{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T21:52:26Z","timestamp":1790805146651,"version":"4.1.0"},"reference-count":27,"publisher":"BMJ","issue":"10","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Gut"],"published-print":{"date-parts":[[1980,10]]},"abstract":"<jats:p>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 ahd Crohn's disease. The prognosis was variable. Corticosteriods 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.<\/jats:p>","DOI":"10.1136\/gut.21.10.870","type":"journal-article","created":{"date-parts":[[2007,9,18]],"date-time":"2007-09-18T17:41:31Z","timestamp":1190137291000},"page":"870-877","source":"Crossref","is-referenced-by-count":577,"title":["Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology."],"prefix":"10.1136","volume":"21","author":[{"given":"R W","family":"Chapman","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B A","family":"Arborgh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J M","family":"Rhodes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J A","family":"Summerfield","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R","family":"Dick","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P J","family":"Scheuer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S","family":"Sherlock","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[1980,10,1]]},"reference":[{"key":"ref_1","article-title":"Retrecessement du choledoque: cholecystoduodenostomie. Bull Mem Soc Nat Chir 1924; 1144","author":"Delbet, P.","unstructured":"Delbet P. Retrecessement du choledoque: cholecystoduodenostomie. Bull Mem Soc Nat Chir 1924; 1144."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1136\/gut.8.5.435","article-title":"Primary sclerosing cholangitis, the biliary tree and ulcerative colitis","volume":"8","author":"Thorpe, M.E.C.; Scheuer, P.J.; Sherlock, S.","year":"1967","unstructured":"Thorpe MEC, Scheuer PJ, Sherlock S. Primary sclerosing cholangitis, the biliary tree and ulcerative colitis. Gut 1967; 8: 435-48.","journal-title":"Gut"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1016\/0002-9610(65)90018-8","article-title":"Sclerosing cholangitis","volume":"110","author":"Smith, M.P.; Loe, R.H.","year":"1965","unstructured":"Smith MP, Loe RH. Sclerosing cholangitis. Am J Surg 1965; 110: 239-46.","journal-title":"Am J Surg"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1016\/0002-9610(66)90339-4","article-title":"Primary sclerosing cholangitis","volume":"111","author":"Warren, K.W.; Athanassiades, S.; Monge, J.I.","year":"1966","unstructured":"Warren KW, Athanassiades S, Monge JI. Primary sclerosing cholangitis. Am J Surg 1966; 111: 23-38.","journal-title":"Am J Surg"},{"key":"ref_5","first-page":"109","article-title":"Primary sclerosing cholangitis","volume":"65","author":"Danzi, J.T.; Makipour, H.; Farmer, R.G.","year":"1976","unstructured":"Danzi JT, Makipour H, Farmer RG. Primary sclerosing cholangitis. Am J Gastroenterol 1976; 65: 109-16.","journal-title":"Am J Gastroenterol"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1148\/95.2.369","article-title":"The roentgenologic appearance of sclerosing cholangitis","volume":"95","author":"Krieger, J.; Seaman, W.B.; Porter, M.R.","year":"1970","unstructured":"Krieger J, Seaman WB, Porter MR. The roentgenologic appearance of sclerosing cholangitis. Radiology 1970; 95: 369-75.","journal-title":"Radiology"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1016\/S0016-5085(76)80016-9","article-title":"The biliary system in primary biliary cirrhosis","volume":"70","author":"Summerfield, J.A.; Elias, E.; Hungerford, G.D.","year":"1976","unstructured":"Summerfield JA, Elias E, Hungerford GD, et al. The biliary system in primary biliary cirrhosis. Gastroenterology 1976; 70: 240-3.","journal-title":"Gastroenterology"},{"key":"ref_8","first-page":"12","article-title":"Liver biopsy interpretation","author":"Scheuer, P.J.","year":"1980","unstructured":"Scheuer PJ. Liver biopsy interpretation. London: Bailliere Tindall, 1980: 12."