Prognostic factors for remission of and survival in acquired hemophilia A ( XXXX ): results from the GTH-AH 01/2010 study.

@entity2801 @entity9812 @entity1 @entity548 @entity230 @entity204
@entity9812 ( @entity9812 ) is caused by autoantibodies against @entity2801 ( @entity2801 ). Immunosuppressive treatment (IST) results in remission of disease in 60% to 80% of @entity1 over a period of days to months. IST is associated with frequent adverse events, including infections as a leading cause of @entity204 . Predictors of time to remission could help guide IST intensity but have not been established. We analyzed prognostic factors in 102 prospectively enrolled @entity1 treated with a uniform IST protocol. Partial remission (PR; defined as no active @entity548 , @entity2801 restored >50 IU/dL, hemostatic treatment stopped >24 hours) was achieved by 83% of @entity1 after a median of 31 days (range 7-362). @entity1 with baseline @entity2801 <1 IU/dL achieved PR less often and later (77%, 43 days) than @entity1 with >= 1 IU/dL (89%, 24 days). After adjustment for other baseline characteristics, low @entity2801 remained associated with a lower rate of PR (hazard ratio 0.52, 95% confidence interval 0.33-0.81, P < .01). In contrast, PR achieved on @entity230 alone within  <=21 days was more common in @entity1 with @entity2801 >= 1 IU/dL and inhibitor concentration <20 BU/mL (odds ratio 11.2, P < .0001). Low @entity2801 was also associated with a lower rate of complete remission and decreased survival. In conclusion, presenting @entity2801 and inhibitor concentration are potentially useful to tailor IST in @entity9812 .
