2024 Management of Familial Mediterranean Fever Recommendations

Jack Cush, MD

Apr 28, 2025 10:00 am

A EULAR/PReS combined task force has developed recommendations for the management of Familial Mediterranean fever (FMF), the most common monogenic autoinflammatory disease worldwide. These evidence-based recommendations update the 2016 recommendations developed for rheumatologists and other HCPs who care for FMF patients.

A multidisciplinary panel reviewed new literature and evidence and voted recommendations via a Delphi survey. They proposed 4 overarching principles and 12 recommendations.

Overarching principles

FMF has complex clinical presentation and genetics, which requires expertise in diagnosis and management
The treatment goal in FMF is to achieve minimal or no clinical activity and complete control of subclinical inflammation to prevent associated damage
FMF requires lifetime management, including long-term prophylaxis with colchicine; therefore, treatment adherence is the cornerstone of FMF management
Patient-centred management is required to promote a good quality of life and support health and well-being

Recommendations

Treatment with colchicine should start as soon as a clinical diagnosis is made
Colchicine dosing can be in single or divided doses depending on adherence and tolerance
In adherent patients, the persistence of attacks or subclinical inflammation represents an indication to increase the colchicine dose within the recommended range. The maximum dose should not exceed 2 mg/d in children and 3 mg/d in adults
Adherent patients not responding adequately to the maximum tolerated dose of colchicine should be considered for additional treatments; the highest level of evidence is for IL-1-targeting treatments
Chronic inflammatory musculoskeletal involvement of FMF may need additional treatments
Response, toxicity, and adherence should be monitored regularly, more often at diagnosis and when the disease is uncontrolled
As overdose with colchicine can be fatal; intentional or accidental overdosing should be carefully assessed
In AA amyloidosis, treatment aims to obtain complete and sustained control of the biochemical inflammation, measured by SAA or equivalent (CRP)
In FMF patients on biologics, doses should be optimised; if remission is achieved, tapering and discontinuation may be considered
Colchicine should be continued during conception, pregnancy, and lactation; amniocentesis is not currently recommended
During a characteristic attack, the usual dose of colchicine should be continued, and symptomatic treatment, such as NSAIDs, is recommended
To standardise patient care and research, a minimum outcome set should include measures of clinical disease activity (eg, attack frequency per 3 mo, physician assessment), patient experience (eg, PROMs and PREMs), and biochemical markers (eg, CRP, SAA)