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[Idiopathic retroperitoneal fibrosis]
1Dipartimento di Clinica Medica, Nefrologia e Scienze della Prevenzione, Università di Parma, Parma.
Summary
Idiopathic retroperitoneal fibrosis (IRF) involves tissue hardening in the abdomen, often requiring medical and surgical treatment. Early intervention with steroids or tamoxifen can improve outcomes for patients with this rare condition.
Area of Science:
- Retroperitoneal fibrosis is an uncommon condition.
- Characterized by fibrosis and inflammation in retroperitoneal tissue.
Context:
- Idiopathic retroperitoneal fibrosis (IRF) accounts for two-thirds of cases.
- Secondary causes include cancer, infections, drugs, autoimmune diseases, and vasculitis.
- IRF presents as a fibrous plaque engulfing adjacent structures like ureters.
Purpose:
- To describe the characteristics, pathogenesis, symptoms, and treatment of retroperitoneal fibrosis.
- To highlight the role of medical and surgical interventions in managing IRF.
Summary:
- Early IRF shows lymphocyte, plasma cell, and macrophage infiltrates.
- Advanced IRF is acellular with abundant collagen and calcifications.
- Pathogenesis is unknown, possibly autoimmune or related to atherosclerosis.
Impact:
- Non-specific symptoms include fever, anorexia, weight loss, and lumbar/abdominal pain.
- Treatment involves surgery for complications and medical therapy (steroids, tamoxifen).
- Other agents like azathioprine, methotrexate, and cyclosporine are used for non-responders.