• Fang-Fei Chen, Xue-Han Zhang, and Yang Jiao.
• aDepartment of General Internal Medicine bDepartment of Health Care, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
• Medicine (Baltimore). 2020 Aug 14; 99 (33): e21636.

RationaleTurner syndrome (TS) is an anomaly caused by loss of part of or all the X chromosomes. Ankylosing spondylitis (AS) is an HLA-B27-associated autoimmune disease with a male predominance. It is widely accepted that TS patients are at higher risk of autoimmune diseases, but AS in TS patients has only rarely been reported.Patient ConcernsA 13-year-old TS patient presented with intermittent pain in both hip joints, and a 27-year-old TS patient presented with thoracic kyphosis and a history of AS.DiagnosesBoth patients were diagnosed with AS according to their symptoms, laboratory results, and imaging.InterventionsThe first patient was treated with tocilizumab for 8 months, whereas the second patient was treated with diclofenac initially with subsequent surgery for thoracic kyphosis.OutcomesTreatment relieved the symptoms of both patients and laboratory parameters improved.LessonsEven though AS has a male predominance, clinicians should be aware that AS and TS may co-exist and that the clinical features are atypical in TS patients with AS.

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