Bilateral staged ureteral stenosis secondary to Wegener’s disease: A case report

Wegener's disease is a rare vasculitis characterized by inflammation of the endometrium of blood vessels. Ureteral stenosis is a rare manifestation of granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis). We report the case of a 38-year-old woman with acute renal failure in which bilateral hydronephrosis due to staged ureteral stenosis was the second manifestation of the disease after necrotico-inflammatory skin lesions. Our patient had high titers of anti-proteinase 3 c-ANCA, but no upper or lower respiratory tract damage was demonstrated. Hydronephrosis and renal function improved rapidly under immunosuppressive therapy with high dose of corticosteroids. We reviewed the literature and found only ten other reported cases of Wegener's granulomatosis and intrinsic ureteral stenosis: in two cases the presenting clinical manifestation was unilateral hydronephrosis and in only three others bilateral hydronephrosis. All of these complications have developed on disease relapse except in one case where the first manifestation of vasculitis is bilateral ureteral stenosis. This case highlights the importance of including ANCA-related vasculitis in the differential diagnosis of unusual cases of unilateral or bilateral ureteral stenosis.