Real-World Data on Patients With Acquired Haemophilia A in Japan Undergoing Rehabilitation or With Low Activities of Daily Living Scores: The ORIHIME II Study.

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Acquired haemophilia A (AHA) is characterized by the development of autoantibodies against factor VIII, reducing its activity and potentially resulting in bleeding. To assess the characteristics of people with AHA undergoing rehabilitation and/or with low activities of daily living (ADL) scores, thereby characterizing unmet needs in the management of AHA and informing treatment optimization. ORIHIME II, the largest epidemiological and treatment survey of AHA in Japan, is a descriptive, retrospective, observational study conducted using health claims data from April 2008 to October 2021. The primary outcome measures were rehabilitation practice and ADL scores on hospital admission and discharge; use of haemostatic agents and immunosuppressive therapy were also assessed. Overall, 427 patients in Japan were eligible for the study. Median (Q1-Q3) age was 78.0 (70.0-84.0) years; 264 patients (61.8%) were male. Median (Q1-Q3) time to start rehabilitation was 9 (4-21) and 14 (6-31) days for those with an admission ADL score of<85 and ≥85, respectively. Of the 427 patients, 249 underwent rehabilitation. The most common rehabilitation type was for disuse syndrome; haemostatic agents were more commonly used in patients undergoing earlier rehabilitation. The physical condition of the patient at hospitalization was associated with rehabilitation practice and the ability of the patient to perform day-to-day activities independently. Treatment strategies should be optimized to allow initiation of rehabilitation as early as possible in the course of AHA.