Idiopathic retroperitoneal fibrosis: Clinicopathologic features and differential diagnosis

D. Corradi, R. Maestri, A. Palmisano, S. Bosio, P. Greco, L. Manenti, S. Ferretti, R. Cobelli, G. Moroni, A. P. Dei Tos, C. Buzio, A. Vaglio

Research output: Contribution to journalArticlepeer-review

Abstract

Idiopathic retroperitoneal fibrosis (IRF) is a rare disease often causing obstructive uropathy. We evaluated the clinicopathologic features of 24 patients with IRF to characterize the histopathology of the disease and to provide a framework for the differential diagnosis with other retroperitoneal fibrosing conditions. Retroperitoneal specimens were analyzed by light and electron microscopy and by immunohistochemistry. Most patients presented with abdominal/lumbar pain, constitutional symptoms, and high acute-phase reactants. Overall, 20 had ureteral involvement and 13 developed acute renal failure. The retroperitoneal tissue consisted of a fibrous component and a chronic inflammatory infiltrate with the former characterized by myofibroblasts within a type-I collagen matrix. The infiltrate displayed perivascular and diffuse patterns containing lymphocytes, macrophages, plasma cells, and eosinophils. The perivascular aggregates had a central core of CD20+ cells and a mantle of CD3+ cells in equal proportions. In the areas of diffuse infiltrate, CD3+ cells outnumbered the CD20+ cells. Most plasma cells were positive for the IgG4 isotype. Small vessel vasculitis was found in the specimens of 11 patients. Our study indicates that a sclerotic background with myofibroblasts associated with a diffuse and perivascular infiltrate mainly consisting of T and B lymphocytes may be a pathological hallmark of IRF.

Original languageEnglish
Pages (from-to)742-753
Number of pages12
JournalKidney International
Volume72
Issue number6
DOIs
Publication statusPublished - Sep 2007

Keywords

  • Acute renal failure
  • Fibrosis
  • Histopathology
  • Inflammation
  • Obstructive uropathy
  • Vasculitis

ASJC Scopus subject areas

  • Nephrology

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