Wegener's Granulomatosis Presenting as Necrosis of the Left Mainstem Bronchus

2002 ◽  
Vol 10 (3) ◽  
pp. 277-279 ◽  
Author(s):  
Paul Schneider ◽  
Jörn Gröne ◽  
Jürgen Braun ◽  
Alejandra Perez-Canto ◽  
Heinz J Buhr
Keyword(s):  
Wegener’S Granulomatosis ◽  
Renal Involvement ◽  
Left Lung ◽  
Wegener's Granulomatosis ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Saddle Nose ◽  
Bronchial Arteries ◽  
Left Mainstem ◽  
Saddle Nose Deformity ◽  
Bronchial Inflammation

A patient with pansinusitis, nasal septum necrosis, and saddle nose deformity showed necrosis of the left mainstem, upper, and lower bronchi, with complete loss of left lung perfusion and ventilation. Pneumonectomy was performed. Histological findings showed extensive necrotizing and granulomatous bronchial inflammation with vasculitis of the bronchial arteries and the pulmonary vein. Wegener's granulomatosis was diagnosed, despite a negative cytoplasmic pattern of antineutrophil cytoplasmic antibodies and the lack of renal involvement.

scholarly journals Orbital presentation of systemic vasculitis: a diagnostic and management challenge

2015 ◽  
Vol 7 (1) ◽  
pp. 65-68 ◽  
Author(s):  
M Singh ◽  
U Singh ◽  
Z Zadeng
Keyword(s):  
Wegener’S Granulomatosis ◽  
Vision Loss ◽  
Systemic Vasculitis ◽  
Wegener's Granulomatosis ◽  
Good Prognosis ◽  
Saddle Nose ◽  
Orbital Mass ◽  
Saddle Nose Deformity ◽  
Ulcerative Lesions ◽  
Management Challenge

Background: Orbital involvement in Wegener’s Granulomatosis (WG) is rare and has an overall good prognosis. Case: A 60-year-old hypertensive Indian female presented with vision loss and painful proptosis of left eye. Orbital incisional biopsy suggested necrotising small vessel inflammation. The saddle nose deformity and pedal nodulo- ulcerative lesions further consolidated the diagnosis of Wegener’s granulomatosis. Systemic immunosuppressant provided remission and the only relapse was managed successfully with intravenous Rituximab. Conclusion: Wegener’s granulomatosis should be kept in the differential diagnosis of painful proptosis with a diffuse orbital mass in an elderly patient presenting with profound vision loss. Newer immune modulating agents are useful adjuncts in preventing relapses of this fatal disease.

scholarly journals Unusual Heart Involvement of Wegener's Granulomatosis and Literature Review

2016 ◽  
Vol 8 ◽  
Author(s):  
Esther Cambronero-Cortinas ◽  
Miguel Jose Corbi-Pascual ◽  
Gonzalo Gallego-Sanchez ◽  
Nuria Baxeiras-Gonzalez ◽  
Tomas Cros Ruiz de la Galarreta ◽  
...  
Keyword(s):  
Heart Failure ◽  
Mitral Regurgitation ◽  
Wegener’S Granulomatosis ◽  
Renal Involvement ◽  
Wegener's Granulomatosis ◽  
Saddle Nose ◽  
Severe Mitral Regurgitation ◽  
Atrioventricular Junction ◽  
Posterior Mitral Leaflet ◽  
Coronary Care

<p>A female patient, 60 years of age, was presented to our hospital with chest pain and monomorphic ventricular tachycardia (VT). She was transferred to the Coronary Care Unit and amiodarone perfusion restored basal rhythm in atrial fibrillation. She has not sign of heart failure. A transtoracic echocardiogram (TTE) was performed and an one mitral mass was found at atrioventricular junction with displacement of the posterior mitral leaflet A transesophageal echocardiogram (TEE) demonstrated a mass at atrioventricular junction level with severe mitral regurgitation. Cardiac Magnetic Resonance (CMR) confirmed the mass and anterolateral papillary muscle was thickening and hypertrophied with hyperenhancement consistent with fibrosis.  Moreover, T2-weighted imaging demonstrated hyperintense mass with respect to the surrounding myocardium in relation of inflammatory mass. She had saddle nose by destruction of the septum, bilateral hearing loss, sinusitis and scleritis and renal involvement as well. This patient was diagnosed of Wegener's Granulomatosis (WG) and she was treated with methylprednisolone during 3 days, continued with prednisolone and cyclophosphamide. An 8 days later echocardiogram did not find the mass. However, the patient developed symptomatology of heart failure and in the context of severe mitral regurgitation, mitral valve replacement was decided in multi-disciplinary Cardiology-Cardiothoracic meeting.</p>

scholarly journals Successful Treatment of ANCA-Negative Wegener's Granulomatosis with Rituximab

2010 ◽  
Vol 2010 ◽  
pp. 1-2 ◽  
Author(s):  
Afsha Khan ◽  
Catherine A. Lawson ◽  
Mark A. Quinn ◽  
Amanda H. Isdale ◽  
Michael J. Green
Keyword(s):  
B Cell ◽  
Small Proportion ◽  
Wegener’S Granulomatosis ◽  
Systemic Vasculitis ◽  
Renal Involvement ◽  
Wegener's Granulomatosis ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
B Cell Depletion ◽  
Cd20 Antibody ◽  
Anti Cd20