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"674","DOI":"10.1056\/NEJM197309272891306","article-title":"The presentation and diagndsis of 100 patients with primary biliary cirrhosis","volume":"289","author":"Sherlock, S.; Scheuer, P.J.","year":"1973","unstructured":"Sherlock S, Scheuer PJ. The presentation and diagndsis of 100 patients with primary biliary cirrhosis. N Engi J Med 1973; 289: 674-8.","journal-title":"N Engi J Med"},{"key":"ref_10","first-page":"299","article-title":"The course and prognosis of ulcerative colitis. Part 1","volume-title":"Gut","volume":"4","author":"Edwards, F.C.; Truelove, S.C.","year":"1963","unstructured":"Edwards FC, Truelove SC. The course and prognosis of ulcerative colitis. Part 1. Gut 1963; 4: 299-308."},{"key":"ref_11","first-page":"\"777","article-title":"Thompson BW, Read RC. Sclerosing cholangitis and other intra-abdominal fibroses","volume":"128","author":"Br Med J","year":"1974","unstructured":"Thompson BW, Read RC. Sclerosing cholangitis and other intra-abdominal fibroses. Am J Surg 1974; 128: 777-81. 2Anonymous. An unusual cholangitis. Br Med J 1976; 2: 1090-1.","journal-title":"Am J Surg; 2Anonymous. An unusual cholangitis"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1136\/gut.9.2.193","article-title":"Immunoglobulins in chronic liver disease","volume":"9","author":"Feizi, T.","year":"1968","unstructured":"Feizi T. Immunoglobulins in chronic liver disease. Gut 1968; 9: 193-8.","journal-title":"Gut"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1093\/oxfordjournals.bmb.a070914","article-title":"Immunological aspects of viral hepatitis","volume":"28","author":"Holborrow, E.J.","year":"1972","unstructured":"Holborrow EJ. Immunological aspects of viral hepatitis. Br Med Bull 1972; 28: 142-4.","journal-title":"Br Med Bull"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1056\/NEJM196307042690102","article-title":"Sclerosing cholangitis","volume":"269","author":"Bartholomew, L.G.; Cain, J.C.; Woolner, L.B.; Utz, D.C.; Ferris, D.O.","year":"1963","unstructured":"Bartholomew LG, Cain JC, Woolner LB, Utz DC, Ferris DO. Sclerosing cholangitis. N Engl J Med 1963; 269: 8-12.","journal-title":"N Engl J Med"},{"key":"ref_15","first-page":"183","article-title":"Sclerosing cholangitisassociation with regional enteritis","volume":"188","author":"Atkinson, A.J.; Carroll, W.W.","year":"1964","unstructured":"Atkinson AJ, Carroll WW. Sclerosing cholangitisassociation with regional enteritis. JAMA 1964; 188: 183-4.","journal-title":"JAMA"},{"key":"ref_16","first-page":"113","article-title":"The spectrum of hepatic dysfunction in inflammatory bowel disease","volume":"48","author":"Dew, M.J.; Thompson, H.; Allan, R.N.","year":"1979","unstructured":"Dew MJ, Thompson H, Allan RN. The spectrum of hepatic dysfunction in inflammatory bowel disease. Q JMed 1979; 48: 113-35.","journal-title":"Q JMed"},{"key":"ref_17","first-page":"263","article-title":"Biliary tract carcinoma associated with ulcerative colitis","volume":"43","author":"Ritchie, J.K.; Allan, R.N.; Macartney, J.; Thompson, H.; Hawley, P.R.; Cooke, W.T.","year":"1974","unstructured":"Ritchie JK, Allan RN, Macartney J, Thompson H, Hawley PR, Cooke WT. Biliary tract carcinoma associated with ulcerative colitis. Q J Med 1974; 43: 263-79.","journal-title":"Q J Med"},{"issue":"10","key":"ref_18","first-page":"886","article-title":"Primary intrahepatic obliterating cholangitis, a possible variant of 'sclerosing cholangitis'","volume":"1969","author":"Bhathal, P.S.; Powell, L.W.","unstructured":"Bhathal PS, Powell LW. Primary intrahepatic obliterating cholangitis, a possible variant of 'sclerosing cholangitis'. Gut 1969; 10: 886-93.","journal-title":"Gut"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"907","DOI":"10.1016\/S0016-5085(19)32742-8","article-title":"Endoscopic retrograde cholangiopancreatography in the diagnosis of jaundice associated with ulcerative colitis","volume":"67","author":"Elias, E.; Summerfield, J.A.; Dick, R.; Sherlock, S.","year":"1974","unstructured":"Elias E, Summerfield JA, Dick R, Sherlock