Living with Familial Mediterranean Fever (FMF): what helps you cope better?

Article title: The effects of self-efficacy in managing the disease and disease adaptation levels of Familial Mediterranean

Fever (fmf) patients on satisfaction with life: a web-based cross-sectional study

First author: Demir RN

Journal: Orphanet Journal of Rare Diseases

Link to the article: https://ojrd.biomedcentral.com/articles/10.1186/s13023-025-03931-w

Author of the abstract: Rim BOURGUIBA

Abstract

Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disease in the world. It is characterized by recurrent inflammatory episodes of fever, abdominal pain, chest pain, and joint pain. Treatment is based on colchicine as first-line therapy, with biotherapy sometimes necessary. In addition to medical management, patients' ability to adapt to chronic disease and their sense of self-efficacy, defined as their feeling of personal effectiveness in managing the disease, can influence their quality of life. The objective of this study was to evaluate the impact of self-efficacy in FMF management and levels of adaptation to the disease on the satisfaction/quality of life of patients with FMF.

Methods:

A cross-sectional observational study was conducted using an online questionnaire distributed on Facebook and Instagram (FMF patient groups) between February and April 2024. The authors included adult Turkish patients (≥18 years old) who had been diagnosed at least one year prior.

The assessment tools used were:

- Self-Efficacy Scale for Chronic Disease (6 items).

- Adaptation to Chronic Illness Scale (25 items: physical, psychological, and social adaptation).

- Satisfaction With Life Scale.

Results

In this study of 423 adult Turkish patients with FMF, there was a predominance of females (73.8%), with ages ranging from 32 to 45 years. A disease duration of 21 years or more was reported in 38.3% of patients. The average self-efficacy score in disease management was relatively high (4.67/10). Adaptation to the disease was moderate overall (3.10/5), with better physical adaptation (3.36), followed by psychological adaptation (2.92) and social adaptation (2.87). Life satisfaction was below average (2.68/5). The correlation study revealed positive and significant associations between self-efficacy and adaptation (r = 0.532), between self-efficacy and life satisfaction (r = 0.417), and between adaptation and life satisfaction (r = 0.564) (Table 1). Regression analysis showed that self-efficacy explained 17.4% of the variance in life satisfaction, while adaptation to the disease explained 31.8%, confirming their decisive role in the self-management of FMF on quality of life (Table 2).

Conclusion

This study shows that in patients with FMF, self-efficacy and adaptation to the disease directly influence the overall low quality of life in this population. Strengthening therapeutic education and psychosocial support appears essential to improving the quality of life of adult patients with FMF.

Table 1: Correlation study between self-efficacy and quality of life in FMF patients

Table 2: Regression analysis of self-efficacy on quality of life and disease management

Vivre avec la fièvre méditerranéenne familiale : qu’est-ce qui aide à mieux s’en sortir.pd

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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

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.

Recommandations approuvées par l’EULAR et la PReS FMF.pdf

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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.

Link to article: https://pubmed.ncbi.nlm.nih.gov/40108720/

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.

La dose quotidienne nécessaire de colchicine chez les patients atteints de Fièvre Méditerr.

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