Atypical Presentation of Granulomatosis with Polyangiitis

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An Unusual Presentation of Granulomatosis with Polyangiitis

The granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a pauci-immune vasculitis involving small- and medium-sized blood vessels. Classically, the patients inflicted with this disease present with a triad of necrotizing granulomatous inflammation of the respiratory tract, cutaneous necrotizing vasculitis, and glomerulonephritis (GN). The antinuclear (ANA) and...

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Granulomatosis with polyangiitis (GPA) is a multi-organ disease which mostly affects lungs, kidney, and head and neck region. We report a rare case of acute laryngeal dyspnea and rapidly progressive pulmonary changes as first manifestations of disease. A 53 year-old woman presented with symptoms of two-week dyspnea, which aggravated rapidly in the preceding hours. Laryngological examination rev...

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A Presentation of Cerebritis Secondary to Granulomatosis with Polyangiitis (Wegener)

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 ...

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Granulomatosis With Polyangiitis (Wegener’s)

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 cyclop...

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ژورنال

عنوان ژورنال: Journal of the Belgian Society of Radiology

سال: 2018

ISSN: 2514-8281

DOI: 10.5334/jbsr.1623