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Constrictive pericarditis in familial Mediterranean fever
Abstract:
Fibrosing peritonitis and constrictive pericarditis occurred in an 18-year-old patient who manifested the classic features of familial Mediterranean fever (FMF). Pericardial calcification had been present in chest X-rays taken when the patient was five years old. Intermittent intestinal obstruction and congestive heart failure were relieved by appropriate surgical intervention, but attacks of FMF subsided only after colchicine therapy. This is the first instance of nonuremic pericarditis in our experience with over 1,000 FMF patients. Critical analysis of the reported cases in which pericarditis was attributed to FMF strengthens our belief that the occurrence of pericarditis in a patient with FMF probably represents a fortuitous intercurrent disease.
Insights
Familial Mediterranean Fever (FMF) can cause serious complications like constrictive pericarditis. However, pericarditis in FMF patients may often be unrelated to the disease itself.
Area of Science:
- Rheumatology
- Internal Medicine
- Genetics
Background:
- Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
- FMF is characterized by recurrent episodes of fever and serositis.
- Pericarditis is a rare but serious potential complication.
Observation:
- An 18-year-old patient with classic FMF symptoms developed fibrosing peritonitis and constrictive pericarditis.
- The patient had pre-existing pericardial calcification noted at age five.
- Gastrointestinal and cardiac symptoms improved with surgery, but FMF attacks resolved with colchicine.
Findings:
- This case represents the first documented instance of nonuremic pericarditis in over 1,000 FMF patients observed by the authors.
- A critical review of existing literature suggests pericarditis in FMF patients is often coincidental.
Implications:
- The occurrence of pericarditis in FMF patients warrants careful evaluation to distinguish between FMF-related and unrelated causes.
- While FMF can have systemic inflammatory effects, pericarditis may frequently be an incidental comorbidity.
- Further research is needed to clarify the precise relationship between FMF and pericardial disease.