PBC is a progressive nonsuppurative destructive intrahepatic cholangitis. Strong female predominance, median age of 50 years. Presents as asymptomatic elevation in alkaline phosphatase (better prognosis) or with pruritus, progressive jaundice, consequences of impaired bile excretion, and ultimately cirrhosis and liver failure.
Clinical Manifestations
Pruritus, fatigue, jaundice, xanthelasma, xanthomata, osteoporosis, steatorrhea, skin pigmentation, hepatosplenomegaly, portal hypertension; elevations in serum alkaline phosphatase, bilirubin, cholesterol, and IgM levels.
Associated Diseases
Sjögren's syndrome, collagen vascular diseases, thyroiditis, glomerulonephritis, pernicious anemia, renal tubular acidosis.
Diagnosis
AMA in 90% (directed against enzymes of the pyruvate dehydrogenase complex and other 2-oxo-acid dehydrogenase mitochondrial enzymes). Liver biopsy most important in AMA-negative PBC. Biopsies identify four stages: stage 1-destruction of interlobular bile ducts, granulomas; stage 2-ductular proliferation; stage 3-fibrosis; stage 4-cirrhosis.
Prognosis
Correlates with age, serum bilirubin, serum albumin, prothrombin time, edema.
| TREATMENT | ||
Primary Biliary CirrhosisUrsodeoxycholic acid 13-15 mg/kg per day has been shown to improve the biochemical and histologic features of disease. Response is greatest when given early. Cholestyramine with meals for pruritus; in refractory cases consider rifampin, naltrexone, plasmapheresis. Calcium, vitamin D, and bisphosphonates are given for osteoporosis. Liver transplantation for end-stage disease. |