Hypereosinophilia, Ulcerative Colitis, Sclerosing Cholangitis, and Bile Duct Carcinoma

A case of ulcerative colitis and pericholangitis-sclerosing cholangitis bile duct carcinoma complex associated with hypereosinophilia and possibly the hypereosinophilic syndrome is reported, and the association of eosinophilia with various hepatobiliary and gastrointestinal diseases is discussed. Hypereosinophilia may foretell a more serious underlying condition such as bile duct carcinoma in some patients with primary sclerosing cholangitis.     

Colorectal neoplasia in patients with ulcerative colitis and primary sclerosing cholangitis

PURPOSE: Most patients with primary sclerosing cholangitis also have ulcerative colitis. It has been suggested that in the presence of primary sclerosing cholangitis the risk of colorectal dysplasia and carcinoma is greater than in patients with ulcerative colitis alone. METHODS: In a retrospective study, we evaluated the possibility of colorectal cancer or dysplasia in 35 consecutive patients with primary sclerosing cholangitis and ulcerative colitis seen at The Johns Hopkins Hospital between 1979 and 1991. RESULTS: Thirteen of the 35 patients (37 percent) with ulcerative colitis and primary sclerosing cholangitis had colorectal neoplasia (5 with adenocarcinoma and 8 with dysplasia). In the 27 patients undergoing colonoscopic biopsy surveillance, the cumulative incidence at 28 years of colorectal cancer was 18.5 percent and for colorectal dysplasia it was 29.6 percent. The high incidence of colorectal cancer was less than the rate of colorectal cancer in patients with extensive colitis of childhood onset without primary sclerosing cholangitis (35 percent), but the rate of colorectal cancer and dysplasia (48.1 percent) is similar to the highest rates of cancer noted in the comparison group. Because patients had subtle, quiescent colitis, a short time from diagnosis of ulcerative colitis to diagnosis of colorectal neoplasia was noted (mean, 12.2±9 years; less than 8 years in 5/13 (38.5 percent) patients). CONCLUSION: Ulcerative colitis patients with primary sclerosing cholangitis appear to have a high frequency of colorectal cancer but a rate lower than expected in patients with extensive quiescent ulcerative colitis of childhood onset alone. However, exact conclusions are complicated by the high incidence of colorectal dysplasia found, which portends malignant transformation. Because of the subtle nature of colitis, the diagnosis of ulcerative colitis is often delayed, and surveillance programs should start as soon as ulcerative colitis is diagnosed.     

Does primary sclerosing cholangitis occurring in association with inflammatory bowel disease differ from that occurring in the absence of inflammatory bowel disease? A study of sixty-six subjects

Primary sclerosing cholangitis often occurs in association with inflammatory bowel disease, particularly ulcerative colitis but also Crohn's disease of the colon either with or without terminal ileal disease. Little data exist as to the effect of inflammatory bowel disease on the presenting symptoms, radiological features, response to liver transplantation, and potential risk of bile duct carcinoma in individuals with primary sclerosing cholangitis. In an effort to answer these questions, 66 patients with primary sclerosing cholangitis were studied. The definitive diagnosis of primary sclerosing cholangitis in each was accomplished using cholangiography, which in each case demonstrated characteristic beading, ectasia and stricturing of the intrahepatic and extrahepatic bile ducts. Inflammatory bowel disease was present in 47 (71.2%) patients. Thirty nine (59.1%) had ulcerative colitis; their mean age was 42.5 +/- 11.6 yr (mean +/- SD), and the male/female ratio was 2.9:1. In addition, eight patients (12.1%) had Crohn's colitis; their mean age was 40.5 +/- 6.5 yr, and the male/female ratio of this group was 1:1. Nineteen patients (28.8%) had primary sclerosing cholangitis without any inflammatory bowel disease; their mean age was 42.0 +/- 12.1 yr, and the male/female ratio in this group was 0.72:1. Seventy-two percent of the patients without inflammatory bowel disease had either jaundice, pruritus or fatigue at presentation compared with 41% of the patients with inflammatory bowel disease (p less than 0.05). In contrast, abnormal liver function tests were more common as the first manifestation of liver disease in the latter group (38% vs. 11%; p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)     

Cholangiographic abnormalities in ulcerative colitis associated pericholangitis which resemble sclerosing cholangitis

Cholangiographic abnormalities in asymptomatic patients with pericholangitis and longstanding ulcerative colitis, which resemble sclerosing cholangitis, have not been previously reported. Endoscopic retrograde cholangiography (ERC) performed in one such patient suggesting intrahepatic sclerosing cholongitis stimulated the study of seven additional patients with largely asymptomatic pericholangitis. In seven of these eight patients. ERC demonstrated abnormalities which resembled sclerosing cholangitis. These consisted of beading and structures mainly of the intrahepatic biliary tree (IHB). In two of the eight, the common bile duct was involved. In one, this was associated with histologic progression to cirrhosis and frank cholangitic episodes even though the initial clinical presentation and hepatic histology 2 1/2 years earlier suggested only pericholangitis. We therefore conclude that bile duct abnormalities resembling sclerosing cholangitis may be demonstrated cholangiographically in patients with ulcerative colitis who present with the typical picture of pericholangitis.     

