Idiopathic Retroperitoneal Fibrosis: A Systemicconnective Tissue Disease?

Abstract

Renal histology of a patient with idiopathic retroperitoneal fibrosis demonstrated a proliferative crescentic glomerulonephritis with intramembranous electron-dense deposits. These findings were interpreted as being consistent with an immune complex glomerulonephritis. Serologic studies revealed a positive antismooth muscle antibody titer of 1:80 and a weakly positive antinuclear antibody titer of 1:40. No distinct systemic disease was identified. While fibrosis of the retroperitoneum can occur in association with a number of distinct pathologic conditions and pharmacologic agents, the mechanism responsible for the development of the idiopathic variety of retroperitoneal fibrosis remains unclear. Our observation of an immune complex glomerulonephritis and the cumulative data on idiopathic retroperitoneal fibrosis are consistent with the concept that the fibrosis may be a local expression of an immunologically mediated systemic disease.

ANCA‐positive periaortic vasculitis: does it fall within the spectrum of vasculitis?

“…Retroperitoneal fibrosis (RPF) is a connective tissue disorder whose primary pathological characteristic is periaortic collagen deposition [1].…”

Treatment of idiopathic retroperitoneal fibrosis with tamoxifen

“…Moreover, [1][2][3][4][5] is infrequent and must be distinguished from there was no indication of tumours or metastases. A other retroperitoneal processes [6][7][8][9][10][11][12][13]. Administration weak signal from the fibrous tissue was obtained with of steroids is effective, but causes serious side-effects.…”

Case 27-1996

“…12 Idiopathic retroperitoneal fibrosis is probably immunologically mediated, in view of its response to corticosteroid and immunosuppressive therapy and its occasional association with other immune-mediated diseases. [13][14][15] Immunohistochemical studies have shown that the inflammatory infiltrate is composed The New England Journal of Medicine of B lymphocytes, T lymphocytes that are predominantly of the T-helper phenotype, and macrophages, as in this case. The presence of several cytokines and adhesion molecules has also been demonstrated, confirming the active nature of the inflammatory infiltrate and supporting an immune-mediated mechanism.…”