with evacuation disorders. It consists of the use of static radiography and fluoroscopy to record different situations within anorectal dynamics. Conventionally, rectal parameters are measured using radiograms. It is rare for fluoroscopy alone to be used. Computer software has been developed with the specific aim of calculating these measurements from digitized videotaped images obtained during fluoroscopy, without the need for radiographic film, thereby developing a computerized videodefecography method. The objective was thus to compare measurements obtained via computerized videodefecography with conventional measurements and to discuss the advantages of the new method. DESIGN AND SETTING: Prospective study at the radiology service of Hospital das Clínicas, Universidade de São Paulo. METHOD: Ten consecutive normal subjects underwent videodefecography. The anorectal angle, anorectal junction, puborectalis muscle length, anal canal length and degree of anal relaxation were obtained via the conventional method (using radiography film and via computerized videodefecography using the ANGDIST software. Measurement and analysis of these parameters was performed by two independent physicians. RESULTS: Statistical analysis confirmed that the measurements obtained through direct radiography film assessment and using digital image analysis (computerized videodefecography were equivalent. CONCLUSIONS: Computerized videodefecography is equivalent to the traditional defecography examination. It has the advantage of offering reduced radiation exposure through saving on the use of radiography.

  8. Computerized photogrammetry used to calculate the brow position index.

    Science.gov (United States)

    Naif-de-Andrade, Naif Thadeu; Hochman, Bernardo; Naif-de-Andrade, Camila Zirlis; Ferreira, Lydia Masako

    2012-10-01

    The orbital region is of vital importance to facial expression. Brow ptosis, besides having an impact on facial harmony, is a sign of aging. Various surgical techniques have been developed to increase the efficacy of brow-lift surgery. However, no consensus method exists for an objective measurement of the eyebrow position due to the curvature of the face. Therefore, this study aimed to establish a method for measuring the eyebrow position using computerized photogrammetry. For this study, 20 orbital regions of 10 volunteers were measured by direct anthropometry using a digital caliper and by indirect anthropometry (computerized photogrammetry) using standardized digital photographs. Lines, points, and distances were defined based on the position of the anthropometric landmarks endocanthion and exocanthion and then used to calculate the brow position index (BPI). Statistical analysis was performed using Student's t test with a significance level of 5 %. The BPI values obtained by computerized photogrammetric measurements did not differ significantly from those obtained by direct anthropometric measurements (p > 0.05). The mean BPI was 84.89 ± 10.30 for the computerized photogrammetric measurements and 85.27 ± 10.67 for the direct anthropometric measurements. The BPI defined in this study and obtained by computerized photogrammetry is a reproducible and efficient method for measuring the eyebrow position. This journal requires that authors assign a level of evidence to each article.

  9. Computerized tomographic evaluation of intracranial metastases

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Bo Yong; Lee, Mi Sook; Choi, Jin Ok; Jeon, Doo Sung; Kim, Hong Soo; Rhee, Hak Song [Presbyterian Medical Center, Chonju (Korea, Republic of)

    1986-12-15

    In a study of intracranial metastases, 46 cases having satisfactory clinical, operative and histological proofs were analyzed by computerized tomography at Presbyterian Medical Center from May, 1982 to February, 1986. The results were as follows: 1. The male to female ratio of intracranial metastases were 67:33. The 5th decade group (34.8%) was the most prevalent age group, followed by the 6th decade (21.7%) and 7th decade (21.7%). 2. The number of lesions was found be: single -25 cases (54.3%); multiple -21 cases (45.7%). 3. The source of intracranial metastases found to be: lung 15 cases (32.6%); unknown 12 cases (26.0%); chorioca 3 cases (6.5%); liver 3 cases (6.5%); stomach 2 cases (4.3%); parotid, breast, kidney, prostate, melanoma, rectal ca, rhabdomyosarcoma, nasal ca, lymphoma, testicular ca, cervix, each 1 case (2.2%). 4. The locations of the intracranial metastases were as follows: Cerebral hemisphere 37.7% in parietal region Cerebral hemisphere 15.9% in in frontal region Cerebral hemisphere 13.4% in occipital region Cerebral hemisphere 10.5% in temporal region Cerebellar hemisphere 3.2% Cerebellopontine angle 3.2% Intraventricular 4.8% Meninges 4.8% Skull vault 6.5% 5. Peritumor edema was found to be: Grade II-17 cases (37.0%): Grade III-14 cases (30.4%); Grade I-8 cases (17.4%); Grade 0-7 cases (15.2%) in that order. 6. The chief complaints of intracranial metastases on admission, were as follows: Headache 30 cases (65.2%); Vomiting 11 cases (23.9%); deteriorated mental state 10 cases (21.7%); Hemiplegia 7 cases (15.2%); visual disturbance 6 cases (13.0%); hemiparesis 4 cases (8.7%); seizure 4 cases (8.7%); other symptoms were less frequent. 7. On pre-contrast scan, hyperdense lesions were present in 18 cases (39.1%); hypodense lesions in 15 cases (32.6%); mixed density in 8 cases (17.4%); isodensity was present in 5 cases (10.9%). On post-contrast scan, ring enhancement was seen in 19 cases (41.3%); nodular enhancement in 17 cases (37%), mixed ring

