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THU0402 Canakinumab Therapy in Patients with Familial Mediterranean Fever
  1. S. Ugurlu,
  2. E. Seyahi,
  3. G. Hatemi,
  4. A. Hacioglu,
  5. N.F. Akkoc,
  6. H. Ozdogan
  1. Department of Internal Medicine, Division Of Rheumatology, Cerrahpasa Medical Faculty, University Of Istanbul, Istanbul, Turkey

Abstract

Background In a recent pilot study, it was reported that Canakinumab reduced the frequency of attacks in 9 patients with Familial Mediterranean Fever (FMF) resistant to colchicine with no apparent side effects (1).

Objectives 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 24 patients with FMF (14 F/10 M) who were receiving canakinumab for various indications. Here we report 19 (11 F/8 M) who had at least 3 injections. Four patients had concomitant diseases such as psoriasis, ankylosing spondylitis (2), polyarteritis nodosa. The indications for canakinumab (150mg/mo) were insufficient response to colchicine in 10 (>1 attack/month), amyloidosis in 6 and injection site reaction to anakinra in3 patients. The mean age of the patients was 31,10±13,04 years, while the disease duration was 24,84±10,29 years. The mean colchicine dose was 2,28±0,38 mg/day. The median injection number with canakinumab was 5 (range 3-14). The mean duration of canakinumab therapy was 9,68±5,26 months. Eleven of the patients had no attacks after canakinumab, while in five patients attack frequency was reduced more than %50. In 6 cases with FMF amyloidosis, proteinuria decreased in 2 (from 15020 mg/dl to 2226 mg/dl, and from 6135 mg/dl to 4000 mg/dl), increased in the other 2 (from 1700mg/dl to 4700mg/dl and from 5001 mg/dl to 7061 mg/dl), and did not change in the remaining 2. Ten of the 24 patients complaining of severe myalgia and calf pain unresponsive to colchicine treatment, improved significantly on CAN. According to patient global assessment 18 patients reported significant improvement while only one, reported no change. CAN was stopped because of remission (no attacks at least for 3 months) in 3 and for pregnancy demand in one patient. Attacks recurred after 4, 6, 12 months from discontinuation of CAN in 3 patients and 4th patient is still attack-free for the last 7months. 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 recur on a 2-monthly regimen. Treatment was switched to another biological agent in 2 patients with amyloidosis because of increasing proteinuria. One other patient with amyloidosis whose proteinuria was stable, developed lichen planus lesions and CAN treatment had to be stopped.

Conclusions Canacinumab is effective in controlling the attacks in patients with inadequate response to colchicine. In a selected group of FMF patients, Canacunimab 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.

References

  1. Gul A, Ozdogan H, et al. Efficacy and Safety of Canakinumab in Adults with Colchicine Resistant Familial Mediterranean Fever. Arthritis Rheum. 2013 Mar;64(10): S322.

Disclosure of Interest : None declared

DOI 10.1136/annrheumdis-2014-eular.5654

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