高免疫球蛋白D综合征（HIDS）是一种罕见的自身炎症性疾病，具有常染色体隐性遗传方式。它由于mevalonate激酶基因（MVK）的特定突变引起。迄今为止，尚未批准针对HIDS的特定治疗方法；然而，根据病例报告和观察性研究，使用非甾体类消炎药、类固醇、秋水仙素、肿瘤坏死因子-α抑制剂以及抗白细胞介素-1治疗等方法。我们在此报告了一个反复发热和关节炎发作的病例，该病例对阿那基拉无效，并成功地接受了卡纳西麦治疗。通过每4周300mg卡纳西麦的治疗，持续5年达到了长期缓解，没有任何副作用。© 2023 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.

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.© 2023 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.