ABSTRACT
Objective To analyze the clinical features and treatment of connective tissue disease (CTD) complicated with acquired hemophilia A (AHA).Methods A retrospective analysis of 8 cases of CTD [5 cases of systemic lupus erythematosus (SLE),2 cases of Sj(o)gren's syndrome (SS),1 case of rheumatoid arthritis (RA)] related to clinical manifestations,diagnostic methods,treatment options and outcomes.Results At the onset of AHA,active disease was shown in 7 patients with CTD,and 5 cases had bleeding symptoms in different parts.There were 3 cases of anti-phospholipid syndrome in 5 cases of SLE,2 of which had thrombosis.In 8 patients,the activated partial thromboplastin time (APTF) was prolonged by 1.7 to 3.times,FⅧ∶ C was 9.2％ to 21％ (50％ to 150％),and the factor Ⅷ inhibitor titer was increased by 7.6 to 56 BU/m1 (Bethesda method).Seven patients were treated with sufficient hormones,immunosuppressive agents,human immunoglobulin (IVIG),and blood products.Five patients had clinically improved bleeding tendency and APIT,and one patient was ineffective.Conclusion CTD is easy to combine with AHA.Glucocorticoid combined with immunosuppressive agent can effectively treat CTD-related AHA.For refractory patients,rituximab can be an alternative.