  10. Granulomatosis with polyangiitis in children: report of a case with kidney-lung syndrome = Granulomatosis con poliangitis en pediatría: a propósito de un caso con síndrome pulmón-riñón

    Directory of Open Access Journals (Sweden)

    Muñoz Grajales, Carolina

    2013-04-01

    Full Text Available Granulomatosis with polyangiitis as a cause of kidney-lung syndrome is uncommon in children. We report the case of a 13 year old patient with alveolar hemorrhage, rapidly progressive glomerulonephritis, episcleritis and anti PR3. We refer to features of the ANCA-associated vasculitis in children and to its therapeutic approach.

  11. Granulomatosis with Polyangiitis Presenting with Acute Aortic and Mitral Regurgitation: Case Report and Big-Data Analysis.

    Science.gov (United States)

    Al-Kindi, Sadeer G; Amer Al-Aiti, M; Yang, Michael; Josephson, Richard A

    2017-01-01

    Granulomatosis with polyangiitis (GPA) is a rare vasculitis that can have multisystem involvement, though cardiac involvement is very rare. The case is described of a 53-year-old woman who presented with acute aortic and mitral valve regurgitation requiring surgical intervention. Pathology from the excised aortic valve showed geographic necrosis concerning for GPA. Subsequent rheumatologic testing was positive for anti-serine proteinase 3 (PR3) antibody, consistent with GPA. A year after the valve surgery the patient was found to have a vegetation of the mitral valve and elevated PR3 antibody levels, and was successfully treated with an intensification of immunosuppression. The cardiac involvement of GPA is discussed and big data analyzed to identify the epidemiology of valvular involvement. In conclusion, GPA can have multiple different valvular manifestations with a high recurrence rate. GPA should be considered in patients who present with acute valvular disease without any clear precipitant.

  12. The use of abatacept in debilitating cavitating lung disease associated with rheumatoid arthritis, bronchocentric granulomatosis and aspergillosis.

    LENUS (Irish Health Repository)

    Neff, K

    2010-06-01

    A case of debilitating cavitating lung disease associated with rheumatoid arthritis and bronchocentric granulomatosis, which failed to respond to conventional medical or surgical treatment, is described. The patient was treated over 10 years with steroids, antimicrobial agents, disease-modifying antirheumatoid drugs and surgery. Lung function continued to decline and the patient presented for admission with recurrent pneumonia. Abatacept was initiated to modify the underlying immunopathology. Following 12 months of treatment with abatacept the patient has demonstrable improvement in lung function and lung anatomy, and has not presented to hospital with pneumonia. She has tolerated the treatment without complication. The use of abatacept has stabilised the lung disease in this case in the medium term and prevented readmission to hospital. These results suggest a larger role for abatacept in those with such disease in the future and may warrant further investigation.

  13. Pain Perception: Computerized versus Traditional Local Anesthesia in Pediatric Patients.

    Science.gov (United States)

    Mittal, M; Kumar, A; Srivastava, D; Sharma, P; Sharma, S

    2015-01-01

    Local anesthetic injection is one of the most anxiety- provoking procedure for both children and adult patients in dentistry. A computerized system for slow delivery of local anesthetic has been developed as a possible solution to reduce the pain related to the local anesthetic injection. The present study was conducted to evaluate and compare pain perception rates in pediatric patients with computerized system and traditional methods, both objectively and subjectively. It was a randomized controlled study in one hundred children aged 8-12 years in healthy physical and mental state, assessed as being cooperative, requiring extraction of maxillary primary molars. Children were divided into two groups by random sampling - Group A received buccal and palatal infiltration injection using Wand, while Group B received buccal and palatal infiltration using traditional syringe. Visual Analog scale (VAS) was used for subjective evaluation of pain perception by patient. Sound, Eye, Motor (SEM) scale was used as an objective method where sound, eye and motor reactions of patient were observed and heart rate measurement using pulse oximeter was used as the physiological parameter for objective evaluation. Patients experienced significantly less pain of injection with the computerized method during palatal infiltration, while less pain was not statistically significant during buccal infiltration. Heart rate increased during both buccal and palatal infiltration in traditional and computerized local anesthesia, but difference between traditional and computerized method was not statistically significant. It was concluded that pain perception was significantly more during traditional palatal infiltration injection as compared to computerized palatal infiltration, while there was no difference in pain perception during buccal infiltration in both the groups.