S. Endoscopic retrograde cholangiopancreatography in the diagnosis of jaundice associated with ulcerative colitis. Gastroenterology 1974; 67: 907-11.","journal-title":"Gastroenterology"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"143","DOI":"10.3949\/ccjm.41.3.143","article-title":"Endoscopic retrograde cholangiographic evaluation of sclerosing cholangitis","volume":"41","author":"Lefton, H.B.; El, Winkelman","year":"1974","unstructured":"Lefton HB, Winkelman El. Endoscopic retrograde cholangiographic evaluation of sclerosing cholangitis. Cleve Clin Q 1974; 41: 143-7.","journal-title":"Cleve Clin Q"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1007\/BF01072745","article-title":"Retrograde cholangiography and sclerosing cholangitis","volume":"19","author":"Abbruzzese, A.A.","year":"1974","unstructured":"Abbruzzese AA. Retrograde cholangiography and sclerosing cholangitis. Am J Dig Dis 1974; 19: 571-4.","journal-title":"Am J Dig Dis"},{"issue":"34","key":"ref_22","first-page":"41","article-title":"Copper and liver disease","volume":"10","author":"Ritland, S.; Steinnes, E.","year":"1975","unstructured":"Ritland S, Steinnes E. Copper and liver disease. Scand J Gastroenterol 1975; 10: suppl 34, 41-2.","journal-title":"Scand J Gastroenterol"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1080\/00365521.1977.12031116","article-title":"Hepatic copper content, urinary copper excretion and serum ceruloplasmin in liver disease","volume":"12","author":"Ritland, S.; Steinnes, E.; Skrede, S.","year":"1977","unstructured":"Ritland S, Steinnes E, Skrede S. Hepatic copper content, urinary copper excretion and serum ceruloplasmin in liver disease. Scand J Gastroenterol 1977; 12: 81-8.","journal-title":"Scand J Gastroenterol"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1","DOI":"10.7326\/0003-4819-63-1-1","volume":"63","author":"Pathology, etiology; pathogenesis","year":"1965","unstructured":"Pathology, etiology and pathogenesis. Ann Intern Med 1965; 63: 1-16.","journal-title":"Ann Intern Med"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"17","DOI":"10.7326\/0003-4819-63-1-17","article-title":"Pericholangitis and ulcerative colitis: II Clinical aspects","volume":"63","author":"Mistilis, S.P.; Skyring, A.P.; Goulston, S.J.M.","year":"1965","unstructured":"Mistilis SP, Skyring AP, Goulston SJM. Pericholangitis and ulcerative colitis: II Clinical aspects. Ann Intern Med 1965; 63: 17-26.","journal-title":"Ann Intern Med"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1007\/BF01072591","article-title":"Cholangiographic abnormalities in ulcerative colitis associated pericholangitis which resemble sclerosing cholangitis","volume":"23","author":"Blackstone, M.O.; Nemchausky, B.A.","year":"1978","unstructured":"Blackstone MO, Nemchausky BA. Cholangiographic abnormalities in ulcerative colitis associated pericholangitis which resemble sclerosing cholangitis. Am J Dig Dis 1978; 23: 579-85.","journal-title":"Am J Dig Dis"},{"key":"ref_27","first-page":"2465","article-title":"La cholangeite sclerosante primitive","volume":"28","author":"Roseau, E.","year":"1978","unstructured":"Roseau E. La cholangeite sclerosante primitive. Nouv Presse Med 1978; 28: 2465-7.","journal-title":"Nouv Presse Med"}],"container-title":["Gut"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/gut.21.10.870","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,8]],"date-time":"2025-09-08T06:19:27Z","timestamp":1757312367000},"score":1,"resource":{"primary":{"URL":"https:\/\/gut.bmj.com\/lookup\/doi\/10.1136\/gut.21.10.870"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1980,10]]},"references-count":27,"journal-issue":{"issue":"10","published-online":{"date-parts":[[1980,10,1]]},"published-print":{"date-parts":[[1980,10]]}},"alternative-id":["10.1136\/gut.21.10.870"],"URL":"https:\/\/doi.org\/10.1136\/gut.21.10.870","relation":{},"ISSN":["0017-5749","1468-3288"],"issn-type":[{"value":"0017-5749","type":"print"},{"value":"1468-3288","type":"electronic"}],"subject":[],"published":{"date-parts":[[1980,10]]}}}