Wegener's Granulomatosis (WG) is a systemic vasculitis typically associated with antineutrophil cytoplasmic antibodies (ANCAs). A small proportion of patients are ANCA negative, however, and this is more commonly found in individuals with disease limited to the ears, nose, throat, and lungs, who do not have renal involvement. Rituximab is a monoclonal anti-CD20 antibody that has been demonstrated to be effective in the treatment of autoantibody-associated rheumatic diseases, including systemic WG. We report the case of a patient with ANCA-negative WG who responded well to rituximab, illustrating that even in the absence of detectable autoantibodies, B-cell depletion can be effective.

scholarly journals Wegener’s granulomatosis mimicking a parotid abscess

2005 ◽  
Vol 119 (9) ◽  
pp. 746-749 ◽  
Author(s):  
G L Jones ◽  
A D Lukaris ◽  
H V Prabhu ◽  
M J K M Brown ◽  
J Bondeson
Keyword(s):  
Salivary Gland ◽  
Parotid Gland ◽  
Wegener’S Granulomatosis ◽  
Wegener's Granulomatosis ◽  
Clinical Suspicion ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Surgical Exploration ◽  
First Case

We present the case of a previously healthy 59-year-old man who was under treatment for scleritis and episcleritis when he developed a parotid-gland swelling and pus-producing sinus. On surgical exploration, the features were those of a parotid abscess, but the lesion not only failed to heal post-operatively but increased in size very significantly. There was also severe necrotizing keratitis of the eyes. Due to clinical suspicion and a positive antineutrophil cytoplasmic antibodies test, Wegener’s granulomatosis was diagnosed and the patient successfully treated with cyclophosphamide and steroids. Previously, a number of cases of Wegener’s granulomatosis causing salivary-gland swelling have been reported in the literature; this is the first case in which the disease has masqueraded as a parotid abscess.

scholarly journals Wegener's granulomatosis: clinical course in 108 patients with renal involvement

2000 ◽  
Vol 15 (5) ◽  
pp. 611-618 ◽  
Author(s):  
Knut Aasarød ◽  
Bjarne M. Iversen ◽  
Jens Hammerstrøm ◽  
Leif Bostad ◽  
Lars Vatten ◽  
...  
Keyword(s):  
Wegener’S Granulomatosis ◽  
Clinical Course ◽  
Renal Involvement ◽  
Wegener's Granulomatosis

scholarly journals A Case of Wegener’s Granulomatosis Presenting with Unilateral Facial Nerve Palsy

2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Roy Ujjawal ◽  
Pan Koushik ◽  
Panwar Ajay ◽  
Chakrabarti Subrata
Keyword(s):  
Facial Nerve ◽  
Wegener’S Granulomatosis ◽  
Nerve Palsy ◽  
Acute Inflammation ◽  
Granulomatosis With Polyangiitis ◽  
Medical Literature ◽  
Facial Nerve Palsy ◽  
Renal Involvement ◽  
Wegener's Granulomatosis ◽  
Classical Triad

Wegener’s granulomatosis or granulomatosis with polyangiitis is a necrotizing vasculitis affecting both arterioles and venules. The disease is characterized by the classical triad involving acute inflammation of the upper and lower respiratory tracts with renal involvement. However, the disease pathology can affect any organ system. This case presents Wegener’s granulomatosis presenting with facial nerve palsy as the first manifestation of the disease, which is rarely reported in medical literature.

Long-term outcome of 37 patients with Wegener's granulomatosis with renal involvement

2007 ◽  
Vol 36 (5) ◽  
pp. 771-778 ◽  
Author(s):  
Jacques-Eric Gottenberg ◽  
Alfred Mahr ◽  
Christian Pagnoux ◽  
Pascal Cohen ◽  
Luc Mouthon ◽  
...  
Keyword(s):  
Wegener’S Granulomatosis ◽  
Renal Involvement ◽  
Wegener's Granulomatosis ◽  
Term Outcome ◽  
Long Term Outcome

scholarly journals Myeloperoxidase-antineutrophil Cytoplasmic Antibodies with Cytoplasmic Fluorescence Pattern

2010 ◽  
Vol 2 (01) ◽  
pp. 042-043 ◽  
Author(s):  
Seema Chhabra ◽  
Ranjana Walker Minz ◽  
Lekha Goyal ◽  
Nidhi Sharma
Keyword(s):  
Indirect Immunofluorescence ◽  
Wegener’S Granulomatosis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Wegener's Granulomatosis ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Fluorescence Pattern ◽  
Myeloperoxidase Antineutrophil Cytoplasmic Antibody

ABSTRACTWe report here two rare cases of myeloperoxidase–antineutrophil cytoplasmic antibody (MPO-ANCA)-positive Wegener’s granulomatosis (limited variant) which deceptively produced a cytoplasmic (C-ANCA) pattern on indirect immunofluorescence.

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