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home - Liver - Autoimmune Conditions - Primary Sclerosing Cholangitis 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

Primary sclerosing cholangitis Secondary sclerosing cholangitis causes: Operative trauma (most common). Choledocholithiasis. Chronic pancreatitis. Ischaemic cholangiopathy. Histiocytosis X. Cholangiocarcinoma. PruritusSteatorrhea and vitamin deficiency (decreased secretion of conjugated bile acids into the small intestine)Vitamin A deficiency- 82 % In addition, vitamin D and vitamin E deficiencies in 50% with advanced disease.Metabolic bone disease35% have osteoporosis- not vitamin D deficient- probably same aetiology as PBCSame treatment as PBCDOMINANT BILIARY STRICTURES20 % get a dominant stricture- can occur anywhere.Need to be brushed for cytology.Medical treatment doesnt work.Endoscopic stents or balloons should be tried first.CHOLANGITIS AND CHOLELITHIASIS30% have cholesterol and/or pigment stones.Treat as galltones in other patientsCHOLANGIOCARCINOMA15 % lifetime risk of developing cholangiocarcinoma (IBD and cirrhosis highest risk)Diagnosis and screeningDifficult to distinguish a dominant stricture from a cholangiocarcinoma with any modalityScreening not usefulPrognosis 10 % of patients survived two years in one report.Transplantation not helpfulCOLON CANCERIncreased risk of CRC so need to be on surveillance programme Clinical ManifestationsFatigue and pruritus are common features at presentation.Patients are usually asymptomatic with abnormal LFT's.Cholangitis and Fat malabsorption can occur CHOLESTASIS BILIARY ANATOMY COLON Complications zzzzz Abn LFTS’s ADEK ALP ^^^ Bilirubin ^ ALT < 300 IU/L. IgG ^ ( 30 %) IgM ^ (45 %) Elevated serum IgG4 - 10%. Hepatic and urinary copper levels inc and serum ceruloplasmin is reduced in most patients with PSC. Antibodies Atypical (P-ANCA) 60 % HLA DRw52a 50% ANA/SMA/anticardiolipin, thyroperoxidase, and RA may be present AMA negative 97 % positive for 1/+ autoantibody;81 % positive for >3. Only anticardiolipin antibodies correlated with disease severity X-Ray Characteristic multifocal stricturing (focal or diffuse) and dilation of intrahepatic and/or extrahepatic bile ducts Intrahepatic alone11 % ; Extrahepatic alone 2 %; Both 87 % NB Small duct PSC: Cholestasis Liver histology compatible with PSC Normal biliary tree on cholangiography Usually also have IBD Epidemiology Prevalence of UC in patients with PSC is 90 %. Prevalence of PSC in patients with IBD is 5%. 70% are male and diag - nosed by the age of 40. PrognosisMedium survival is 9-12 years after diagnosis.Mayo risk score for modelling is based on bilirubin;Albumin; PT;Oedema; Age.Other things that predict survivalinclude splenomegaly and histol-ogy. Labs = bile duct characterised by information/fibrosis/thickening/strictures Written by Dr Sebastian Zeki

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