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Thoracic and lung involvement in familial Mediterranean fever (FMF)

Merav Lidar1, Mordechai Pras, Pnina Langevitz

  • 1Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer, Israel.

Clinics in Chest Medicine
|July 3, 2002
PubMed

Insights

Familial Mediterranean fever (FMF) rarely involves the lungs, but can manifest as pleuritis, amyloidosis, vasculitis, mesothelioma, or thromboembolism. Further research is needed to confirm any protective effect against asthma.

Area of Science:

  • Rheumatology
  • Pulmonology
  • Genetics

Background:

  • Familial Mediterranean fever (FMF) is a genetic autoinflammatory disorder.
  • Lung involvement in FMF is typically limited to transient pleuritis during acute attacks.
  • Other pulmonary manifestations are rare but recognized complications.

Purpose of the Study:

  • To review and summarize the spectrum of lung involvement in Familial Mediterranean fever.
  • To discuss rare pulmonary complications associated with FMF.
  • To explore potential associations between FMF and asthma.

Main Methods:

  • Literature review of reported cases and studies on FMF and lung involvement.
  • Analysis of the association between FMF and pulmonary conditions like amyloidosis, vasculitis, mesothelioma, and asthma.
  • Discussion of underlying mechanisms and genetic factors.

Main Results:

  • Lung involvement in FMF is predominantly transient pleuritis.
  • Rare manifestations include pulmonary amyloidosis, vasculitis (except one isolated case), and mesothelioma.
  • An inverse association with asthma is suggested but not established, possibly due to genetic linkage or eosinophil function.
  • Thromboembolism is a consideration in FMF amyloidosis patients with respiratory distress.

Conclusions:

  • While FMF primarily affects the pleura, rare but serious pulmonary complications can occur.
  • Pulmonary amyloidosis, vasculitis, mesothelioma, and thromboembolism are potential FMF-related lung issues.
  • The relationship between FMF and asthma requires further investigation.

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