SUMMARY Twenty-nine patients with primary sclerosing cholangitis were reviewed. Males predominated (2:1). Seventy-six per cent presented with cholestasis and cholangitis, 17 % with cirrhosis and portal hypertension, and 7% were asymptomatic, presenting with a raised serum alkaline phosphatase. The serum immunoglobulin IgM concentration was raised in 45 % of the patients, but no patient had serum mitochondrial antibody present. Seventy-two per cent had ulcerative proctocolitis. There was no relationship between either duration or severity of ulcerative proctocolitis and the development of primary sclerosing cholangitis. Four patients were not benefited by colectomy. None of the patients had Crohn's disease. The prognosis was variable. Corticosteroids and azathioprine were ineffective. Eleven patients (38 %) had died with a mean survival time of seven years from diagnosis. Three patients with ulcerative proctocolitis developed bile duct carcinoma. The cholangiograms and liver biopsies were reported without reference to clinical information together with 41 patients with other biliary diseases. Cholangiography was diagnostic in 18/22 (82 %). Hepatic histology was diagnostic in 8/22 (36 %). Ten showed features of large bile duct disease and three were misdiagnosed as primary biliary cirrhosis. Reduced numbers of bile ducts, ductular proliferation, portal inflammation, and substantial copper deposition, in combination with piecemeal necrosis, are commonly seen in primary sclerosing cholangitis and indicate the need for cholangiography.Primary sclerosing cholangitis (PSC), first described by Delbett in 1924, is a rare disease of unknown aetiology. It is characterised by an intense inflammatory fibrosis usually affecting both the intra-and extrahepatic biliary tree.2Reported series of patients with PSC have been small34 and until the advent of percutaneous and endoscopic cholangiography did not include detailed descriptions of the biliary tree. The present paper describes the clinical features, natural history, and treatment of 29 patients with primary sclerosing cholangitis.Cholangiographic and hepatic histological appear-

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1. Pruritus was assessed in 19 patients by measurement of nocturnal limb movement. 2. Serum (nine pruritic, ten non-pruritic) and interstitial fluid (five pruritic, three non-pruritic) bile acids were fractionated according to their mode of conjugation by using DEAP-Sephadex LH-20 and measured by gas chromatography-mass spectrometry. 3. No correlation was found between serum or interstitial fluid total bile acid or individual bile acid concentrations and pruritus. Bile acid profiles in the two groups of patients were similar and there was no correlation between pruritus and the conjugation pattern. 4. Te bile acid profile of interstitial fluid reflected that of serum and a linear relationship was found between serum and interstitial fluid bile acid concentrations (r ;.95, P less than 0.001). 5. The proportion of bile acid sulphate in interstitial fluid was significantly smaller than that in serum (P less than 0.025), where sulphates accounted for up to 46% of the total bile acids. 6. In three patients, a decrease in serum bile acid concentrations achieved by percutaneous transhepatic biliary drainage had little or no effect on pruritus. 7. These findings suggest that bile acids do not have a causative role in the pruritus of cholestatic liver disease.

In a retrospective study of 755 patients undergoing endoscopic retrograde cholangiopancreatography from 1973 to 1977 at the Royal Free Hospital, 38 (5%) had a juxtapapillary diverticulum. This resulted in a significantly higher failure rate of cannulation of the ampulla of Vater (40 vs. 20% in the remainder of the group; p < 0.02). The presence of a juxtapapillary diverticulum was also significantly associated with an increase of cholelithiasis (48 vs. 24%; p < 0.01), and with jaundice or cholangitis developing after cholecystectomy (27 vs. 8%; p < 0.001). There was no evidence that the diverticula were the cause of biliary or pancreatic obstruction.

SUMMARY The bile ducts were visualised using endoscopic retrograde cholangiopancreatography (ERCP), percutaneous or intravenous cholangiography in 38 patients with non-gallstone chronic pancreatitis. Stenosis of the intrapancreatic portion of the distal common bile duct was demonstrated in 11 patients. Ten of the 11 developed transient cholestasis during exacerbations of their chronic pancreatitis. In six cholestasis eventually persisted requiring surgical relief. Secondary biliary cirrhosis was present in one patient. No evidence of pancreatic carcinoma was found in the patients explored surgically. Ten of the patients are alive more than one year after diagnosis. Chronic pancreatitis was of alcoholic aetiology in 10 of the patients with biliary stenosis. Cholestasis and biliary stricture are common but poorly recognised complications of non-gallstone chronic pancreatitis, especially when pancreatitis is severe and due to alcohol.

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