A 39 Year Old Man with Chronic Hepatitis

Abstract

The diagnosis of primary sclerosing cholangitis in association with autoimmune hepatitis was made in this case after the discovery of inflammatory bowel disease prompted a cholangiogram. Immunosuppressive therapy with cyclosporine prior to ERCP resulted in a significant decrease in serum aminotransferase. Liver histology and autoimmune serologies favored a diagnosis of AIH, while cholangiographic findings suggested PSC. A review of similar cases describing the simultaneous occurrence of AIH and PSC is presented. The shared autoimmune characteristics of the two diseases, such as serum autoantibodies, peripheral lymphocyte subsets and HLA haplotypes, raises the question of whether similar mechanisms of immune dysfunction can result in both processes. The existence of a PSC-AIH overlap syndrome may help provide the linkage between the two diseases.

EASL Clinical Practice Guidelines: Management of cholestatic liver diseases

“…PSC-AIH overlap syndrome is an ill-defined immune-mediated disorder which is predominantly found in children, adolescents and young adults [98,[140][141][142][143][144][145][146][147][148]. Its characteristics include clinical, biochemical, and histologic features of AIH as summarized in the modified AIH score defined by an international group of experts for study purposes [62] and cholangiographic features typical of PSC [60].…”

Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: A 16-year prospective study

“…Median follow-up time was 7 (2-16) years (ASC: 6 [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] vs. AIH: 8 [3][4][5][6][7][8][9][10][11][12][13][14][15]; P ϭ .17).…”

“…1 An overlapping syndrome between autoimmune hepatitis (AIH) and sclerosing cholangitis has been anecdotally reported on several occasions both in children [2][3][4] and adults. [5][6][7][8] A retrospective study 4 from our Unit on 13 children with sclerosing cholangitis showed that in 40% of the cases, the clinical, biochemical, immunological, and histological presentation was indistinguishable from that of AIH. In both conditions, there was an increase in the levels of immunoglobulin G (IgG) and presence of circulating non-organ-specific nuclear (ANA) and/or smooth muscle (SMA) autoantibodies.…”

“…Histological abnormalities common to both diseases were inflammatory portal tract infiltrate and interface hepatitis. Most of the reported overlapping cases [4][5][6][7][8] were originally diagnosed as AIH, the diagnosis of sclerosing cholangitis having been made years later after demonstration of characteristic bile duct changes on cholangiography, which was performed because of the development of biliary features on follow-up liver biopsy. This has been interpreted as evolution from AIH to sclerosing cholangitis, although cholangiographic studies excluding sclerosing cholangitis at presentation were not available.…”

Clinical Presentation and Outcomes of Autoimmune Hepatitis in Inflammatory Bowel Disease

“…Per AIHG guidelines, typically each disease is treated separately according to the predominant features [19]. Treatment of the overlap syndrome in the majority of cases consists of corticosteroids or combination of corticosteroids and azathioprine [4,[21][22][23][24][25][26][27][28][29][30][31].…”