Abstract
BACKGROUND: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease. Acquired hemophilia A (AHA) is a rare acquired bleeding disorder characterized by the presence of autoantibodies against coagulation factor VIII (FVIII) in the circulation, leading to reduced FVIII activity. Cases of SLE complicated by AHA are scarce and challenging to treat. Conventional immunosuppressants are often associated with inadequate efficacy or adverse effects. Telitacicept, a dual-target biologic agent against B-cell activating factor (BAFF) and a proliferation-inducing ligand (APRIL), offers a novel therapeutic approach for such refractory comorbidities. CASE INTRODUCTION: This report describes a 52-year-old female with a 10-year history of SLE, previously diagnosed with concomitant Sjögren's syndrome and antiphospholipid antibody syndrome currently seronegative for respective antibodies following prolonged immunosuppression. Her disease was recurrent despite long-term corticosteroid and immunosuppressive therapy. She was later diagnosed with SLE-associated AHA due to fever, epistaxis, and coagulation abnormalities. Subsequently, half-dose telitacicept (80mg/week, adjusted due to financial constraints) combined with corticosteroids was administered. The patient experienced reduced bleeding symptoms, decreased FVIII antibody levels, no recurrent bleeding during follow-up, and stable disease. CONCLUSION: Half-dose telitacicept combined with corticosteroids effectively controlled the disease in this patient with SLE-associated AHA, improving bleeding symptoms and coagulation function while allowing corticosteroid dose reduction. This provides a feasible treatment option for such patients with limitations to conventional therapy or financial constraints. However, efficacy differences between different dosages and its long-term safety still require further verification.