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General Reference ⬇

Nejm 1995;332:924

Pathophys and Cause ⬆ ⬇

Cause:Genetic, plus viral precipitation?; HLA-DRw52a present in all, and HLA-Cw7 present in 86% (Nejm 1990;322:1842)

Pathophys:Ongoing inflammation, destruction, and fibrosis of intra- and extrahepatic bile ducts, possibly from toxic bacterial products of the gut?

Epidemiology ⬆ ⬇

Associated with inflammatory bowel disease often (70%), esp ulcerative colitis, and retroperitoneal/mediastinal fibrosis; highest incidence in young men; 70% males; 70% under age 45 yr

Signs and Symptoms ⬆ ⬇

Sx:Insidious (gteq.gif1 yr) onset, fatigue, pruritus, jaundice; attacks of RUQ pain and chills

Si:Jaundice, big liver, big spleen (one or more present upon dx in 75%)

Course ⬆ ⬇

Many fatal within 10 yr w/o early detection and rx, but many cases much more benign

Complications ⬆ ⬇

Cirrhosis and portal hypertension

r/o primary biliary cirrhosis

Lab and Xray ⬆ ⬇

Lab:

Chem:Alkaline phosphatase gteq.gif2× normal, ceruloplasmin and other copper studies elevated (75%), bilirubin elevated in 50%

Noninv:ERCP is diagnostic, showing narrowings and dilatations of ducts

Path:Liver biopsy shows cholangitis/portal hepatitis, and later periportal hepatic fibrosis with bridging and eventual biliary cirrhosis

Xray:Cholangiograms show strictures, irregular tortuosity of intra- and extrahepatic bile ducts; extrahepatic duct involvement distinguishes from primary biliary cirrhosis

Treatment ⬆

Rx:

None short of liver transplant; 70% 1-yr survival with transplant

Antibiotics for acute bouts of pain

Ursodiol (ursodeoxycholic acid) (Nejm 1997;336:691; Hepatol 1992;16:707); plus perhaps immunosuppressants (Ann IM 1999;131:943) like azathioprine, prednisone, and/or methotrexate (Gastroenterol 1994;106:494 vs Ann IM 1987;106:231), all may help LFTs but no effect on crs; ERC to rx strictures