Idiopathic retroperitoneal fibrosis is a macrophage-rich process. Implications for its pathogenesis and treatment.

Journal Article (Journal Article)

Idiopathic retroperitoneal fibrosis (IRF) is an uncommon disease of obscure etiology and pathobiology. Using sections of frozen and paraffin-embedded tissue, an immunohistochemical technique, and antibodies to a variety of macrophage- and lymphocyte-associated antigens, we studied six examples of IRF. The results showed a large population of spindle-shaped cells that expressed the immunophenotype of a tissue macrophage, that is, Leu 3a,b (CD4)+, MY7 (CD13)+, Leu M5 (CD11c)+, KP-1 and EBM-11 (CD68)+, human leukocyte antigen (HLA-DR)+, leukocyte common antigen (CD45)+, HAM-56+, and MAC387+. A subpopulation of these cells also reacted with an antibody to the "activation" antigen, interleukin 2R (CD25). A control group of "fibroblastic" lesions including keloids, desmoid tumors, and an aggressive fibromatosis displayed minimal reactivity with this panel of antibodies. The abundance of macrophages suggests that they may play an important role in the pathogenesis of IRF. If, as has been suggested by some studies, IRF is an immune-mediated phenomenon, the macrophages may be triggered to produce cytokines that stimulate fibroblast proliferation and subsequent fibrosis that characterize this disease.

Full Text

Duke Authors

Cited Authors

  • Hughes, D; Buckley, PJ

Published Date

  • May 1993

Published In

Volume / Issue

  • 17 / 5

Start / End Page

  • 482 - 490

PubMed ID

  • 8470762

International Standard Serial Number (ISSN)

  • 0147-5185

Digital Object Identifier (DOI)

  • 10.1097/00000478-199305000-00007


  • eng

Conference Location

  • United States