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©2008 The WJG Press and Baishideng.
World J Gastroenterol. Jan 21, 2008; 14(3): 331-337
Published online Jan 21, 2008. doi: 10.3748/wjg.14.331
Published online Jan 21, 2008. doi: 10.3748/wjg.14.331
Table 1 Hepatobiliary disorders associated with inflammatory bowel disease
Hepatobiliary disorders | Associated with | |
Ulcerative colitis | Crohn’s disease | |
Primary sclerosing cholangitis (PSC) | ||
Large duct PSC | + | + |
Small duct PSC (‘pericholangitis’) | + | + |
Cirrhosis | + | + |
Hepatoma | + | + |
Cholangiocarcinoma | + | + |
Miscellaneous disorders | ||
Fatty liver | + | + |
Granulomas | + | |
Amyloidosis | + | |
Hepatic abscess | + | |
Gallstones | + | |
Autoimmune hepatitis | + | |
Primary biliary cirrhosis | (+) | |
Budd-Chiari syndrome | (+) | (+) |
Table 2 Prevalence of primary sclerosing cholangitis in patients with ulcerative colitis
Table 3 Prevalence of inflammatory bowel disease in patients with primary sclerosing cholangitis (PSC)
Table 4 Evidence for the influence of immune mechanisms on the aetiology of PSC
Humoral immunity | Increased circulating immune complexes |
Elevated immunoglobulin levels (IgG and IgM) | |
Low titres of non-organ specific autoantibodies (ANA and SMA) | |
High titres of antineutrophil antibodies | |
Autoantibodies to BEC surface antigens | |
Cell mediated immunity | Decreased levels of circulating peripheral CD8+ve Tcells |
Portal T cell and NK cell infiltrate | |
Increased activated and memory T cells | |
Restricted T cell receptor repertoire (Vβ3) | |
Aberrant expression of HLA-DR on BEC | |
Coexpression of costimulatory molecules and HLA-DR on BECs | |
Increased circulating and tissue bound adhesion molecules | |
Immune effector mechanisms | Enhanced cytokine expression in the liver |
Immunogenetic mechanisms | HLA associations |
Table 5 Key HLA haplotypes associated with primary sclerosing cholangitis
Haplotype | Significance in PSC |
B8-TNF*2-DRB3*0101-DRB1*0301-DQA1*0501-DQB1*0201 | Strong association with disease susceptibility |
DRB3*0101-DRB1*1301-DQA1*0103-DQB1*0603 | Strong association with disease susceptibility |
DRB5*0101-DRB1*1501-DQA1*0102-DQB1*0602 | Weak association with disease susceptibility |
DRB4*0103-DRB1*0401-DQA1*03-DQB1*0302 | Strong association with protection against disease |
MICA*008 | Strong association with disease susceptibility |
Table 6 Clinical, Endoscopic, and Histological findings that characterize IBD-PSC
Male predominance |
Quiescent colitis |
Pancolitis |
Rectal sparing |
Backwash ileitis |
Pouchitis |
Colorectal dysplasia/carcinoma |
Different extracolonic manifestations |
- Citation: Saich R, Chapman R. Primary sclerosing cholangitis, autoimmune hepatitis and overlap syndromes in inflammatory bowel disease. World J Gastroenterol 2008; 14(3): 331-337
- URL: https://www.wjgnet.com/1007-9327/full/v14/i3/331.htm
- DOI: https://dx.doi.org/10.3748/wjg.14.331