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EULAR/PReS endorsed recommendations for the management of familial Mediterranean fever (FMF): 2024 update

First author : Ozen S

Review: Annals of the Rheumatic Diseases

Reference: Ann Rheum Dis. 2025 Apr 9:S0003-4967(25)00084-6

Link to pubmed: EULAR/PReS endorsed recommendations for the management of familial Mediterranean fever (FMF): 2024 update - PubMed

Recommandations approuvées par l’EULAR et la PReS pour la FMF

2024 European Recommendations on Familial Mediterranean Fever (FMF) – Summary:


Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disease worldwide. Due to its clinical and genetic variability, specialized management is essential. In 2024, the EULAR and PReS societies updated their guidelines.


General Principles:

  • FMF requires specialist expertise for both diagnosis and management.

  • The primary goal is complete control of inflammation, including subclinical inflammation, to prevent complications such as AA amyloidosis.

  • Lifelong treatment is necessary, with strict adherence, primarily based on daily colchicine therapy.

  • Care should be patient-centered, aiming to preserve quality of life.


Key Recommendations:

  • Colchicine should be initiated as soon as a clinical diagnosis is made.

  • The dosage must be tailored to tolerance and adherence (single or divided daily doses).

  • If symptoms persist or subclinical inflammation remains, the dose should be increased within recommended limits (maximum 2 mg/day in children, 3 mg/day in adults).

  • If colchicine fails despite good adherence, interleukin-1 blockers (anakinra, canakinumab) are recommended.

  • Chronic musculoskeletal manifestations may require additional treatments (DMARDs, biologics).

  • Regular monitoring (clinical, biological, toxicity, adherence) is essential.

  • Colchicine should be continued during pregnancy and breastfeeding.

  • During acute attacks, colchicine should be maintained at the same dose, with symptomatic treatment added (e.g., NSAIDs).

  • A minimum core set of assessment criteria is proposed: attack frequency, quality of life, biological markers (CRP, SAA).


Quality indicators, clinical priorities (especially adherence), and implementation strategies are provided to harmonize care across centers.



National Protocol for Diagnosis and Care of Familial Mediterranean Fever

Updated: Dec 11, 2024

S. Georgin-Laviallea,h,∗, L. Saveya,h, L. Cuissetc, G. Boursiere,h, J.-J. Boffab,h,

M. Delplanquea,h, R. Bourguibaa,h, J.-B. Monfortd,h, I. Touitoue,h, G. Grateaua,h,

I. Kone-Pautf,h, V. Hentgeng,h, Collaborators1


Protocole national de diagnostic et de soin de la FMF




Summary:


Familial Mediterranean Fever is the world's most common monogenic autoinflammatory disease. It mainly affects people from the Mediterranean region. The mutated gene is MEFV, which codes for pyrin. Transmission is autosomal recessive. Patients present with recurrent attacks of fever since childhood, associated with abdominal and/or thoracic pain lasting an average of 2 to 3 days, and a biological inflammatory syndrome. Other symptoms include arthralgia or arthritis of large joints such as the knees and ankles, myalgia of the lower limbs and pseudo-eryzipelas of the ankles. Its most severe complication is inflammatory amyloidosis, or AA amyloidosis, which can lead to renal failure. Treatment is based on colchicine, which helps prevent relapses and the onset of renal amyloidosis.


This work presents national recommendations for the diagnosis, management and follow-up of Familial Mediterranean Fever in France, where we estimate there are between 5,000 and 10,000 patients with the disease at all stages of life. Diagnosis is suspected on the basis of clinical and anamnestic elements, and confirmed by genetic analysis. These recommendations also propose a “treat-to-target” approach to disease treatment, particularly in cases of suspected colchicine resistance - a very rare situation that should remain a situation of elimination, particularly after verification of colchicine compliance. Two special situations are also addressed in these recommendations: renal failure and pregnancy.

​© 2023 Publié par Elsevier Masson SAS au nom de Société Nationale Franc¸ aise de Médecine Interne (SNFMI).



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