Bilateral Ureteral Stenosis with Hydronephrosis as First Manifestation of Granulomatosis with Polyangiitis (Wegener's Granulomatosis): A Case Report and Review of the Literature. (23rd December 2020)

• Record Type: Journal Article
• Authors: Suillot, Joelle; Bollmann, Jürg; Rotman, Samuel; Descombes, Eric
• Other Names: Pham Phuong Chi Academic Editor.
• Is Part Of: Case reports in nephrology. Volume 2020(2020)
• Publication Date: 2020-12-23
• Subjects: Nephrology -- Periodicals; Kidneys -- Diseases -- Periodicals; Nephrology; Kidneys -- Diseases; Nephrology; Periodicals; Electronic journals; Periodicals; 616.61
• DOI: 10.1155/2020/7189497
• Languages: English
• ISSNs: 2090-6641

Abstract

Abstract : Ureteral stenosis is a rare manifestation of granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis). We report the case of a 76-year-old woman with progressive renal failure in which bilateral hydronephrosis due to ureteral stenosis was the first manifestation of the disease. Our patient also had renal involvement with pauci-immune crescentic glomerulonephritis associated with high titers of anti-proteinase 3 c-ANCAs, but no involvement of the upper or lower respiratory tract. The hydronephrosis and renal function rapidly improved under immunosuppressive therapy with high-dose corticosteroids and intravenous pulse cyclophosphamide. We reviewed the literature and found only ten other reported cases of granulomatosis with polyangiitis/Wegener's granulomatosis and intrinsic ureteral stenosis: in two cases, the presenting clinical manifestation was unilateral hydronephrosis and in only two others was the hydronephrosis bilateral, but this complication developed during a relapse of the disease. This case emphasizes the importance of including ANCA-related vasculitis in the differential diagnosis of unusual cases of unilateral or bilateral ureteral stenosis.