  14. A computerized business simulation for dental practice management.

    Science.gov (United States)

    Willis, D O; Smith, J R; Golden, P

    1997-10-01

    Computerized simulations have been used for many years for teaching principles of management in business schools. This paper describes the development of a computerized business simulation for use in dental school practice management courses. The simulation is in a competitive game format. It requires students to formulate strategies and to implement management decisions that reinforce and fulfill that strategy. Participants use the outcomes of these decisions to formulate new management decisions for the upcoming period. Student response to participation in the simulation has been positive, with students indicating that participation is valuable for developing better understanding of analytical business management techniques and interpersonal techniques such as group process and leadership skills.

  15. THE VALIDITY OF HUMAN AND COMPUTERIZED WRITING ASSESSMENT

    Energy Technology Data Exchange (ETDEWEB)

    Ronald L. Boring

    2005-09-01

    This paper summarizes an experiment designed to assess the validity of essay grading between holistic and analytic human graders and a computerized grader based on latent semantic analysis. The validity of the grade was gauged by the extent to which the student’s knowledge of the topic correlated with the grader’s expert knowledge. To assess knowledge, Pathfinder networks were generated by the student essay writers, the holistic and analytic graders, and the computerized grader. It was found that the computer generated grades more closely matched the definition of valid grading than did human generated grades.

  16. Design and use of a computerized test generating program

    Science.gov (United States)

    Schaefer, Edward; Marschall, Laurence A.

    1980-07-01

    An easy-to-use set of programs for the computerized generation of multiple-choice and essay examinations in an introductory astronomy course is described. The programs allow the user to establish files of test questions and to rapidly assemble printed copies of examinations suitable for photocopying. Written in ALGOL for a Burroughs B6700 computer, the programs can, in principle, be implemented on large mainframe computers or on microcomputers of a size increasingly available to physics departments. The advantages and costs of computerized test generation are discussed.

  17. Computerization of a Nursing Chart According to the Nursing Process.

    Science.gov (United States)

    Schachner, María B; González, Zulma A; Sommer, Janine A; Recondo, Francisco J; Gassino, Fernando D; Luna, Daniel R; Benítez, Sonia E

    2016-01-01

    The benefits associated with the computerization of clinical records are known since a long time ago. Documentation evolution from paper to electronic format aims to always improve communication, reduce errors and facilitate continuity of care. Ideally when improvements to nursing records are contemplated, they should consider the nurses needs, new functionality workflow impacts and correspondence with representation models of standardized data that are specific to their domains practices. The aim of this study was to describe the development and implementation of computerized nursing record at Hospital Italiano de Buenos Aires.

  18. Computerized Workstation for Tsunami Hazard Monitoring

    Science.gov (United States)

    Lavrentiev-Jr, Mikhail; Marchuk, Andrey; Romanenko, Alexey; Simonov, Konstantin; Titov, Vasiliy

    2010-05-01

    We present general structure and functionality of the proposed Computerized Workstation for Tsunami Hazard Monitoring (CWTHM). The tool allows interactive monitoring of hazard, tsunami risk assessment, and mitigation - at all stages, from the period of strong tsunamigenic earthquake preparation to inundation of the defended coastal areas. CWTHM is a software-hardware complex with a set of software applications, optimized to achieve best performance on hardware platforms in use. The complex is calibrated for selected tsunami source zone(s) and coastal zone(s) to be defended. The number of zones (both source and coastal) is determined, or restricted, by available hardware resources. The presented complex performs monitoring of selected tsunami source zone via the Internet. The authors developed original algorithms, which enable detection of the preparation zone of the strong underwater earthquake automatically. For the so-determined zone the event time, magnitude and spatial location of tsunami source are evaluated by means of energy of the seismic precursors (foreshocks) analysis. All the above parameters are updated after each foreshock. Once preparing event is detected, several scenarios are forecasted for wave amplitude parameters as well as the inundation zone. Estimations include the lowest and the highest wave amplitudes and the least and the most inundation zone. In addition to that, the most probable case is calculated. In case of multiple defended coastal zones, forecasts and estimates can be done in parallel. Each time the simulated model wave reaches deep ocean buoys or tidal gauge, expected values of wave parameters and inundation zones are updated with historical events information and pre-calculated scenarios. The Method of Splitting Tsunami (MOST) software package is used for mathematical simulation. The authors suggest code acceleration for deep water wave propagation. As a result, performance is 15 times faster compared to MOST, original version

