Long-term outcomes in antimitochondrial antibody negative XXXX .

@entity2835 @entity1 @entity648 @entity204
UNASSIGNED: Objectives Antimitochondrial antibodies (AMA) are a sensitive and specific marker for @entity2835 ( @entity2835 ). AMAs are present in 95% of @entity1 with @entity2835 . However, 5% do not have AMAs and data on these @entity1 is scarce. We aim to evaluate the long-term outcomes of @entity1 with AMA negative @entity2835 . Methods A retrospective chart review of 71 AMA negative @entity2835 @entity1 . Disease presentation, laboratory results, and clinical endpoints were recorded. AMA negative @entity1 were matched on year of diagnosis to a control group of 71 AMA positive @entity1 . Results Ninety-six percent of the AMA negative @entity1 were of female gender with a median age at diagnosis of 55 years and a length of follow-up of 7.5 years vs. 86% females, a median age of 56 and a follow-up of 8.3 years in the control group. Mean total @entity648 and alkaline phosphatase levels were 0.7   mg/dL vs. 0.6 and 570   U/L vs 341, in AMA negative vs. AMA positive @entity1 at presentation, respectively (p   =   NS). AMA negative @entity1 did not differ in terms of age, serum IgM levels, ANA status, or length of follow-up. Notably, AMA negative @entity1 had a significantly reduced survival free of liver-related complications including transplantation and @entity204 compared to AMA positive @entity1 (p   =   0.0182). Conclusion In this large experience, AMA negative @entity2835 @entity1 had a significantly worse prognosis compared to AMA positive @entity2835 @entity1 . The reason for the difference in prognosis is unclear, as it may be true difference or reflect delays in case detection among AMA negative @entity1 .
