Acquired haemophilia in a patient with systemic lupus erythematosus

Acquired haemophilia (AH) is a rare disease caused by the spontaneous development of autoantibodies against coagulation factor VIII, with an incidence of at least 1.3-1.5 per million per year. More than half of AH cases is idiopathic. Other cases of acquired haemophilia are associated with connective tissue disease like SLE, RA and Sjögren’s syndrome. Malignancy, pregnancy, postpartum period and some medications may also be the causes of AH. Unlike congenital haemophilia, which occurs in males, acquired haemophilia occurs in both sexes, appears suddenly and the course of the disease may be fulminant and severe. In AH, bleeding into the joints is rare while extensive ecchymosis and mucous bleeding dominate. We report the case of a 27-year-old female patient, with an 8-year history of systemic lupus erythematosus (SLE), who developed acquired haemophilia caused by factor VIII inhibitors. The patient manifested spontaneous ecchymosis (Fig. 1–3). She was diagnosed in the active phase of SLE based on the presence of the high level of anti-double stranded DNA antibodies, low level of complement, anaemia and proteinuria of over 1.3 g/day. Coagulation tests showed isolated prolongation of the activated partial thromboplastin time, 98 s (26-37 s). She was tested negatively for lupus anticoagulant (LA). FVIII activity was found to be reduced to 0.3%. Autoantibody against FVIII has a very high titre (49 Bethesda units). The patient was successfully treated with intravenous steroids and cyclophosphamide pulse therapy, followed by 60 mg/day prednisone in combination with oral cyclosporine (CsA) administration. The inhibitor level was gradually reduced with an improvement in FVIII activity (Table I). There has been no recurrence of the disease after 1-year follow-up due to treatment with 10 mg/day prednisone and CsA (150 mg/day). She is in remission without proteinuria and skin changes.