  19. WegenerNet climate station network region Feldbach/Austria: From local measurements to weather and climate data products at 1 km-scale resolution

    Science.gov (United States)

    Kabas, T.; Leuprecht, A.; Bichler, C.; Kirchengast, G.

    2010-12-01

    South-eastern Austria is characteristic for experiencing a rich variety of weather and climate patterns. For this reason, the county of Feldbach was selected by the Wegener Center as a focus area for a pioneering observation experiment at very high resolution: The WegenerNet climate station network (in brief WegenerNet) comprises 151 meteorological stations within an area of about 20 km × 15 km (~ 1.4 km × 1.4 km station grid). All stations measure the main parameters temperature, humidity and precipitation with 5 minute sampling. Selected further stations include measurements of wind speed and direction completed by soil parameters as well as air pressure and net radiation. The collected data is integrated in an automatic processing system including data transfer, quality control, product generation, and visualization. Each station is equipped with an internet-attached data logger and the measurements are transferred as binary files via GPRS to the WegenerNet server in 1 hour intervals. The incoming raw data files of measured parameters as well as several operating values of the data logger are stored in a relational database (PostgreSQL). Next, the raw data pass the Quality Control System (QCS) in which the data are checked for its technical and physical plausibility (e.g., sensor specifications, temporal and spatial variability). In consideration of the data quality (quality flag), the Data Product Generator (DPG) results in weather and climate data products on various temporal scales (from 5 min to annual) for single stations and regular grids. Gridded data are derived by vertical scaling and squared inverse distance interpolation (1 km × 1 km and 0.01° × 0.01° grids). Both subsystems (QCS and DPG) are realized by the programming language Python. For application purposes the resulting data products are available via the bi-lingual (dt, en) WegenerNet data portal (www.wegenernet.org). At this time, the main interface is still online in a system in which

  20. [Ergonomics in computerized workplace--an ophthalmological view].

    Science.gov (United States)

    Mrugacz, Małgorzata; Szumiński, Michał

    2009-01-01

    Ergonomics in computerized workplace contributes to create special environmental conditions depending on people's needs and possibilities. Ergonomically created room with accurate type of lighting, temperature and humidity, furniture and location of PC unit and monitor is essential to avoid symptoms of computer stress syndrome.

  1. Physicians' responses to computerized drug interaction alerts with password overrides.

    Science.gov (United States)

    Nasuhara, Yasuyuki; Sakushima, Ken; Endoh, Akira; Umeki, Reona; Oki, Hiromitsu; Yamada, Takehiro; Iseki, Ken; Ishikawa, Makoto

    2015-08-28

    Although evidence has suggested that computerized drug-drug interaction alert systems may reduce the occurrence of drug-drug interactions, the numerous reminders and alerts generated by such systems could represent an excessive burden for clinicians, resulting in a high override rate of not only unimportant, but also important alerts. We analyzed physicians' responses to alerts of relative contraindications and contraindications for coadministration in a computerized drug-drug interaction alert system at Hokkaido University Hospital. In this system, the physician must enter a password to override an alert and continue an order. All of the drug-drug interaction alerts generated between December 2011 and November 2012 at Hokkaido University Hospital were included in this study. The system generated a total of 170 alerts of relative contraindications and contraindication for coadministration; 59 (34.7 %) of the corresponding orders were cancelled after the alert was accepted, and 111 (65.3 %) were overridden. The most frequent contraindication alert was for the combination of 3-hydroxy-3-methylglutaryl-coenzyme A reductase inhibitors and fibrates. No incidents involving drug-drug interactions were reported among patients who were prescribed contraindicated drug pairs after an override. Although computerized drug-drug interaction alert systems that require password overrides appear useful for promoting medication safety, having to enter passwords to override alerts may represent an excessive burden for the prescribing physician. Therefore, both patient safety and physicians' workloads should be taken into consideration in future designs of computerized drug-drug interaction alert systems.

