Autoinflammatory & rare systemic diseases
Efficacy and safety of treatments in familial Mediterranean fever and its complications: a systematic review informing the EULAR/PReS recommendations for familial Mediterranean fever
Authors' conclusion
Biological agents, particularly IL-1 inhibitors, are effective and relatively safe options for FMF patients who do not respond to colchicine.
Study context
- Population
- Patients with familial Mediterranean fever (FMF), including those with complications such as AA amyloidosis; studies published after October 1, 2014 (MEDLINE, Embase, Cochrane Library)
- Participants
- 1,798 patients42 studies
- Comparison
- Biological agents and tofacitinib, and colchicine preparations and dosing strategies, in FMF
- Outcome
- Efficacy and safety of biological agents and tofacitinib in FMF and its complications, and of colchicine preparations and dosing strategies
Methodological detailsqualitative synthesis, Cochrane Risk of Bias tool and Newcastle-Ottawa Scale, Results synthesised qualitatively due to excess heterogeneity
- Qualitative synthesis
- Cochrane Risk of Bias tool and Newcastle-Ottawa Scale
- Results synthesised qualitatively due to excess heterogeneity
Pooled result
As reported in the abstract
Efficacy of IL-1 blockers (6 randomised controlled trials (RCTs) and observational studies)
- IL-1 blockers seem to be effective in decreasing the number of attacks and acute-phase reactants and improving disease activity scores and patient-reported outcomes
Further pooled results
Colchicine dosing (1 RCT)
- Equivalence of once-daily vs twice-daily doses of colchicine
AA amyloidosis (31 studies)
- Evidence entirely observational but reassuring for a deadly complication
Tapering
- 13 studies included for tapering
Safety
Safety of IL-1 blockers
- They also seem relatively safe
Original publication
Efficacy and safety of treatments in familial Mediterranean fever and its complications: a systematic review informing the EULAR/PReS recommendations for familial Mediterranean fever. Ann Rheum Dis. Published 24 June 2025. doi:10. 1016/ j. ard. 2025. 05. 020
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