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home - Liver - Autoimmune Conditions - Primary Biliary Cirrhosis 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 Biliary Cirrhosis Treatment

Written by Dr Sebastian Zeki Also: Metabolic bone disease in PBCThis is related to the duration and severity of PBC and to the intensity and duration of jaundice.The aetiology is of “Low-turnover" osteoporosis (bone formation is inhibited by cholestasis action on osteoblasts) and bone resorption is low or normal.Vitamin D levels are usually normal. Refer for OLT Not better- discontinue MTX Further rpt liver bx worse If rpt LFT’s normal, continue UDCA for 16m then do liver BxIf repeat LFTs abnormal or rpt liver bx abnormal, then add colchicine for 12 m Rpt liver bx UDCA (+?colchicine/ +?MTX) Portal hypertension/ jaundice Stage IV UDCA for 6m Stage I/II/III Liver biopsy Liver transplantation Naloxone (first infusion and then oral naltrexone Phenobarbital Rifampicin +Methotrexate +Colchicine UDCA PBC- Treatment UDCA -It improves LFT’s and reduces disease progression and possibly transplant-free survival.- It doesn’t relieve fatigue and pruritus.It is more effective in patients with early disease.Cirrhotics do not benefit from UDCA.Additional therapy is probably needed in patients with florid bile duct lesions and severe lymphocytic piecemeal necrosis and lobular inflamm-tion since these patients appear to be at increased risk for disease progression despite UDCA. Treatments:-Vitamin D supplemention- not useful unless the patient is deficient.-Calcium supplementation (at least 1500 mg/day)-especially post liver transplant.-Oestrogen therapy- in postmenopausal women with PBC this may be useful but not routinely recommended and not transdermal (first-pass hepatic metabolism).-Standard osteoporosis management applies.For severe symptomatic, refractory bone disease patients should be referred for a liver transplanta-tion (bone loss is accelerated 3-4 months after transplantation).Osteomalacia- Rare- this is due to vitamin D deficiency and fat malabsorption. Bile acids UDCA Bile acids UDCA decreases plasma and biliary endogenous [bile acid] possibly by competing for uptake or encouraging hepatic clearance of bile acids The endogenous bile acids have greater deter-gent activity and are therefore more hepatocyte toxic than UDCA. HLA I and II UDCA (or one of its metabolic conjugates) may decrease immune-mediated destruc-tion of hepatocytes (decreases the expression of HLA class I and II antigens) UDCA inhibits eosinophil activa-tion and degranula-tion. Mechanism of action of UDCA UDCA does not appear to influence the prevalence of florid bile duct lesions, suggest-ing that it exerts its beneficial effects by protec-ing against the consequence of bile duct destruc-tion rather than preventing it. Liver transplantation in primary biliary cirrhosisThe Mayo model is most widely used Optimal time for transplantationWhen 6m survival is < 80 %. (MELD or Mayo score)Transplantation Indications:In addition to Mayo scorePlasma [bilirubin] > 5 mg/dL and increasingSerum [albumin] < 2.8 g/dL (28 g/L) and decreasingSigns of decompensation or portal hypertension Intractable pruritusRecurrent, debilitating nontraumatic bone fracturesOutcome after liver transplantation -1y- survival 95 %Effect of transplantation on symptoms: Resolution rates vary.Pruritus/ complications of end-stage liver disease, resolve quicklyJaundice and ascites: days to months.Splenomegaly usually persists although the enlarged spleen may decrease slightly in size.Skin xanthomas also resolve within a few weeks.Hepatic osteodystrophy 12-18mPBC Recurrence in the transplant- 22 % after 10 years.Risk factors For RecurrenceOlder recipient ageLonger cold ischemia timeTreatment with tacrolimus (compared with cyclosporine) Younger donor age.TreatmentUDCA (effect on the natural history of recurrent PBC is uncertain) 1. Malabsorption ADEK supplementaion2. Lipid lowering - no need3. UDCA4. Liver transplantation = 50% 5 year survival5. Monitoring = 6/12 alpha fetoprotein + USS6. Azathioprine/steroids - marginal improvement7. For pruritis...(see section on pruritis in liver disease)8. For fatigue If severe, treat with modafinil (narcolepsy drug)9. Osteoporosis Pruritis in PBC

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