  2. Discovering Visual Scanning Patterns in a Computerized Cancellation Test

    Science.gov (United States)

    Huang, Ho-Chuan; Wang, Tsui-Ying

    2013-01-01

    The purpose of this study was to develop an attention sequential mining mechanism for investigating the sequential patterns of children's visual scanning process in a computerized cancellation test. Participants had to locate and cancel the target amongst other non-targets in a structured form, and a random form with Chinese stimuli. Twenty-three…

  3. Wilderness Management... A Computerized System for Summarizing Permit Information

    Science.gov (United States)

    Gary H. Elsner

    1972-01-01

    Permits were first needed for visits to wilderness areas in California during summer 1971. A computerized system for analyzing these permits and summarizing information from them has been developed. It produces four types of summary tables: point-of-origin of visitors; daily variation in total number of persons present; variations in group size; and variations in...

  4. The Design Of Computerized Database Of Scientific Reports Of ...

    African Journals Online (AJOL)

    ... journal articles, seminar papers, conference proceedings etc. The analysis revealed the shortcomings of the manual system and the alternative system design, that is, computerized database was developed to address these problems. Visual Basic 6.0 and Microsoft Access 2000 were the software packages used for the ...

  5. 381 Developing of a Computerized Brain Diagnosing System for ...

    African Journals Online (AJOL)

    User

    computerized brain diagnosing system that would be used in carrying out the daily diagnosing activity in the clinic .... Stand-alone Units are being used to monitor and control operations in factories and office buildings. .... he understood in terms of the numeric manipulations involved in expected value computations, which is ...

  6. Computerized Adaptive Testing System Design: Preliminary Design Considerations.

    Science.gov (United States)

    Croll, Paul R.

    A functional design model for a computerized adaptive testing (CAT) system was developed and presented through a series of hierarchy plus input-process-output (HIPO) diagrams. System functions were translated into system structure: specifically, into 34 software components. Implementation of the design in a physical system was addressed through…

  7. Computerized Typesetting and Other New Applications in a Publishing House

    Science.gov (United States)

    Järvi, Timo

    The author was involved during years 1964-67 in a change process from manual typesetting to a computerized one. This paper describes the design and implementation of new graphical applications as well as the programming of IBM 1401. It also presents some reflections on the social affects of these changes.

  8. A Computerized Reference Library Using ZOG. Technical Report No. 7.

    Science.gov (United States)

    Gregg, Lee W.; Hannah, Joyce E.

    To assist researchers, practitioners, and students in locating journal articles, books, papers, and reports relevant to all aspects of document design, the Document Design Project staff at Carnegie-Mellon University (CMU) is designing and building a computerized reference library using the operating system ZOG. The library will include material…

  9. Duplicated laboratory tests: evaluation of a computerized alert intervention abstract.

    Science.gov (United States)

    Bridges, Sharon A; Papa, Linda; Norris, Anne E; Chase, Susan K

    2014-01-01

    Redundant testing contributes to reductions in healthcare system efficiency. The purpose of this study was to: (1) determine if the use of a computerized alert would reduce the number and cost of duplicated Acute Hepatitis Profile (AHP) laboratory tests and (2) assess what patient, test, and system factors were associated with duplication. This study used a quasi-experimental pre- and post-test design to determine the proportion of duplication of the AHP test before and after implementation of a computerized alert intervention. The AHP test was duplicated if the test was requested again within 15 days of the initial test being performed and the result present in the medical record. The intervention consisted of a computerized alert (pop-up window) that indicated to the clinician that the test had recently been ordered. A total of 674 AHP tests were performed in the pre-intervention period and 692 in the postintervention group. In the pre-intervention period, 53 (7.9%) were duplicated and in postintervention, 18 (2.6%) were duplicated (pimplementation of the alert was shown to significantly reduce associated costs of duplicated AHP tests (p≤.001). Implementation of computerized alerts may be useful in reducing duplicate laboratory tests and improving healthcare system efficiency. © 2012 National Association for Healthcare Quality.

  10. Attitudes towards Computerization in Canadian Universities. Technical Paper #4.

    Science.gov (United States)

    Misfeldt, Renee; Stahl, William A.

