Pulmonary Manifestations in Familial Mediterranean Fever

Although probably existing since ancient times, familial Mediterranean fever (FMF) was not recognized as a unique entity until 1945, when Siegal (1) described 10 cases of ‘‘benign paroxysmal peritonitis.’’ Since then, this condition has been given a variety of names (e.g., hereditary recurrent polyserositis, periodic disease, Armenian disease), emphasizing its genetic and episodic nature and the geographic distribution. FMF is characterized by recurrent episodes of fever and serositis, involving primarily the peritoneum (affecting 95% of patients), the pleura (40% of patients) and the synovial membrane of the joints (75% of patients). Typical febrile episodes of skin rash (erysipelas-like erythema), pericarditis, scrotal pain, and myalgia occurless frequently. The poorly understood leg pain, together with the rare manifestation of protracted febrile myalgia, the uncommon development of chronic arthritis and the nephropathic AA amyloidosis complete the clinical spectrum (2).