Wegener’s granulomatosis and retroperitoneal fibrosis: a case report and review of the literature

  • Lia Salvati | liasal88@gmail.com Internal Medicine Division, Department of Life, Health and Environmental Sciences, University of L’Aquila, Italy.
  • Valeria Ludovici Internal Medicine Division, Department of Life, Health and Environmental Sciences, University of L’Aquila, Italy.
  • Giuliana Properzi Internal Medicine Division, Department of Life, Health and Environmental Sciences, University of L’Aquila, Italy.
  • Laura Natali Internal Medicine Division, S. Salvatore Hospital, L’Aquila, Italy.
  • Angelo Viscido Internal Medicine Division, Department of Life, Health and Environmental Sciences, University of L’Aquila, Italy.
  • Claudio Ferri Internal Medicine Division, Department of Life, Health and Environmental Sciences, University of L’Aquila, Italy.

Abstract

Granulomatosis with polyangiitis (GPA), also known as Wegener’s granulomatosis (WG), is a rare systemic auto-inflammatory disease characterized by necrotizing granulomatous inflammation and antineutrophil cytoplasmic antibodies-associated small vessel vasculitis. Retroperitoneal fibrosis (RPF) is an uncommon collagen vascular disease of unclear etiology, characterized by a chronic non-specific inflammation of the retroperitoneum, which can entrap and obstruct retroperitoneal structures, notably abdominal aorta, iliac arteries and ureters. RPF is mostly idiopathic, but an association with vasculitis has been shown. However, only few cases of retroperitoneal fibrosis with ureterohydronephrosis secondary to GPA have been described in literature. The outcome seems to be better when compared to the idiopathic form, and immunosuppressive treatment demonstrated to be effective, with low need in surgery. We provide the case report of a 57-year-old patient, affected by GPA, referred to our Division for massive vein thrombosis and ureteral stenosis secondary to retroperitoneal fibrosis. We also provide a review of the literature currently available on this topic.

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Published
2019-06-18
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Section
Case Reports
Keywords:
Vasculitis, retroperitoneal fibrosis, rheumatology, ureterohydronephrosis, tamoxifen.
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How to Cite
Salvati, L., Ludovici, V., Properzi, G., Natali, L., Viscido, A., & Ferri, C. (2019). Wegener’s granulomatosis and retroperitoneal fibrosis: a case report and review of the literature. Italian Journal of Medicine, 13(2), 109-115. https://doi.org/10.4081/itjm.2019.1138