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Abstract
Wegener’s granulomatosis (WG) is a systemic inflammatory disease whose histopathologic features often include necrosis, granuloma formation, and vasculitis of small-to-medium-sized vessels. We report a new case of Wegener’s granulomatosis occurring in a 41-year-old man who was admitted for respiratory symptoms, fever, asthenia, night sweats and weight loss since one month. Chest X-ray and computed tomography showed multiple bilateral pulmonary excavated lesions. A trransparietal lung biopsy was performed. Histological examination showed an area of necrosis, surrounded by a palisade of histiocytes. Some of the histiocytes were multinucleated and formed true granulomas within the wall of the adjacent pulmonary artery. Serology was strongly positive for ANCA. A diagnosis of Wegener granulomatosis was conducted and the patient was started on cyclophosphamide and methylprednisolone as an initial treatment, with a favorable evolution.
Highlights
Wegener’s granulomatosis (WG) is a rare disease of uncertain cause characterized by necrotizing granulomatosis vasculitis of small and medium-sized vessels [1]
Wegener’s granulomatosis is a systemic vasculitic syndrome, which affects mainly the upper and lower airways [3]. It was first described by Klinger in 1933, followed by other investigators, including Rossle in 1933, Wegener in 1936 and 1939, and Ringertz in 1947 [2]
Fever and weight loss may be reported at the onset of the disease and more frequently during the course of the illness [1,2,5]
Summary
Wegener’s granulomatosis (WG) is a rare disease of uncertain cause characterized by necrotizing granulomatosis vasculitis of small and medium-sized vessels [1]. It is distinguished clinically by its predilection for affecting the upper and lower respiratory tracts and kidneys and by the histologic presence of necrosis, granulomatous inflammation, and vasculitis [2]
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