Background/Purpose:

It has been  reported that Canakinumab reduced the frequency of attacks in  patients with Familial Mediterranean Fever (FMF) resistant to colchicine  with no apparent side effects. 

We present our experience with Canakinumab in FMF patients resistant or intolerant  to colchicine.  

Methods:

The charts of the patients with FMF who were on Canacinumab were evaluated retrospectively with regard to response and safety. 

Results:

There were 36 patients with FMF (19 F/17 M) receiving canakinumab for various indications. We report 32 (17 F/15 M) who had at least 3 injections. Six patients had concomitant diseases such as psoriasis (1), AS (4), PAN (1), AS and PAN (1) and vasculitis(1). The indications for canakinumab (150mg/mo) were insufficient response to colchicine in 22 (>1 attack/month), amyloidosis in 6, injection site reaction to anakinra in 6 and adverse effects of colchicine (azoospermia in 1, neuropathy in 1, hepatotoxicity in 1, myopathy in 2 patients).  The mean age of the patients was 35,71 ± 12,82 years, the mean duration of FMF was 14,87 ± 9,19 years, the mean injection number was 10,53 ± 7,06 and the mean duration of canakinumab  therapy was 16,81 ± 10,46 months. Twenty five of the patients had no attacks after canakinumab, 5 patients’ attack frequency was reduced more than %50 while two patient’s attack frequency remained more than %50. In 6 cases with FMF amyloidosis, proteinuria decreased in 2 (from 15020mg/dl to 364mg/dl and from 6135mg/dl to 280mg/dl), increased in the other 2 (from 1700mg/dl to 4700mg/dl and from 5001mg/dl to 7061mg/dl), and did not change in the remaining 2. Fourteen patients complaining of severe myalgia and calf pain unresponsive to colchicine treatment improved significantly. According to patient global assessment the mean score decreased from 8,18 ± 2,48 to 1,53 ± 2,43 (p<0.001). Canacinumab was stopped because of remission (no attacks at least for 3 months) in 5, for pregnancy demand in 1, for unresponsiveness in 2, for increasing proteinuria in 2, and the treatment was also stopped in the patient with oligospermia after being fertile.

Attacks recurred after 4, 7, 12, 12 months from discontinuation of the therapy in 4 patients, and 3 patients are attack-free for 12, 17 and 17 months till now.  

Canakinumab was well tolerated in general. None of the patients had injection site reactions. The patient with psoriasis reported a flare in psoriatic plaques, which responded to local treatment. Therapy was discontinued temporarily in one patient who developed mild leucopenia, which did not reccur on a 2-monthly regimen. One other patient with amyloidosis whose proteinuria was stable, developed lichen planus lesions and the treatment had to be stopped. One patient had  pneumonia, also he is attack-free for 15 months untill last dose.

Conclusion:

Canacinumab is effective in controlling the attacks in patients with inadequate response to colchicine and may serve as a treatment alternative with a favorable side effect profile. For better understanding the drug’s efficacy and safety in the long term there is a need for controlled trials.

« Back to 2015 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/canakinumab-therapy-in-patients-with-familial-mediterranean-fever-2/