Long-term efficacy of canakinumab in hyperimmunoglobulin D syndrome

Ozdemir Isik O., Karadag D. T., Tekeoglu S., YAZICI A., Cefle K., ÇEFLE A.

International Journal of Rheumatic Diseases, vol.27, no.1, 2024 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 27 Issue: 1
  • Publication Date: 2024
  • Doi Number: 10.1111/1756-185x.14857
  • Journal Name: International Journal of Rheumatic Diseases
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, EMBASE, MEDLINE
  • Keywords: canakinumab, hyperimmunoglobulin D syndrome
  • Istanbul University Affiliated: Yes


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.