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About 5-10% of patients with FMF do not to colchicine treatment to control inflammation.
In these patients anti-IL1 therapy seems to be effective in controlling inflammation,
although clinical trials are lacking. However, many patients on Anakinra experience
side effects including headache and injection-site reactions. How Anakinra treatment
influences quality-of-life (QoL) in these patients is unknown.
To investigate the effect of Anakinra on inflammation and QoL in a family with a severe
phenotype colchicine-resistant FMF.
Patients and methods
Four sisters of Italian descent with homozygote M694V-positive FMF, had an extraordinary
phenotype with continuous inflammation, daily fevers, erysipelas-like skin lesions
and serositis. Colchicine was initiated in all patient, but showed ineffective in
controlling inflammation. Eventually, Anakinra was initiated. Before and during treatment
with Anakinra inflammatory parameters (CRP, leukocyte-count, Serum amyloid A) were
measured. One patient only started Anakinra after type AA amyloidosis had developed
leading to a renal replacement therapy and renal transplantation. In the other three
patients QoL questionnaire (RAND-36) was obtained, before and 4 weeks after start
of Anakinra-therapy. The study was approved by the local ethical committee. Because
of reimbursement was initially declined, Anakinra was discontinued. Later, it could
be re-initiated. QoL questionnaires were obtained after stopping and after re-initiation
During Anakinra treatment the CRP values were significantly lower than without Anakinra
(mean 3.7 mg/L vs 30 mg/L, p<0.001). The QoL improved significantly after initiation
of Anakinra in 5 out of 6 scales of the RAND-36 (p<0.05). The QoL deteriorated after
withdrawal of Anakinra to pre-treatment level and improved again after re-initiation
Here we show that Anakinra can effectively suppress inflammation in a family with
severe colchicine-resistant FMF. This lead to a significant improvement in quality
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8th International Congress of Familial Mediterranean Fever and Systemic Autoinflammatory