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Primary Sclerosing Cholangitis

The diagnosis is likely to be based on elevation of serum alkaline phosphatase found during a routine examination, a positive mitochondrial antibody found in autoantibody screening, or in an investigation of an associated disease involving collagen or thyroid primary sclerosing cholangitis, colangitis, sclorosing cholangitis. The course of these asymptomatic patients is extremely difficult to predict. Once symptoms develop, liver function deteriorates slowly during the


Eventually, all patients will develop symptoms. Fatigue is very common, affecting 68% of patients. It is associated with depression and poor sleep quality. (Cholestatic rats show behavior consistent with that seen in depression primary sclerosing cholangitis, colangitis, sclorosing cholangitis. With time, pruritus increases, bones become thin, jaundice deepens, and, finally, the picture is that of chronic hepatocellular failure. Hepatocellular carcinoma is rare in cirrhotic patients but is a relatively common cause of death in male PBC patients with cirrhosis primary sclerosing cholangitis, colangitis, sclorosing cholangitis.

Associated Conditions

Primary biliary cirrhosis is associated with almost every known or postulated autoimmune condition. Collagen diseases, especially Sjogren's syndrome, rheumatoid arthritis, and thyroid disorders are particularly common. Primary biliary cirrhosis may be associated with scleroderma and with calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telegiangiectasia (CREST) syndrome. Celiac disease may coexist; patients with PBC should be checked for antigliadin antibodies, and testing for PBC by checking mitochondrial antibodies should be performed in patients with celiac disease.

Pulmonary abnormalities include lymphocytic interstitial pneumonitis. Gas-transfer studies may be abnormal. Chest radiograms may show nodules and interstitial fibrosis. Mediastinal lymphadenopathy is common.


The course of PBC is variable and unpredictable. Some patients never become symptomatic, and others run a progressively 

At present, the patient with end-stage PBC faces possible liver transplantation.

Indications for Transplantation for Primary Biliary Cirrhosis

Quality of life
Bone thinning
Child class B or C
1-year survival rate, 90%

A rise in serum bilirubin level is a bad prognostic sign. The development of hepatocellular failure (edema, ascites, and encephalopathy) or significant portal hypertension (bleeding varices) is 

Prognostic models have been developed. The Mayo model depends on age, serum bilirubin, and albumin levels, prothrombin time, and edema. Serum bilirubin level and the Mayo model are very good predictors of survival. The Mayo model can yield a precise estimate of survival in an individual patient. Such models do not take into


General measures, including maintenance of nutrition and replacement of fat-soluble vitamins, apply to all patients with cholestasis.

The pathogenesis of pruritus in cholestatic patients is unknown, but recent evidence suggests increased neurotransmission and neuromodulation by endogenous opioids in the central nervous system. Pruritus is usually controlled by cholestyramine, but oral nalmefene and opiate antagonists have given promising results in

Osteomalacia is rare and can be corrected by vitamin D and calcium supplements. The major problem is osteoporosis, which is of low-turnover type. The osteoporosis may be related to the accumulation of toxins that impair osteoblast function. Calcitonin is of no value. Biphosphonates may be useful, and cyclical etidronate can be tried. Estrogens stimulate bone formation, and postmenopausal women with PBC should receive 

The recognition that PBC is probably an autoimmune disease led to trials of immunosuppressive drugs. The trials tended to be poorly controlled, with small numbers enrolled and short follow-up of participants. Drugs tested included azathioprine,

Ursodeoxycholic Acid

Ursodeoxycholic acid (URSO) is a nonhepatotoxic, hydrophilic bile acid that protects cell membranes against the detergent effect of hydrophobic bile acids, stimulates the excretion of toxic bile acids, and increases anion exchange in the liver. It reduces the expression of class 1 HLA on

Original studies from France showed that administration of URSO improved liver function and slowed the progression of disease, thus reducing probability of death or the need for transplantation. Results were less satisfactory if, on entry to the trial, the patient had a high serum bilirubin level and cirrhosis was present. Symptoms such as pruritus and fatigue are unaffected. A multinational controlled trial confirmed that URSO reduces the time to death or transplantation. It does not cure the disease. A 6-year follow-up study from Spain of early-stage cases showed biochemical and histologic improvement but no difference in time to

Liver Transplantation

Indications for liver transplantation are a poor quality of life, so that the patient is house-bound, has intractable pruritus, hepatocellular failure (deepening jaundice, encephalopathy, ascites), or bleeding varices. Results are better if referral is early. A model using age, renal failure, Child's class, and United Network for Organ Sharing (UNOS) criteria can be used to predict the effects of transplantation in terms of intraoperative blood loss, time in intensive care, and severe complications. Results are

The 1-year survival rate after transplantation is about 85% to 90%, and a 5-year survival rate of 60% to 70% is reported. Twenty-five percent of patients will need a retransplant, usually because of the development of the