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

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.