Korean J Nephrol.  2009 May;28(3):248-252.

A Case of Idiopathic Retroperitoneal Fibrosis Associated with Rapidly Progressive Glomerulonephritis with Anti-myeloperoxidase Antineutrophil Cytoplasmic Antibodies

Affiliations
  • 1Department of Medicine, Samsung Medical Center Sungkyunkwan University School of Medicine, Seoul, Korea. yoongoo.kim@samsung.com

Abstract

Idiopathic retroperitoneal fibrosis is a rare disease characterized by the presence of retroperitoneal fibroinflammatory tissue, which often surrounds the abdominal aorta, the ureters, or other abdominal organs. There have been sporadic reports of an association with autoimmune diseases, although the pathogenesis of idiopathic retroperitoneal fibrosis is unclear. However, there are very few reports ofidiopathic retroperitoneal fibrosis associated with rapidly progressive glomerulonephritis. We report a case with idiopathic retroperitoneal fibrosis associated with rapidly progressive glomerulonephritis, and anti-myeloperoxidase antineutrophil cytoplasmic antibodies (anti MPO-ANCA), that was improved by the combination therapy of steroids and cyclophosphamide.

Keyword

Retroperitoneal fibrosis; Glomerulonephritis; Antineutrophil cytoplasmic antibodies

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Aorta, Abdominal
Autoimmune Diseases
Cytoplasm
Glomerulonephritis
Rare Diseases
Retroperitoneal Fibrosis
Steroids
Ureter
Antibodies, Antineutrophil Cytoplasmic
Steroids
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