Biologic agentsInterleukin-1 Targeting Drugs in Familial Mediterranean Fever: A Case Series and a Review of the Literature
Section snippets
Methods
Electronic mailing lists of French pediatric and adult rheumatologists societies were used to call for the medical history of FMF patients (confirmed by carriage of 2 MEFV-mutations) who had been treated with interleukin-1 targeting drugs. Information about age, sex, disease course, treatment before anti-IL-1 targeting drugs, reasons for the use of anti-IL-1 targeting drugs, treatment modalities, clinical effect, side effects, and duration of follow-up were recorded. As the genetic analyses
Results
Seven patients (4 male, 3 female) from 5 different hospitals were identified. The patients' ages when starting IL-1 targeting drugs were 51, 45, 12, and 7 years (4 patients). Disease duration before the use of interleukin-1 targeting drugs was 3 to 6 years in children and approximately 20 years in adult patients. MEFV gene analyses showed homozygote M694V mutations in 6 patients and composite I692de/V726A, E149Q mutations in 1 patient (case 4). FMF manifested with fever and abdominal pain in
Discussion
Here, we report 7 FMF patients who were treated with interleukin-1 targeting drugs. Additionally, in the literature 8 single cases have been published (9, 10, 11, 12, 13, 14, 15, 16). Considering all published cases, the reasons for using interleukin-1 targeting drugs in FMF patients can be divided into the following categories: (1) incomplete control of FMF disease activity despite colchicine treatment; (2) high SAA levels and/or renal complications despite colchicine treatment; (3)
Conclusions
Colchicine treatment is safe and effective in the large majority of FMF patients. However, the published cases indicate that in some, rare patients the availability of an alternative treatment would be desirable. The data for the use of interleukin-1 targeting drugs in FMF patients are currently limited to uncontrolled off-label use. Altogether, the published cases indicate that interleukin-1 targeting drugs may be good candidates when looking for a treatment alternative to or supplementary to
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IKP received research and consulting fees for lecturing (<10,000 Euros) from Novartis Pharma.