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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.



The needed daily dose of colchicine in patients with Familial Mediterranean Fever may be higher in women: a study on behalf of the JIR cohort.

Author: Di Cola et al.

Ref : Di Cola et al, Arthritis Res Ther. 2025 Mar 19;27(1):59.


La dose quotidienne nécessaire de colchicine chez les patients atteints de Fièvre Méditerranéenne Familiale pourrait être plus élevée chez les femmes

Summary


To date, no data exist on the relationship between daily colchicine dosage and body weight in patients with Familial Mediterranean Fever (FMF). This question is frequently raised by patients or their parents during consultations. The objective of our study was to describe the daily colchicine dosage in a cohort of patients with FMF.


We conducted a retrospective analysis from 2016 to 2023 on adult FMF patients who were prospectively followed at the French National Reference Center for Auto-inflammatory Diseases at Tenon Hospital.


Among the 272 patients studied, 149 were women (57.8%), with a mean age of 43 years. The average weight was 67.8 kg, and the mean BMI was 24.2 kg/m². Colchicine was taken by 96% of the patients. A subgroup of 30 patients was receiving 2.5 mg/day of colchicine: the majority were women (n=23; 76.7%; p=0.018), with a significantly lower average weight (p=0.019); in fact, 26 out of 30 (87%) weighed less than 50 kg. Female sex was associated with a higher daily dose of colchicine (p=0.0208), whereas no significant correlation was found with weight (p=0.4073).


No signs of toxicity were observed in patients receiving 2.5 mg/day of colchicine, including those weighing under 50 kg, the majority of whom were women.


One hypothesis is that this increased need for colchicine in some women may be related to hormonal factors, with a possible hyperactivation of pyrin.


This is the first study to examine the relationship between weight and colchicine dosage in adults with FMF, highlighting a potential link with female sex.


This work provides reassurance to patients receiving 2.5 mg/day of colchicine: there is no toxicity at this dose in the absence of renal impairment.




An update on autoinflammatory diseases

Updated: Dec 11, 2024


First author: S. Georgin-Lavialle

Mise au oint en français sur les MAI

Abstract:


Autoinflammatory diseases (AIDs) are defined as disorders of innate immunity. They were initially defined in opposition to autoimmune diseases, due to the absence of involvement of the adaptive immune system and circulating autoantibodies. The 4 IADs first described are known as the “historic” IADs and include: familial Mediterranean fever (associated with mutations in the MEFV gene), cryopyrinopathies (associated with mutations in NLRP3), tumor necrosis factor receptor-associated periodic syndrome (associated with mutations in TNFRSF1A) and mevalonate kinase deficiency (associated with mutations in MVK). Over the past 10 years, more than 50 new monogenic IBDs have been discovered thanks to advances in genetics. Diagnosis is facilitated by personal and family history-taking and detailed analysis of the signs and symptoms associated with febrile attacks, which must be associated with the presence of elevated blood biomarkers of inflammation. Increasingly powerful genetic analysis techniques can help refine the diagnosis. This chapter describes the main types of IJD, and helps to guide the clinician in the suspicion and diagnosis of IJD.





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