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Multifocal fibrosclerosis combined with idiopathic retro-peritoneal and pericardial fibrosis

Yoko Omura1, Katsunobu Yoshioka, Yuka Tsukamoto

  • 1Department of Internal Medicine, Osaka City General Hospital, Osaka.

Internal Medicine (Tokyo, Japan)
|May 9, 2006
PubMed

Insights

Retroperitoneal fibrosis (RPF) can spread to the pericardium, causing fatal cardiovascular complications. Long-term patient monitoring is crucial for early detection and management of multifocal fibrosclerosis.

Area of Science:

  • Cardiology
  • Pathology
  • Oncology

Background:

  • Retroperitoneal fibrosis (RPF) is a rare condition characterized by excessive fibrous tissue in the retroperitoneum.
  • RPF can lead to serious complications, including ureteral obstruction and, rarely, cardiac involvement.

Observation:

  • A 70-year-old male with known RPF presented with dyspnea due to massive pericardial effusion.
  • Pericardial biopsy revealed fibrosis and lymphocytic infiltration, mirroring RPF findings.
  • Autopsy confirmed multifocal fibrosclerosis affecting multiple organs.

Findings:

  • The patient's condition progressed to cardiovascular failure despite treatment, leading to death.
  • Pericardial fibrosis, a manifestation of multifocal fibrosclerosis, was identified as a fatal complication.
  • Histological findings were consistent with RPF, indicating a systemic fibrotic process.

Implications:

  • This case highlights the potential for retroperitoneal fibrosis to manifest as pericardial fibrosis and multifocal fibrosclerosis.
  • Aggressive treatment for RPF may not prevent systemic progression, underscoring the need for vigilant long-term follow-up.
  • Early recognition and management of systemic fibrotic diseases are critical for improving patient outcomes and preventing fatal complications.

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