A specific component of familial Mediterranean fever: Menstrual attacks only

Familial Mediterranean fever (FMF) is the prototype of monogenic autoinflammatory diseases. The disease is characterized by recurrent attacks of fever, abdominal pain, and chest pain. Menstruation can be a triggering factor for these attacks. We report two cases of FMF in which abdominal pain attacks occurred exclusively during menstrual periods. A 14-year-old female patient was diagnosed with FMF at the age of 10 after experiencing recurrent episodes of fever and abdominal pain lasting 1–3 days and recurring every 1–2 months since the age of 3. After colchicine treatment, her fever and abdominal pain attacks resolved; however, she reported severe abdominal pain lasting for 3 days during every menstrual period over the past year. The patient was considered to have FMF characterized by isolated menstrual attacks. The family acknowledged that the patient was not taking colchicine regularly. With regular colchicine therapy, a significant reduction in isolated menstrual attacks was observed. A 15-year-old female patient was referred because of recurrent abdominal pain over the past 3 years. Upon further questioning, she reported no associated fever or chest pain. It was noted that the abdominal pain occurred specifically during menstrual periods and lasted for 2–4 days. Significant elevations in C-reactive protein levels were detected during episodes of abdominal pain and menstruation. Given her typical history of isolated menstrual attacks, she has remained attack-free for 2 years while receiving colchicine therapy. Although the exact mechanism underlying the relationship between menstruation and FMF attacks remains unclear, FMF attacks typically begin immediately before the onset of menstruation. In some cases, however, the attacks begin just after menstruation starts. These patients appear to have a higher rate of dysmenorrhea and an increased frequency of attacks before and after menarche compared with patients with typical clinical presentations of FMF. Fever may not always be present during attacks; therefore, elevated acute-phase reactant levels may be useful in distinguishing menstrual FMF attacks from dysmenorrhea.

Keywords: Colchicine, familial mediterranean fever, isolated menstrual attacks.