- Email: [email protected]
Severe bile duct lesions without biochemical evidence of cholestasis in a case of sclerosing cholangitis
Journal of Hepatology, 1986;3:72-74 Elsevier
72 HEP 00143
Severe Bile Duct Lesions Without Biochemical Evidence of Cholestasis in a Case of Sclerosing Cholangitis
D. C l e m e n t s , J . M . R h o d e s a n d E . Elias Department of Medicine, Queen Elizabeth Hospital, Edgbaston, Birmingham BI5 (U. K.) (Received 29 November, 1985) (Accepted 5 February, 1986)
Summary
We report a patient with very marked cholangiographic changes of sclerosing cholangitis with normal serum alkaline phosphatase and no other detectable biochemical evidence of cholestasis. He has remained asymptomatic and still has no biochemical evidence of cholestasis despite follow-up over 3 years. This case demonstrates that patients with sclerosing cholangitis may have normal liver function tests including serum alkaline phosphatase, that asymptomatic sclerosing cholangitis may therefore be under diagnosed, and that severe radiological changes of sclerosing cholangitis may not necessarily be associated with a poor prognosis.
Introduction
Primary sclerosing cholangitis is a disease of unknown aetiology characterised by inflammation and stricturing of the intra- and extrahepatic bile ducts. It is associated with ulcerative colitis in over 70% of cases [1]. In the past when the diagnosis was made almost exclusively in patients with cholestatic jaundice and/or cholangitis, the patients generally had a poor prognosis. More recently endoscopic retrograde cholangiography, a reliable diagnostic test for this condition [1,2], has shown that asymptomatic patients may have extensive bile duct lesions [3]. These have typi-
cally been patients with ulcerative colitis who have been investigated because of elevated serum alkaline phosphatase.
Case Report
A 56-year-old Caucasian man with a 3-year history of episodic abdominal pain underwent cholecystectomy in 1982. Operative cholangiography showed that there were two strictures in the common bile duct. The gallbladder contained several small stones. He made an uneventful post-operative recovery and bio-
Address for correspondence: Dr. D. Clements, Department of Medicine, Queen Elizabeth Hospital, Edgbaston, Birmingham B15 2TH, U.K. 0168-8728/86/$03.50© 1986Elsevier SciencePublishers B.V. (BiomedicalDivision)
BILE DUCT LESIONS IN SCLEROSING CHOLANGITIS
73
Fig. 1. Endoscopic retrograde cholangiogram showing marked stricturing and beading of intrahepatic and extrahepatic bile ducts diagnostic of sclerosing cholangitis.
chemical tests were normal: bilirubin 11/lmol/1 (normal 1-25), alkaline phosphatase 175 IU/I (normal 70-350), aspartate transaminase 17 IU/I (normal 5-35). It was noted that he had been diagnosed as having ulcerative colitis since the age of 16 but had no symptoms since the age of 20 and had been receiving no treatment for it. In 1983 he had intermittent epigastric pain so was referred for endoscopic retrograde cholangiopancreatography (ERCP). There had been no pruritus. Biochemical tests at this time were still normal (bilirubin 16 !Lmol/l, alkaline phosphatase 226 IU/1, aspartate transaminase 19 IU/I, fasting serum bile acids 6.3 g~mol/1 [normal <7.5 !lmol/l]). Haemoglobin, white cell and platelet estimations were all normal
and the ESR was 2 mm/h. ERCP showed florid stricturing and beading of intrahepatic and extrahepatic bile ducts diagnostic of sclerosing cholangitis (Fig. 1). The pancreatic duct was normal. Barium enema examination showed slight granularity of the mucosa of the rectum and sigmoid colon but was otherwise normal. At sigmoidoscopy the rectal mucosa appeared granular and mucosal biopsy showed mild chronic inflammatory changes. His epigastric pain subsequently resolved without treatment, and he has remained completely asymptomatic for 27 months since the ERCP examination and still has no biochemical evidence of cholestasis 3 years after cholecystectomy. He is currently prescribed sulphasalazine 2 g/day.
