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Attacks of pericarditis as a manifestation of familial Mediterranean fever (FMF)
1Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer, Israel.
Abstract:
Familial Mediterranean fever (FMF) is characterized by recurrent attacks of febrile serositis. While arthritis, pleuritis and peritonitis are common in FMF, no association of pericarditis with FMF has been described in detail. We retrospectively studied about 4000 FMF patients, using a computer chart review. Pericarditis was diagnosed when patients sustained attacks of pleuritic retrosternal chest pain and had typical findings in the electrocardiogram, echocardiogram or chest radiogram. The incidence and features of pericarditis in FMF were compared to published data. Over a period of 20 years, one or more episodes of pericarditis were recorded in 27 patients, a significantly higher incidence than in the general population (68 vs. 6 per 10(5) per year, p < 0.001). Each patient experienced 1-3 pericarditis attacks, lasting a mean of 4.2 days, accompanied by high temperature and symptoms of FMF attack at another site. The pericarditis attack resolved spontaneously and left no sequelae. FMF patients with pericarditis were comparable to other FMF patients in most demographic and clinical parameters. Pericarditis may be considered another rare manifestation of FMF.
Insights
Familial Mediterranean fever (FMF) can cause pericarditis, a rare inflammation of the heart sac. This study found a significantly higher incidence of pericarditis in FMF patients, suggesting it is another FMF manifestation.
Area of Science:
- Rheumatology
- Cardiology
- Genetics
Background:
- Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by recurrent febrile serositis.
- Common FMF manifestations include arthritis, pleuritis, and peritonitis.
- Pericarditis has not been previously well-described as an FMF complication.
Purpose of the Study:
- To investigate the incidence and clinical features of pericarditis in patients with Familial Mediterranean fever.
- To determine if pericarditis is a significant manifestation of FMF.
Main Methods:
- Retrospective chart review of approximately 4000 FMF patients over a 20-year period.
- Diagnosis of pericarditis based on pleuritic chest pain, and electrocardiogram, echocardiogram, or chest radiogram findings.
- Comparison of pericarditis incidence in FMF patients with general population data.
Main Results:
- Twenty-seven FMF patients experienced one or more pericarditis episodes.
- The incidence of pericarditis in FMF patients was significantly higher (68 per 10^5 per year) than in the general population (6 per 10^5 per year).
- Pericarditis attacks were self-limiting, lasted an average of 4.2 days, and were associated with fever and other FMF symptoms.
Conclusions:
- Pericarditis is a rare but significant manifestation of Familial Mediterranean fever.
- The incidence of pericarditis is notably higher in FMF patients compared to the general population.
- Pericarditis in FMF patients typically resolves without long-term complications.