Sclerosing Cholangitis and Biliary Tract Calculi -Primary Or Secondary? Gut: First Published As 10.1136/Gut.33.10.1376 on 1 October 1992
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1376 Gut 1992; 33: 1376-1380 Sclerosing cholangitis and biliary tract calculi -primary or secondary? Gut: first published as 10.1136/gut.33.10.1376 on 1 October 1992. Downloaded from C S Pokorny, G W McCaughan, N D Gallagher, W S Selby Abstract subsequently developed, intrahepatic and/or The clinical features of 61 patients with extrahepatic bile duct calculi. The features of sclerosing cholangitis were reviewed. This these patients were compared with those of group included 23 patients with biliary tract patients without calculi. The results indicate that calculi, commonly considered as excluding the both groups ofpatients can be considered to have diagnosis of primary scierosing cholangitis. primary sclerosing cholangitis. The aim of this study was to compare these 23 patients (group A) with 38 patients with sclerosing cholangitis free ofcalculi (group B). Methods Both groups had the following features in common: (i) age at presentation, (ii) incidence PATIENTS of inflammatory bowel disease, (iii) extent of Sixty one patients diagnosed as having sclerosing radiological disease, (iv) prevalence of HLA- cholangitis were seen between 1984 and 1991 at B8 and DR3 haplotype, (v) incidence of the Gastroenterology and Liver Centre of the cholangiocarcinoma, and (vi) progression to Royal Prince Alfred Hospital, Sydney. The hepatic transplantation (mean follow up 49.9 diagnosis was made in 57 patients by endoscopic months). All patients in group A were sympto- retrograde cholangiopancreatography and in matic at diagnosis compared with 23 of the 38 three by percutaneous transhepatic cholangio- patients (61%) in group B. Recurrent ascend- graphy. In one patient, the diagnosis of scleros- ing cholangitis occurred in 12 patients in group ing cholangitis became apparent only after A (52%) and two patients (5%) in group B. The examination of the native liver after hepatic similarity between the two groups was main- transplantation. All radiological examinations tained when the nine patients in group A who were reviewed by at least two gastroenterologists developed calculi after sclerosing cholangitis and two radiologists. The date of diagnosis was was diagnosed were excluded. It is concluded defined as the point when radiological evidence http://gut.bmj.com/ that choledocholithiasis is part ofthe spectrum of intrahepatic and/or extrahepatic duct of primary sclerosing cholangitis and that it is strictures were first demonstrated. not necessary to invoke choledocholithiasis as The patients were divided into two groups the initial lesion of the bile ducts in such based on whether or not biliary tract calculi were patients. present, either at diagnosis or at any stage during (Gut 1992; 33: 1376-1380) the course of their illness. Their clinical features were compared, as was the extent of disease, the on September 24, 2021 by guest. Protected copyright. clinical course, and prognosis. The mean period Primary sclerosing cholangitis is a chronic of follow up was 49-9 months (range 1-192 inflammatory disorder which results in fibrosis months). and obliteration of the intrahepatic and/or extra- HLA class I and class II alleles were serologic- hepatic bile ducts leading to the typical radio- ally typed at the Tissue Typing Laboratory, logical appearance of stricturing and beading of NSW Division, Australian Red Cross Society the biliary tree. The aetiology is unknown, Blood Transfusion Service using the standard although immunological factors have been National Institutes ofHealth test for class I and a implicated.' Between 50% and 75% of cases are modified National Institutes of Health test using associated with inflammatory bowel disease, immunomagnetic beads for class II. ` Anti- mostly ulcerative colitis. Ultimately biliary neutrophil cytoplasmic antibody was measured cirrhosis results and the median survival has in 31 patients by indirect immunofluorescence. been estimated to be 12 years.2 The diagnosis of The substrate used was an alcohol fixed neutro- primary sclerosing cholangitis is based on typical phil rich preparation with patient sera screened A W Morrow clinical, histological, and radiological features. at a dilution of 1:20." Gastroenterology and By conventional criteria, it is excluded by (a) the Comparisons between the two groups were Liver Centre, Royal Prince Alfred Hospital, presence of biliary tract calculi, (b) a history made using Fisher's exact test. Camperdown, NSW, of previous biliary tract surgery, and (c) the Australia presence of a cholangiocarcinoma.36 Despite C S Pokorny G W McCaughan these criteria, patients with biliary tract calculi Results N D Gallagher have been included in several studies of primary In 23 patients (group A) biliary tract calculi were W S Selby sclerosing cholangitis.7 We have also encount- present either at the time of diagnosis (14 Correspondence to: ered a number of patients with the radiological patients) (Fig 1) or subsequently developed some Dr C S Pokorny, A W Morrow Gastroenterology and Liver features of sclerosing cholangitis but in whom time after the diagnosis of sclerosing cholangitis Centre, Royal Prince Alfred there were also biliary tract calculi. The aim of had been made (nine patients). The calculi in Hospital, Camnperdown, NSW 2050, Australia. the present study was to review a group of these latter nine patients were found at intervals Accepted for publication patients in whom sclerosing cholangitis was varying from 18 to 156 months (mean 58.7 11 March 1992 present. Twenty three of these patients had, or months) after sclerosing cholangitis had been Sclerosing cholangitis and biliary tract calculi -primary or secondary? 