Familial Mediterranean fever and menstruation.

Objective: To study the prevalence, the nature and the genotype correlation of menstruation associated familial Mediterranean fever attacks.

Methods: One hundred and forty-one female patients with familial Mediterranean fever were studied. A questionnaire regarding the presence and nature of menstruation associated with familial Mediterranean fever was designed and filled in by the authors during the patients' visits to the familial Mediterranean fever clinic. The patients who had a positive history for this manifestation were analysed for their familial Mediterranean fever mutations.

Results: Ten out of 141 familial Mediterranean fever female patients (7%) had menstruation-associated familial Mediterranean fever attacks. These patients varied in their disease age of onset and disease duration. Increase of colchicine dose, daily or during the perimenstrual period or oral contraceptives were beneficial in preventing these familial Mediterranean fever attacks. No correlation was found with specific mutations causing familial Mediterranean fever.

Conclusions: Menstruation-associated familial Mediterranean fever attacks are relatively uncommon. They are not related to the age of the women, the chronicity of their disease or to the mutations they bear. Various therapeutic approaches have to be tried in order to abolish these attacks. A decrease in oestrogen level during menstruation may have a role in this unique manifestation of familial Mediterranean fever.