Unusual renal manifestations of Wegener's granulomatosis. Report of two cases. 1978

S B Baker, and D R Robinson

Two patients with previously undescribed renal manifestations of Wegener's granulomatosis are described. A 24 year old man, who presented with typical necrotizing granulomatous sinus disease and cavitary pulmonary lesions, had multiple bilateral renal arterial aneurysms demonstrated angiographically. One of these aneurysms ruptured, leading to a massive perinephric hematoma. The bleeding artery was successfully occluded with Gelfoam embolization, thereby obviating the need for nephrectomy. A 60 year old woman presented with glomerulonephritis and mononeuritis multiplex two years before the development of classic necrotizing granulomatous inflammation of her sinuses and nose, along with pulmonary nodules. In addition, her left ureter became obstructed due to necrotizing vasculitis of the periureteral vessels. Both patients responded dramatically to cyclophosphamide therapy. The diagnosis of Wegener's granulomatosis should be considered in patients who present with multiple renal aneurysms, a spontaneous perinephric hematoma, necrotizing glomerulitis or ureteral obstruction due to vasculitis, even though the characteristic granulomatous respiratory involvement may be absent at that time. It is important to recognize these unusual renal manifestations as features of Wegener's granulomatosis because of the therapeutic efficacy of cytotoxic immunosuppressive agents in this disease.

UI MeSH Term Description Entries
D007668 Kidney Body organ that filters blood for the secretion of URINE and that regulates ion concentrations. Kidneys
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000783 Aneurysm Pathological outpouching or sac-like dilatation in the wall of any blood vessel (ARTERIES or VEINS) or the heart (HEART ANEURYSM). It indicates a thin and weakened area in the wall which may later rupture. Aneurysms are classified by location, etiology, or other characteristics. Saccular Aneurysm,Fusiform Aneurysm,Aneurysm, Fusiform,Aneurysms,Aneurysms, Fusiform,Fusiform Aneurysms
D012422 Rupture, Spontaneous Tear or break of an organ, vessel or other soft part of the body, occurring in the absence of external force. Ruptures, Spontaneous,Spontaneous Rupture,Spontaneous Ruptures
D014513 Ureter One of a pair of thick-walled tubes that transports urine from the KIDNEY PELVIS to the URINARY BLADDER. Ureters
D014652 Vascular Diseases Pathological processes involving any of the BLOOD VESSELS in the cardiac or peripheral circulation. They include diseases of ARTERIES; VEINS; and rest of the vasculature system in the body. Disease, Vascular,Diseases, Vascular,Vascular Disease

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