1377 and 19 in group B). Of patients in group A, six (50%) had detectable antibody with a perinuclear pattern on immunofluorescent staining com- pared with three of 19 (16%) in group B. All Gut: first published as 10.1136/gut.33.10.1376 on 1 October 1992. Downloaded from patients in group A were symptomatic at initial presentation (11 patients had right upper quadrant pain, eight had cholangitis, two had pruritus, and two had symptoms related to hepatic decompensation). This was the case whether or not calculi were present at the time. In contrast, only 23 of the 38 patients in group B (61%) had clinical manifestations of their disease (cholangitis in eight patients, right upper quad- rant pain in six patients, pruritus in three patients, and evidence of hepatic decompensa- tion in six patients; p=0 0002). The remainder were asymptomatic and were being investigated for abnormal liver function tests. The radiological extent ofbile duct disease was similar in the two groups (Table II). Of the patients in group A, the biliary calculi were present in the intrahepatic ducts in six patients, in both intrahepatic and extrahepatic ducts in a further six and in the extrahepatic ducts alone in 11 patients. In only three patients were single stones found. The remaining 20 had multiple biliary calculi of varying size. Thirteen patients in group A (57%) and seven in group B (18%) had associated cholecystolithiasis, diagnosed either before or at the time of diagnosis of sclerosing cholangitis (p=0002). This excess was largely Figure 1: Endoscopic accounted for by women (10/13 in group A v two retrograde of seven in group B). cholangiopancreatography diagnosed (Fig 2). In each case, the calculi were Twelve patients (52%) in group A had recur- performed on a patient with ulcerative colitis who found on repeat endoscopic retrograde cholan- rent episodes of ascending cholangitis during presented with right upper giopancreatography, performed because of follow up compared with two patients in group B http://gut.bmj.com/ quadrant pain, fever, and recurrent ascending cholangitis (six patients) or (5%). This difference was highly significant (p= jaundice. Beading and stricturing ofthe intrahepatic progressive liver failure (three patients). Five of 000005). and extrahepatic bile ducts these nine patients had undergone cholecystec- Nineteen patients in group A underwent are evident with numerous tomy from three months to 30 years previously, various therapeutic manoeuvres in an attempt to calculi being present in the intrahepatic ducts making passage of a stone from the gall bladder clear the biliary tree of calculi. Endoscopic (arrowed). an unlikely cause of the biliary tract calculi. sphincterotomy was performed in 16 patients Thirty eight patients (group B) did not have with successful removal of all calculi in seven. In on September 24, 2021 by guest. Protected copyright. biliary calculi either on diagnosis or during the a further five, percutaneous removal of residual course of their illness. Biochemical analysis of intrahepatic stones was possible. Five ofthese 12 calculi was not performed. patients had endoscopic insertion of a biliary The clinical features of the two groups as well stent. A further five underwent percutaneous as the subgroups in group A - that is, those who stent insertion in order to achieve biliary decom- had calculi at the time sclerosing cholangitis was pression. Despite these measures, biliary tract diagnosed and those who developed calculi after calculi recurred in all 12 patients after removal. sclerosing cholangitis was diagnosed, are shown Nine patients in group A (39%) and eight in Table I. Because of the similarities between patients in group B (21%) underwent hepatic the two subgroups in group A, they are hereafter transplantation because of progressive disease referred to as one group. There were proportion- (p=0 07; NS). Uncontrollable intrahepatic ally more women in the group with calculi than sepsis, the result of recurrent calculi, compli- in the group without (16 of 23 in group A, 17 of cated the course ofthree patients in group A, and 38 in group B; p=004). The mean age at was the indication for hepatic transplantation. diagnosis was similar in both groups. No differ- Cholangiocarcinoma occurred in three patients ence was found in the frequency of associated in group A (13%) and five patients in group B inflammatory bowel disease: in group A, 12 (13%). patients of the 23 (52%) had ulcerative colitis, Six deaths occurred in group A (26%) and 10 while in group B, 21 patients (55%) had ulcera- in group B (26%) during the follow up period.