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home - Liver - Autoimmune Conditions - Primary Sclerosing Cholangitis Pathogenesis Written by Dr Sebastian Zeki
Knowledge


Recognises and appropriately investigates patients with auto-immune
liver diseases

Aware of management and complications of autoimmune liver
disease including extra-hepatic manifestations and associations
including malignant complications in PSC

Skills
Appreciates and understands that this range of liver disease is
frequently under-diagnosed and may have been inappropriately
managed

Selects appropriate immunomodulatory therapy has awareness of
side effects and may well require specialist care

Behaviours
Responds urgently to the management challenge of these severe and
often acute diseases and involves more specialist services where
required

Primary Sclerosing Cholangitis Pathogenesis

Immune activationHumoral and cellular immunity (esp CD4+ vs bile duct epithelial cells) is increased.Bile duct cells in PSC express antigens which cross-react with colonic epithelial cells.Bile duct cells aberrantly express HLA class II antigens, and ICAM (intercellular adhesion molecule)-1 is expressed by ductular epithelial cells. Cirrhosis Stage I Damage to isolated bile ducts. Enlargement, edema, and scarring of the portal triadsMononuclear cell infiltration Piecemeal necrosis Stage II — Expansion of portal triads with fibrosis extending into the surrounding paren-chyma Stage III — Bridging fibrosis Stage IV — Cirrhosis Histology Aetiologies:Autoimmune.IBD, sarcoidosis, Riedel's thyroiditis and coeliac disease.A viral association.REO, CMV, HIV.Genetic- HLA-B8, -DR3, and -DRw52a , MICA. PSC Pathogenesis Genetic factorsThere is an increased prevalence of HLA-B8, -DR3, and -DRw52a.HLA-B8 is present in 70 % (vs. 25 % of control).HLA-B8 is associated with a number of other autoimmune diseases, such as coeliac disease, myaesthenia gravis, and diabetes mellitus.Bacterial infections from colon to liver- scant evidence present.Ischaemic ductal injury- this gives a similar picture to PSC.CFTR mutations- present in 67% of PSC patients. Written by Dr Sebastian Zeki

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