%0 Case Reports
%T Long-term efficacy of canakinumab in hyperimmunoglobulin D syndrome.
%A Ozdemir Isik O
%A Karadag DT
%A Tekeoglu S
%A Yazici A
%A Cefle K
%A Cefle A
%J Int J Rheum Dis
%V 27
%N 1
%D 2024 Jan 14
%M 37578023
%F 2.558
%R 10.1111/1756-185X.14857
%X Hyperimmunoglobulin D syndrome (HIDS) is a rare autoinflammatory disorder with autosomal recessive inheritance. It is caused by specific mutations in the mevalonate kinase gene (MVK). No treatment specific to HIDS has been approved to date; however, nonsteroidal anti-inflammatory drugs, steroids, colchicine, tumor necrosis factor-α inhibitors, and anti-interleukin-1 treatments are used, based on case reports and observational studies. Herein, we report a case with recurrent fever and arthritis attacks who did not respond to anakinra and was successfully treated with canakinumab. Long-term remission was achieved without any side effects with 300 mg canakinumab treatment every 4 weeks for 5 years.