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Mixed connective tissue disease and idiopathic retroperitoneal fibrosis: A rare but important association

Thomas Neerhut1, Alexander Mills1, Samuel Davies1

  • 1Princess Alexandra Hospital, Metro South Health Brisbane, PA Hospital, 199 Ipswich Road, Woolloongabba, QLD, 4102, Australia.

Urology Case Reports
|February 11, 2022
PubMed

Insights

Idiopathic Retroperitoneal fibrosis (RPF), a rare fibro-inflammatory condition, was uniquely observed in a patient with mixed connective tissue disease (MCTD). This case highlights potential links and the need for high clinical suspicion for diagnosis.

Area of Science:

  • Rheumatology
  • Nephrology
  • Pathology

Background:

  • Idiopathic Retroperitoneal fibrosis (RPF) is a rare fibro-inflammatory condition.
  • Mixed connective tissue disease (MCTD) is an autoimmune disorder with overlapping features of other connective tissue diseases.
  • RPF is rarely described in patients with pre-existing MCTD.

Observation:

  • This report details a unique case presentation of RPF in a patient with known MCTD.
  • The patient exhibited an unusual clinical manifestation of RPF.

Findings:

  • The case illustrates a rare occurrence of RPF in the context of MCTD.
  • Potential pathophysiological links between RPF and MCTD are suggested.

Implications:

  • Diagnosis of RPF in MCTD patients requires a high level of clinical suspicion.
  • Further research into the relationship between RPF and MCTD is warranted.
  • Understanding these links may improve diagnostic and therapeutic strategies for patients with overlapping autoimmune and fibrotic conditions.
Keywords:
Mixed connective tissue diseaseRetroperitoneal fibrosisVasculitis

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