Familial Mediterranean Fever: A Diagnostic and Therapeutic Challenge

Recurrent pericarditis is a complex and often debilitating clinical entity characterized by repeated episodes of pericardial inflammation following an initial acute insult. The condition poses a significant diagnostic and therapeutic challenge, as its underlying causes span a wide spectrum of etiologies, including autoimmune disorders (such as systemic lupus erythematosus and rheumatoid arthritis), infectious processes (notably viral, bacterial, or tuberculous), neoplastic involvement, and a growing recognition of autoinflammatory mechanisms. While the majority of recurrent pericarditis cases are deemed idiopathic, presumed to reflect a post-viral or immune-mediated phenomenon, there is increasing evidence that a subset of patients may have underlying monogenic autoinflammatory syndromes. We present the case of a middle-aged woman with recurrent, steroid-responsive pericarditis accompanied by episodic fever and elevated inflammatory markers, in whom conventional infectious and autoimmune causes were excluded. Her clinical course raised suspicion of an underlying autoinflammatory mechanism, highlighting the importance of considering familial Mediterranean fever (FMF) and other hereditary periodic fever syndromes in patients with otherwise unexplained, relapsing pericardial disease.