A 38-year-old African-American XXXX with an unusually rapid progression of " @entity2835 ": a missed opportunity!

@entity1 @entity2835 @entity32 @entity1087 @entity39 @entity75 @entity843 @entity409 @entity5990 @entity4053 @entity1578 @entity1448 @entity3960 @entity775 @entity623 @entity10055
The case discussed is of a 38-year-old African-American @entity1 who developed @entity409 . She underwent cholecystectomy for presumed @entity5990 . This was followed by a worsening of her condition, with the development of @entity1087 in the next 2 weeks. Results of reevaluation included transaminases around 1000 IU/L with minimal elevation of @entity623 ( @entity623 ), an antimitochondrial antibody (AMA) titer of 1:320, and an elevated immunoglobulin M (IgM). The antinuclear antibodies (ANA) level was positive, but titers were not obtained. There was no suggestion of @entity1448 . Liver biopsy findings were believed to be consistent with @entity2835 ( @entity2835 ). She was therefore started on, but failed treatment with, @entity10055 . She was transferred to a transplant center 8 weeks later after a brief episode of @entity75 and @entity4053 . The clinical findings were consistent with subfulminant @entity1578 secondary to @entity775 ( @entity775 ) with an ANA titer of 1:1280, an @entity3960 ( @entity3960 ) titer of 1:40, and an elevated IgG. Review of the biopsy showed panlobular @entity32 and bridging @entity39 consistent with severe @entity775 . On imaging, she had @entity843 and a nodular appearance of the liver. An immediate drop in transaminases followed corticosteroid therapy, but her disease was already irreversible, and she underwent successful liver transplantation. The explanted liver was shrunken and noncirrhotic with massive hepatocellular collapse and contained multiple regenerating nodules, explaining the ultrasonographic appearances. The inflammatory component had greatly diminished compared with the earlier biopsy. The case illustrates the importance of knowledge of the natural course of a specific disease and the careful interpretation of clinical data, including autoimmune markers. @entity2835 would rarely cause @entity1578 in a young @entity1 ; it is not a rapidly progressive disease. The original clinical diagnosis was unduly swayed by a positive AMA, which can be seen in up to 20% of @entity1 with @entity775 . Markedly elevated transaminases with minimal elevation of @entity623 and positive ANA in a young @entity1 should have pointed toward @entity775 at an earlier stage. The academic discussion of @entity775 remains intriguing, but prompt institution of aggressive immunosuppressive therapy aimed at the @entity775 component should not be deferred. In retrospect, an opportunity was missed.
