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Recurrent pericarditis as the initial manifestation of Familial Mediterranean fever
Kerem Okutur1, Selcuk Seber, Erkan Oztekin
1Sisli Etfal Research and Training Hospital Department of Internal Medicine, Istanbul, Turkey. keremokutur007@yahoo.com
Background:
Familial Mediterranean fever is an autosomal recessive disease largely restricted to certain ethnic groups and presenting with recurrent febrile serositis attacks. Peritonitis, pleuritis, and synovitis are common manifestations; however, the pericardium is rarely affected.
Case Report:
In this case report, we describe a 25-year-old Turkish woman who presented with recurrent pericarditis of no obvious cause, which eventually responded to colchicine therapy. Using gene mutation analysis to detect the MEFV gene, the patient's condition was finally diagnosed as Familial Mediterranean fever.
Conclusions:
Familial Mediterranean fever should be considered in patients with idiopathic recurrent pericarditis unresponsive to nonsteroidal anti-inflammatory medications and corticosteroids. Mutation analyses should be done.
Insights
Familial Mediterranean fever (FMF) can cause rare cases of recurrent pericarditis. Early diagnosis through MEFV gene mutation analysis and colchicine treatment are key for effective management.
Area of Science:
- Rheumatology
- Genetics
- Internal Medicine
Background:
- Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder.
- Characterized by recurrent febrile serositis attacks, primarily affecting the peritoneum, pleura, and synovium.
- Pericardial involvement is an uncommon manifestation of FMF.
Observation:
- A case report of a 25-year-old Turkish woman with recurrent pericarditis of unknown etiology.
- The patient's pericarditis was refractory to conventional treatments like NSAIDs and corticosteroids.
- The patient experienced a positive response to colchicine therapy.
Findings:
- Genetic analysis identified mutations in the MEFV gene, confirming the diagnosis of FMF.
- The patient's recurrent pericarditis was attributed to FMF.
Implications:
- FMF should be considered in the differential diagnosis of idiopathic recurrent pericarditis, especially in individuals unresponsive to standard treatments.
- MEFV gene mutation analysis is crucial for diagnosing FMF in such cases.
- Colchicine therapy can be effective for managing FMF-related pericarditis.
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