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Features and Prognosis of Patients With Retroperitoneal Fibrosis Developing Fibrosing Mediastinitis: Case-Control

Yishan Ding1,2, Zhiyan Li3, Shibo Liu4

  • 1Department of Research and Education, Peking University International Hospital, Beijing, China.

ACR Open Rheumatology
|July 29, 2025
PubMed
Abstract

Insights

Retroperitoneal fibrosis (RPF) with mediastinal fibrosis (MF) is rare and fatal. Early detection of specific symptoms like emaciation and dyspnea, along with glucocorticoid treatment, may improve outcomes for RPF and MF patients.

Area of Science:

  • Medical Science
  • Pathology
  • Clinical Medicine

Background:

  • Retroperitoneal fibrosis (RPF) complicated by mediastinal fibrosis (MF) is an uncommon but often fatal condition.
  • Understanding the distinct clinical features and prognostic indicators of RPF with MF is crucial for patient management.

Purpose of the Study:

  • To investigate the specific clinical characteristics of patients with RPF and MF.
  • To identify indicators of poor prognosis in this patient population.

Main Methods:

  • A systematic review of literature and a case-control study were conducted.
  • Patients with idiopathic RPF were recruited, with a focus on those also presenting with MF.
  • Comparison was made between patients with RPF and MF and those with RPF alone, and subsequently between deceased and surviving patients with RPF and MF.

Main Results:

  • Patients with RPF and MF exhibited unique symptoms such as emaciation, fever, pericardial effusion, pleurisy, and dyspnea, alongside hyperglobulinemia.
  • Low back pain was less common in patients with RPF and MF compared to those with RPF alone.
  • Pleural effusions and lack of glucocorticoid treatment were identified as risk factors for mortality in patients with RPF and MF.

Conclusions:

  • Retroperitoneal fibrosis (RPF) co-occurring with mediastinal fibrosis (MF) presents with specific clinical features and a poor prognosis.
  • Prompt identification of these features and initiation of glucocorticoid-based therapy are recommended to enhance patient outcomes.

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