A treatment algorithm for familial Mediterranean fever patients with menstruation-associated attacks

Abstract Objectives FMF is characterized by febrile polyserositis attacks. Menstruation could be a trigger for attacks. We aimed to analyse the features of adolescent FMF patients with menstruation-associated attacks and propose a management algorithm. Methods All female FMF patients who had menarche and visited the Pediatric Rheumatology Unit between January and December 2022, were included into this study. Demographics, general characteristics and the features of menstrual cycle and FMF attacks were noted. Results A total of 151 female FMF patients were included. Thirty-five (23.2%) had menstruation-associated attacks. Fever and arthritis were less frequent during the menstruation-associated attacks than the attacks not associated with menstruation in these patients (65.7% vs 88.6%, P = 0.01 and 2.9% vs 20%, P = 0.04, respectively). Patients with menstruation-associated FMF attacks were younger at symptom onset and diagnosis (2.5 vs 5 years, P = 0.004 and 4 vs 7 years, P = 0.01; respectively), had a higher rate of dysmenorrhea (74.3% vs 38.8%, P 0.001, respectively) and higher pre- and post-menarche attack frequency (4 vs 2 and 10 vs 0, respectively; P 0.001 for both) than patients whose attacks were not associated with menstruation. The interventions for menstruation-associated attacks included initiating colchicine, increasing the dose of colchicine, switching from coated to compressed colchicine tablets or adding anti-interleukin 1 drugs and initiating on-demand non-steroidal anti-inflammatory drugs, on-demand glucocorticoids and on-demand anakinra. On-demand therapies were beneficial in controlling menstruation-associated attacks. Conclusions This is the largest cohort of adolescent FMF patients with menstruation-associated attacks. Severe FMF may cause a tendency to this association. On-demand therapies could be preferred in the management.