TY - JOUR
T1 - Comparison of the Clinicopathologic Features of Primary Sclerosing Chol-angitis and Primary Biliary Cirrhosis
AU - Wiesner, Russell H.
AU - Larusso, Nicholas F.
AU - Ludwig, Jurgen
AU - Rolland Dickson, E.
N1 - Funding Information:
Received April 5, 1983. Accepted June 29, 1984. Address requests for reprints to: Russell H. Wiesfler, M.D., Division of Gastroenterology, Mayo Clinic alid Mayo Foundation, Rochester, Minnesota 55905. This research was supported by National Institutes of Health grants AM19448 and RR585, by a grant from Merck, Sharp, and Dohme, and by the Mayo Foundation. Data analysis was performed using the CLINFO Data Analysis System. Part of this work was published previously in abstract form (GASTROENTEROLOGY 1982;82:1250). The authors thank Dawn Warren for secretarial assistance and Sandra Beaver and Roberta Jorgensen for serving as project coordinators for the PSC and PBC projects. © 1985 by the Mayo Foundation
PY - 1985
Y1 - 1985
N2 - Primary sclerosing cholangitis and primary biliary cirrhosis are chronic cholestatic syndromes that may be difficult to differentiate clinically. Destructive cholangitis occurs in both diseases and leads to similar clinical and biochemical abnormalities. Therefore, we compared the clinical, biochemical, immunologic, radiologic, and hepatic histologic features of these syndromes in two large groups of patients prospectively selected by predefined criteria. Primary biliary cirrhosis (n = 258) occurred predominantly in middle-aged women who were usually symptomatic with fatigue and pruritus, commonly had keratoconjunctivitis sicca, and often were hyperpigmented. Tests for antimitochondrial antibodies were always positive, usually in very high titer. Although the extrahepatic bile ducts were normal radiographically, smooth tapering and narrowing of the intrahepatic bile ducts was occasionally noted. Hepatic histology was diagnostic when a florid duct lesion was present. In contrast, primary sclerosing cholangitis (n = 60) occurred primarily in young men who were usually symptomatic with fatigue and pruritus and frequently had chronic ulcerative colitis. Tests for antimitochondrial antibodies were nearly always negative and cholangiography demonstrated abnormalities of the extrahepatic and intrahepatic bile ducts in all cases. Although hepatic histology was often compatible with the diagnosis, it was usually not diagnostic, and considerable overlap existed with the abnormalities seen in primary biliary cirrhosis. Likewise, biochemical tests of copper metabolism were similar in both syndromes. These results call attention to the differences and similarities in the clinicopathologic features of these two cholestatic syndromes and provide a basis for a rational diagnostic strategy.
AB - Primary sclerosing cholangitis and primary biliary cirrhosis are chronic cholestatic syndromes that may be difficult to differentiate clinically. Destructive cholangitis occurs in both diseases and leads to similar clinical and biochemical abnormalities. Therefore, we compared the clinical, biochemical, immunologic, radiologic, and hepatic histologic features of these syndromes in two large groups of patients prospectively selected by predefined criteria. Primary biliary cirrhosis (n = 258) occurred predominantly in middle-aged women who were usually symptomatic with fatigue and pruritus, commonly had keratoconjunctivitis sicca, and often were hyperpigmented. Tests for antimitochondrial antibodies were always positive, usually in very high titer. Although the extrahepatic bile ducts were normal radiographically, smooth tapering and narrowing of the intrahepatic bile ducts was occasionally noted. Hepatic histology was diagnostic when a florid duct lesion was present. In contrast, primary sclerosing cholangitis (n = 60) occurred primarily in young men who were usually symptomatic with fatigue and pruritus and frequently had chronic ulcerative colitis. Tests for antimitochondrial antibodies were nearly always negative and cholangiography demonstrated abnormalities of the extrahepatic and intrahepatic bile ducts in all cases. Although hepatic histology was often compatible with the diagnosis, it was usually not diagnostic, and considerable overlap existed with the abnormalities seen in primary biliary cirrhosis. Likewise, biochemical tests of copper metabolism were similar in both syndromes. These results call attention to the differences and similarities in the clinicopathologic features of these two cholestatic syndromes and provide a basis for a rational diagnostic strategy.
KW - IBD
KW - PBC
KW - PSC
KW - inflammatory bowel disease
KW - primary biliary cirrhosis
KW - primary sclerosing cholangitis
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U2 - 10.1016/S0016-5085(85)80141-4
DO - 10.1016/S0016-5085(85)80141-4
M3 - Article
C2 - 3880553
AN - SCOPUS:0021927412
SN - 0016-5085
VL - 88
SP - 108
EP - 114
JO - Gastroenterology
JF - Gastroenterology
IS - 1
ER -