74 Discussion
This case shows remarkable disparity between the severe bile duct lesions demonstrated by E R C P and normal biochemical tests over prolonged follow-up. Not only were the serum alkaline phosphatase and bilirubin normal but also the fasting serum bile acid concentration which should be a sensitive screening test for cholestasis or hepatocellular dysfunction [4]. The duration of the sclerosing cholangitis in this patient is unknown. It seems probable that the sclerosing cholangitis is 'primary', occurring in association with his colitis, rather than secondary to the gallstones since there was no history to suggest attacks of cholangitis, bile duct strictures were already present at the time of cholecystectomy and no stones have been shown in the bile ducts at any time.
References
1 Chapman RWG, Arborgh BAM, Rhodes JM, et al. Primary sclerosing cholangitis - - A review of its clinical features, cholangiography and hepatic histology. Gut 1980; 21:870-877. 2 Elias E, Summerfield JA, Dick R and Sherlock S. Endoscopic retrograde cholangiopancreatography in the diagnosis of
D. CLEMENTS et al. The patient has maintained normal biochemical tests for the 3 years since presentation which shows that impressive radiological abnormalities due to sclerosing cholangitis may not necessarily be associated with a bad prognosis. Since the introduction of E R C P it has become recognized that severe cholangiographic abnormalities may be present in an asymptomatic patient [3]. This case shows that extensive intrahepatic and extrahepatic bile duct stricturing may be present without even biochemical evidence of cholestasis. This makes it likely that asymptomatic sclerosing cholangitis is c o m m o n e r than previously thought, and that patients who are shown to have extensive, severe changes at cholangiography may have a better prognosis than previously recognized.
jaundice associated with ulcerative colitits. Gastroenterology 1974; 67:907-911. 3 Chapman RWG, Burroughs AK, Bass NM and Sherlock S. Longstanding asymptomatic primary sclerosing cholangitis - - Report of three cases. Dig Dis Sci 1981; 26:778-782. 4 Barnes S, Gallo GA, Trash DB, et al. Diagnostic value of serum bile acid estimation in liver disease. J Clin Path 1975; 28:506-509.
72 HEP 00143
Severe Bile Duct Lesions Without Biochemical Evidence of Cholestasis in a Case of Sclerosing Cholangitis
D. C l e m e n t s , J . M . R h o d e s a n d E . Elias Department of Medicine, Queen Elizabeth Hospital, Edgbaston, Birmingham BI5 (U. K.) (Received 29 November, 1985) (Accepted 5 February, 1986)
Summary
We report a patient with very marked cholangiographic changes of sclerosing cholangitis with normal serum alkaline phosphatase and no other detectable biochemical evidence of cholestasis. He has remained asymptomatic and still has no biochemical evidence of cholestasis despite follow-up over 3 years. This case demonstrates that patients with sclerosing cholangitis may have normal liver function tests including serum alkaline phosphatase, that asymptomatic sclerosing cholangitis may therefore be under diagnosed, and that severe radiological changes of sclerosing cholangitis may not necessarily be associated with a poor prognosis.
Introduction
Primary sclerosing cholangitis is a disease of unknown aetiology characterised by inflammation and stricturing of the intra- and extrahepatic bile ducts. It is associated with ulcerative colitis in over 70% of cases [1]. In the past when the diagnosis was made almost exclusively in patients with cholestatic jaundice and/or cholangitis, the patients generally had a poor prognosis. More recently endoscopic retrograde cholangiography, a reliable diagnostic test for this condition [1,2], has shown that asymptomatic patients may have extensive bile duct lesions [3]. These have typi-
cally been patients with ulcerative colitis who have been investigated because of elevated serum alkaline phosphatase.