    This report summarizes the attitudinal portion of a nation-wide survey on the computerization of Canadian universities. Six different questionnaires, each of which contained the same questions on attitudes, were mailed to faculty, deans, admissions, officers, registrars, computer center directors, and other administrators at 63 Canadian…

  11. A Computerized System for Workplace Design for Visually Impaired Workers.

    Science.gov (United States)

    Chen, J-G; Hou, C-A

    1991-01-01

    VITAL (Vision Impaired Task and Assignment Lexicon) is an integrated computerized system that performs workplace design tasks for visually impaired workers. VITAL consists of three modules: ergonomics consultation, disability index, and work measurement. Evaluation indicated that VITAL could be used as a tool to help nonprofessional vocational…

  12. Computerized adaptive testing for measuring development of young children

    NARCIS (Netherlands)

    Jacobusse, G.; Buuren, S. van

    2007-01-01

    Developmental indicators that are used for routine measurement in The Netherlands are usually chosen to optimally identify delayed children. Measurements on the majority of children without problems are therefore quite imprecise. This study explores the use of computerized adaptive testing (CAT) to

  13. Multidimensional Computerized Adaptive Testing for Indonesia Junior High School Biology

    Science.gov (United States)

    Kuo, Bor-Chen; Daud, Muslem; Yang, Chih-Wei

    2015-01-01

    This paper describes a curriculum-based multidimensional computerized adaptive test that was developed for Indonesia junior high school Biology. In adherence to the Indonesian curriculum of different Biology dimensions, 300 items was constructed, and then tested to 2238 students. A multidimensional random coefficients multinomial logit model was…

  14. California Superfund sites: Insights from a computerized database

    Energy Technology Data Exchange (ETDEWEB)

    Layefsky, M.E.; Smith, D.F.; Mendell, M.J.; Schlag, R.D.; Neutra, R.R. (California Department of Health Services, Berkeley (USA))

    A computerized database of 93 California State Superfund waste sites was created to assess the feasibility of using such a system for a variety of public health purposes. Though available data were limited in many respects, analysis of the database proved useful in summarizing features of hazardous waste sites that could be of considerable public health interest.

  15. Computerized tomography and angiography - competing processes in liver diagnostics

    Energy Technology Data Exchange (ETDEWEB)

    Wunschik, F.; Luetgemeier, J.; Hoerst, M.

    1981-02-01

    In the diagnostics of liver diseases, computerized tomography is a valuable method. It is particularly superior to angiography in detecting cysts, parasitosis and abscesses. Angiography however is better for known solid tumours regarding differencial diagnosis. Both methods supplement each other in the tumour and bile ducts diagnostics.

  16. Changes in the Approaches of Teachers Following Computerization of Schools

    Science.gov (United States)

    Wasserman, Egoza; Millgram, Yitzchak

    2005-01-01

    This article examines the change in teachers' attitudes and instruction following computerization of their schools. Parameters chosen to assure the success of the study were: teachers' training courses, establishment of teachers' teams, teachers' expectations and willingness, and teachers' use of the computer in the school. The study took place in…

  17. Computerized adaptive testing in industrial and organizational psychology

    NARCIS (Netherlands)

    Makransky, Guido

    2012-01-01

    The overarching goal of this dissertation is to increase the precision and efficiency of the measurement tools that are used to make selection decisions in industrial/organizational psychology, by introducing psychometric innovations in the framework of computerized adaptive testing (CAT). Chapter 1

  18. Computerized Classification Testing and Its Relationship to the Testing Goal

    NARCIS (Netherlands)

    van Groen, Maaike; Eggen, Theodorus Johannes Hendrikus Maria; Veldkamp, Bernard P.

    2012-01-01

    Assessment can serve different goals. If the aim of testing is to classify respondents into one of multiple levels instead of obtaining a precise estimate of the respondent’s ability, computerized classification testing can be used. This type of testing requires algorithms for item selection and

  19. The lower esophageal sphincter shown by a computerized representation

    NARCIS (Netherlands)

    Bemelman, W. A.; van der Hulst, V. P.; Dijkhuis, T.; van der Hoeven, C. W.; Klopper, P. J.

    1990-01-01

    The clinical application of manometry of the lower esophageal sphincter (LES) remains controversial. This is because of the large variability in recorded lower esophageal sphincter pressure (LESP). In this paper a computerized method providing a three-dimensional image of the LES is described. In 12

  20. Who Can Afford a Computerized Bookstore? Almost Anyone.

    Science.gov (United States)

    Miller, Charles

    1982-01-01

    The decision to computerize operations in the DeAnza Community College bookstore was followed by a number of decisions about financing options (purchase, lease/purchase, lease, timesharing). A timesharing agreement was reached with a local bank, with no capital outlay and at a cost equivalent to an accountant's position. (MSE)