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home - Liver - Autoimmune Conditions - Primary Sclerosing Cholangitis Treatment 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 Treatment

Treatment 1. As per cholestasis (care of pruritis/ vitamin ADEK deficiency, osteopoenia). 2. Lipid lowering agents not recommended. 3. Ursodeoxycholic acid. Mechanism of Action of ursodeoxycholic acid The does is (15mg/kg).It protects cholangiocytes against cytotoxic hydrophobic bile acids.It stimulates hepatobiliary secretion.It protects hepatocytes against bile-acid induced apoptosis.It induces antioxidants.It improves biochemistry but no survival benefit or a delay in the need for liver transplantation is seen.Higher doses may show benefit. Biliary reconstructionNot really done as not curativeThe only exception is in patients with isolated focal extrahepatic strictures and early disease on biopsyProctocolectomyOnly if it is indicated because of the colitis as doesnt help the PSCLiver transplantation- Treatment of choice for PSC related advanced liver disease.5 year survival of 86% with liver transplantDescision to transplant based on MELD score. There are special circumstances in which liver transplantation is indicated despite a low priority MELD score:-Recurrent or refractory cholangitis-Intractable pruritus-Peripheral or hilar cholangiocarcioma (in the context of a clinical trial).Recurrent Disease Post Transplantation- 20% of patients. 30% need retransplantationThe natural history of UC following liver transplantation is variable.Although the disease may remain quiescent in some patients, it iscome aggressive in others despite the immunosuppression used for the transplant.Two independent risk factors for IBD progression were older age at the time of transplant and liver transplantation itself.CRC incidence is increased in patients with UC and PSC who undergo liver transplantation so need yearly colonoscopy post transplant URSO BILE ACIDS URSO Primary Sclerosing Cholangitis: Treatment Endoscopic TherapyShort-term stenting is beneficial for patients with a dominant extrahepatic biliary stricture as it can improve the cholestasis.If not stented, the LFTs often dont get worse.Whether dilation alone without stenting could produce similar results is being investigated.These patients always need antibiotics when being ERCP'd. Surgical Therapy Risk factors for recurrence:Age.Sex mismatch.Male sex.Coexistent IBD.Presence of an intact colon after transplantation.CMV infection, recurrent acute cellular rejection.Steroid-resistant cellular rejection.Use of OKT3.Presence of cholangiocarcinoma before transplantation.Use of extended donor criteria.Prolonged use of corticosteroids.Ulcerative Colitis. Written by Dr Sebastian Zeki

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