Attacks of severe dysmenorrhea as the sole manifestation of Familial Mediterranean Fever (FMF)

Familial Mediterranean Fever (FMF) is an autosomal recessive disease (MEFV gene) characterized by recurrent fever and inflammatory serositis. Although majority of patients have random pattern of attacks, some reports described precipitating factors. A literature review indicated that FMF attacks occurring only during menstruation are rarely seen. We report the cases of three patients with severe dysmenorrhoic pain as unusual clinical presentation of FMF. They were 3 females with a mean age at onset of 12 years. They never had typical attacks of fever and abdominal or chest pain, but they suffered from regular and severe dysmenorrhoic pain. Leukocytosis and Creactive protein (CRP) elevation were noted during these attacks in all patients. Unlike dysmenorrhoea, none of these patients' attacks responded to non-steroidal antiinflammatory drugs. The diagnosis of FMF was based on typical clinical and laboratory features. On investigation of MEFV, M694V was the most frequent mutation