Because acquired haemophilia (AH) is an uncommon complication of systemic lupus erythematosus (SLE), researchers wanted to look into the clinical characteristics of SLE-related AH in Chinese patients. From 1986 to 2018, a big tertiary care hospital in China conducted a medical records review project. Using the International Classification of Diseases, they searched the case database at Peking Union Medical College Hospital. Clinical information on SLE-related AH patients was gathered. A total of 9282 SLE patients have been admitted to hospitals. There were six female SLE-related AH patients diagnosed. Four individuals developed haemophilia A (AHA), and two had von Willebrand syndrome. Their average age was 33.67 13.77. Five individuals were suffering from active disease. At the time of AH diagnosis, the mean SLE disease activity index was 10.50 5.28. The average activation time of partial thromboplastin was 86.5 seconds. In one example, secondary antiphospholipid syndrome and AHA coexisted, and pulmonary embolism was discovered three years later. An overall remission rate of 83.3 per cent was attained after immunosuppressive medication and symptomatic treatment.

SLE-related AH occurred seldom. In SLE patients, the onset of AH is usually associated with active illness. The AH might be the initial clinical manifestation of SLE. Secondary antiphospholipid syndrome and AHA may coexist in an SLE patient. Early and vigorous therapy helps to improve the prognosis.