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Pulmonary associations in familial Mediterranean fever
A Livneh1, P Langevitz, M Pras
1Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer, Israel.
Abstract:
Familial Mediterranean fever (FMF) is a hereditary periodic fever syndrome expressed by acute episodes of fever and painful manifestations. In this report, the pulmonary manifestations of FMF are reviewed, the most prominent of which are chest attacks due to pleuritis. Nephropathic amyloidosis of the AA type, which complicates FMF in most untreated patients, may progress to affect other organs, including the lungs, but this rarely produces noticeable symptoms. The common association between FMF and vasculitis makes pulmonary hemorrhage, infarction, or infiltrates highly possible. These complications, however, have been reported only rarely. Asthma was found to occur less often than expected in patients with FMF, but methodologic faults make this finding doubtful. Finally, the occurrence of mesothelioma in five patients with FMF who were not exposed to asbestos suggests a role for recurrent FMF serositis in the pathogenesis of this malignancy.
Insights
Familial Mediterranean fever (FMF) can cause lung issues like pleuritis and, rarely, complications from amyloidosis or vasculitis. Recurrent FMF serositis may also link to mesothelioma development.
Area of Science:
- Pulmonology
- Rheumatology
- Genetics
Background:
- Familial Mediterranean fever (FMF) is an inherited autoinflammatory disorder characterized by recurrent febrile episodes and serositis.
- Pulmonary involvement in FMF is often underrecognized, despite significant potential morbidity.
Purpose of the Study:
- To review the spectrum of pulmonary manifestations associated with Familial Mediterranean fever.
- To explore the potential link between FMF and thoracic malignancies.
Main Methods:
- Literature review of pulmonary manifestations in FMF.
- Analysis of case reports and cohort studies detailing FMF complications.
- Examination of the association between FMF, serositis, and mesothelioma.
Main Results:
- Pleuritis is the most common pulmonary manifestation of FMF, presenting as chest attacks.
- AA type nephropathic amyloidosis can affect the lungs in FMF, though typically asymptomatic.
- Pulmonary hemorrhage, infarction, and infiltrates are possible due to FMF-associated vasculitis but are rarely reported.
- A potential association between recurrent FMF-related serositis and the development of mesothelioma in asbestos-unexposed patients was observed.
Conclusions:
- Pulmonary manifestations, particularly pleuritis, are significant in FMF.
- While rare, lung complications from amyloidosis and vasculitis can occur.
- Recurrent serositis in FMF may play a role in the pathogenesis of mesothelioma.