Reported Triggers and Self-reported Management Strategies in Children With Familial Mediterranean Fever

Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disorder. Data regarding the reported triggers of this rare disease are scarce. This study aimed to analyze the demographic data, clinical findings during the attacks, reported triggering, and self-reported management strategies of pediatric patients with FMF with exon 10 MEFV mutations.

Patients diagnosed with FMF according to the Eurofever/PRINTO classification criteria, with heterozygous, homozygous, and compound heterozygous mutations in exon 10 of the Mediterranean fever ( MEFV ) gene, and with a follow-up of more than 6 months were included in the study.

The study included 266 patients (53% female, n = 141). Reported triggers were identified in 189 patients (93.6%), and the most common trigger was fatigue (n = 141; 69.8%). The others were as follows: prolonged standing (49.5%), emotional stress (47%), cold exposure (42.6%), insomnia (36.6%), menstruation (18.5%), high-fat food consumption (15.8%), exercise (15.3%), long-term travel (13.4%), starvation (11.9%), sunlight exposure (5.4%), and physical trauma (2.5%). Self-reported management strategies were used by 89.1% (n = 180) of the patients, primarily nonsteroidal anti-inflammatory drugs (75.2%, n = 152). The others were sleep (50.5%), fluid intake (39.1%), massage (31.2%), hot water compress (30.7%), warm shower (23.3%), fat-free diet (8.4%), and sweet food consumption (5.4%). Long-term travel was found to be a significantly more commonly reported trigger for attacks with arthritis/arthralgia ( p  = 0.036) and erysipelas-like erythema ( p  = 0.001).

This is the first study focused on reported triggers in childhood FMF. Although our study offers unique findings, the data require validation with clinical and laboratory evidence.