What do patients do during a familial Mediterranean fever attack? Their strategies and associated factors

Abstract

Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disease characterised by recurrent fever and serositis. Despite colchicine's proven efficacy, attacks may persist, necessitating the implementation of various mitigation strategies by patients. To identify attack mitigation strategies and associated factors in FMF patients. A cross-sectional study at a tertiary rheumatology clinic included adults (≥ 18 years) meeting Tel-Hashomer criteria, on colchicine for ≥ 6 months, with ≥ 1 attack in the past six months. Patients were asked about their attack mitigation strategies, including colchicine dose increase, emergency department (ED) admission, resting, abdominal hot pack, dietary changes, on-demand anakinra, herbs, paracetamol, non-steroidal anti-inflammatory drugs (NSAIDs), and corticosteroid use. Among 258 patients (98 men, 160 women; median age 35 years, disease duration 18 years), 93% used mitigation strategies. The most common first choice was ED admission (19.8%), followed by colchicine dose increase (18.2%), resting (14.3%), dietary changes (4.3%), NSAIDs (7%), paracetamol (6.6%), on-demand anakinra (6.2%), herbs (6.2%), and corticosteroids (5%). Normal C-reactive protein (CRP) levels, shorter last attack duration, lower international Severity Score for FMF (ISSF), non-working and non-M694V mutations were linked with colchicine dose increase (p < 0.05). M694V positivity, working, persistent CRP elevation, dominant serositis and musculoskeletal attacks, education level ≤ 8 years, longer last attack duration, higher ISSF, current colchicine resistance and non-adherence were linked with ED admission (p < 0.05). Most FMF patients used strategies to mitigate attacks, mainly ED admission and colchicine dose increase. These were influenced by genetic mutations, attack type, working status, education, and CRP levels.

Keywords: Attack; Colchicine; Emergency department; Familial Mediterranean fever; Mitigating strategies.