Case Report
A 56-year-old Caucasian man with a 3-year history of episodic abdominal pain underwent cholecystectomy in 1982. Operative cholangiography showed that there were two strictures in the common bile duct. The gallbladder contained several small stones. He made an uneventful post-operative recovery and bio-
Address for correspondence: Dr. D. Clements, Department of Medicine, Queen Elizabeth Hospital, Edgbaston, Birmingham B15 2TH, U.K. 0168-8728/86/$03.50© 1986Elsevier SciencePublishers B.V. (BiomedicalDivision)
BILE DUCT LESIONS IN SCLEROSING CHOLANGITIS
73
Fig. 1. Endoscopic retrograde cholangiogram showing marked stricturing and beading of intrahepatic and extrahepatic bile ducts diagnostic of sclerosing cholangitis.
chemical tests were normal: bilirubin 11/lmol/1 (normal 1-25), alkaline phosphatase 175 IU/I (normal 70-350), aspartate transaminase 17 IU/I (normal 5-35). It was noted that he had been diagnosed as having ulcerative colitis since the age of 16 but had no symptoms since the age of 20 and had been receiving no treatment for it. In 1983 he had intermittent epigastric pain so was referred for endoscopic retrograde cholangiopancreatography (ERCP). There had been no pruritus. Biochemical tests at this time were still normal (bilirubin 16 !Lmol/l, alkaline phosphatase 226 IU/1, aspartate transaminase 19 IU/I, fasting serum bile acids 6.3 g~mol/1 [normal <7.5 !lmol/l]). Haemoglobin, white cell and platelet estimations were all normal
and the ESR was 2 mm/h. ERCP showed florid stricturing and beading of intrahepatic and extrahepatic bile ducts diagnostic of sclerosing cholangitis (Fig. 1). The pancreatic duct was normal. Barium enema examination showed slight granularity of the mucosa of the rectum and sigmoid colon but was otherwise normal. At sigmoidoscopy the rectal mucosa appeared granular and mucosal biopsy showed mild chronic inflammatory changes. His epigastric pain subsequently resolved without treatment, and he has remained completely asymptomatic for 27 months since the ERCP examination and still has no biochemical evidence of cholestasis 3 years after cholecystectomy. He is currently prescribed sulphasalazine 2 g/day.
74 Discussion
This case shows remarkable disparity between the severe bile duct lesions demonstrated by E R C P and normal biochemical tests over prolonged follow-up. Not only were the serum alkaline phosphatase and bilirubin normal but also the fasting serum bile acid concentration which should be a sensitive screening test for cholestasis or hepatocellular dysfunction [4]. The duration of the sclerosing cholangitis in this patient is unknown. It seems probable that the sclerosing cholangitis is 'primary', occurring in association with his colitis, rather than secondary to the gallstones since there was no history to suggest attacks of cholangitis, bile duct strictures were already present at the time of cholecystectomy and no stones have been shown in the bile ducts at any time.
References
1 Chapman RWG, Arborgh BAM, Rhodes JM, et al. Primary sclerosing cholangitis - - A review of its clinical features, cholangiography and hepatic histology. Gut 1980; 21:870-877. 2 Elias E, Summerfield JA, Dick R and Sherlock S. Endoscopic retrograde cholangiopancreatography in the diagnosis of
D. CLEMENTS et al. The patient has maintained normal biochemical tests for the 3 years since presentation which shows that impressive radiological abnormalities due to sclerosing cholangitis may not necessarily be associated with a bad prognosis. Since the introduction of E R C P it has become recognized that severe cholangiographic abnormalities may be present in an asymptomatic patient [3]. This case shows that extensive intrahepatic and extrahepatic bile duct stricturing may be present without even biochemical evidence of cholestasis. This makes it likely that asymptomatic sclerosing cholangitis is c o m m o n e r than previously thought, and that patients who are shown to have extensive, severe changes at cholangiography may have a better prognosis than previously recognized.
jaundice associated with ulcerative colitits. Gastroenterology 1974; 67:907-911. 3 Chapman RWG, Burroughs AK, Bass NM and Sherlock S. Longstanding asymptomatic primary sclerosing cholangitis - - Report of three cases. Dig Dis Sci 1981; 26:778-782. 4 Barnes S, Gallo GA, Trash DB, et al. Diagnostic value of serum bile acid estimation in liver disease. J Clin Path 1975; 28:506-509.