Unusual presentation of Wegener's granulomatosis

We report an unusual case of Wegener's granulomatosis revealed by spleen infarction and complicated by reno-vascular hypertension. A 33-year-old man with a history of spleen infraction and cerebral venous thrombosis was admitted for malignant hypertension, renal failure and nephritic syndrome....

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Bibliographic Details
Published in:La revue de medecine interne 2010-08, Vol.31 (8), p.e16-e18
Main Authors: Jamoussi, S Ketari, Ben Dhaou, B, Boussema, F, Turki, E, Kochbati, S, Cherif, O, Maamar, M, Rokbani, L
Format: Article
Language:fre
Subjects:
Adult
Granulomatosis with Polyangiitis - complications
Granulomatosis with Polyangiitis - diagnosis
Humans
Hypertension, Renovascular - etiology
Intracranial Thrombosis - etiology
Male
Splenic Infarction - etiology
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Summary:We report an unusual case of Wegener's granulomatosis revealed by spleen infarction and complicated by reno-vascular hypertension. A 33-year-old man with a history of spleen infraction and cerebral venous thrombosis was admitted for malignant hypertension, renal failure and nephritic syndrome. On Doppler renal ultrasonography, ostial stenosis of the right renal artery was evidenced. Right kidney was non functional on scintigraphy and the patient underwent a right nephrectomy. Granulomatous vasculitis of the right renal artery was found but anti-neutrophilic cytoplasmic antibodies were absent. The diagnosis of Wegener's granulomatosis was suspected. The patient was treated with cyclophosphamide, corticosteroids and plasma exchanges, and renal function markedly improved.
ISSN:1768-3122
DOI:10.1016/j.revmed.2009.09.034