Atrophy and Neoplastic Transformation of the Ileal Pouch Mucosa in Patients With Ulcerative Colitis and Primary Sclerosing Cholangitis

INTRODUCTION: Patients with ulcerative colitis and primary sclerosing cholangitis have an increased risk of developing carcinoma both in the bile ducts and in the colon. PURPOSE: To investigate whether this patient group also has an increased risk of developing atrophy and neoplasia in the ileal pouch mucosa after construction of a pelvic pouch with an ileoanal anastomosis or a continent Kock ileostomy. METHODS: Flexible video endoscopic examinations of the ileal pouch were performed in 16 patients with ulcerative colitis and primary sclerosing cholangitis and in 16 matched patients with ulcerative colitis without sclerosing cholangitis. Biopsies were sampled from different locations in the pouch for histologic assessment of mucosal atrophy and dysplasia and for flow cytometric DNA analysis assessing chromosomal aberrations. RESULTS: The patients with sclerosing cholangitis developed moderate or severe atrophy in the pouch significantly more often (P < 0.01). Persistent severe mucosal atrophy was revealed in eight patients with sclerosing cholangitis and only in two controls. One patient with sclerosing cholangitis had high-grade dysplasia in multiple locations. Low-grade dysplasia was assessed in three patients with sclerosing cholangitis and in two of the controls. DNA aneuploidy was displayed in three patients, all with sclerosing cholangitis and dysplasia. All patients with neoplastic transformation had a pouch with ileoanal anastomosis and a long pouch duration (> 8 years). CONCLUSION: Patients with ulcerative colitis and primary sclerosing cholangitis with an ileal reservoir are more prone to developing mucosal atrophy in the pouch and seem to have a higher risk of neoplastic transformation in the pouch mucosa than patients with ulcerative colitis without sclerosing cholangitis.     

Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology.

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.     

6 Investigation of the patient with abnormal liver function tests

About one-half of patients with ulcerative colitis develop abnormal liver function tests at some time during the course of the illness. This should prompt an investigation for primary sclerosing cholangitis and other common hepatobiliary diseases. Primary sclerosing cholangitis occurs in 2–10% of patients with ulcerative colitis. The diagnosis of primary sclerosing cholangitis is most often made by endoscopic retrograde cholangiography. Liver histopathology is often inconclusive but magnetic resonance cholangiography shows promise as a useful non-invasive diagnostic tool. Cholangiocarcinoma complicates 20–40% of patients with end-stage primary sclerosing cholangitis and is now one of the most common causes of death in patients with ulcerative colitis. Distinction between benign and malignant strictures can be difficult and is best done with a combination of clinical suspicion, repeated imaging for mass lesions, cholangiography, and endoscopic brushings and/or biopsies. Dominant lesions of the common bile duct or common hepatic duct produce progressive jaundice and liver damage. Early treatment may improve prognosis. Single strictures can be dilated endoscopically. If the stricture is more complicated and extends into the intrahepatic ducts or there is suspicion of cholangiocarcinoma, surgical resection may be more appropriate. Liver transplantation should be considered in end-stage disease.     

Antineutrophil antibody: a test for autoimmune primary sclerosing cholangitis in childhood?

The detection of an antineutrophil antibody which is highly sensitive and specific for adult primary sclerosing cholangitis using indirect immunoalkaline phosphatase has been previously described. In this study, the diagnostic potential of this method in childhood primary sclerosing cholangitis is described. A range of 72 blinded children's sera (36 boys), aged six months to 21 years (10 primary sclerosing cholangitis, eight autoimmune chronic active hepatitis, 10 alpha-1 antitrypsin deficiency, 12 extrahepatic bile duct atresia, 11 ulcerative colitis and 21 normal subjects) was assayed. Eight of the 10 primary sclerosing cholangitis patients were correctly identified. Three patients with chronic active hepatitis also showed the characteristic primary sclerosing cholangitis pattern of staining. No ulcerative colitis patients or any other patients showed this pattern of staining. All normal subjects were negative. As in adult primary sclerosing cholangitis, there is a specific antineutrophil antibody in childhood primary sclerosing cholangitis and this provides further evidence towards an autoimmune aetiology of this condition. The test may have diagnostic potential.     

Bile Duct Carcinoma in Chronic Ulcerative Colitis*

Summary: Four patients are described in whom a bile duct carcinoma co-existed with chronic ulcerative colitis. They were seen over a 14 year period at the Royal Melbourne Hospital. During this time 29 patients were diagnosed as having bile duct carcinoma, and 292 patients were admitted on one or more occasions with a diagnosis of ulcerative colitis; the observed frequency of ulcerative colitis in patients with bile duct cancer was 14%, while bile duct cancer occurred in 1.4% of inpatients with ulcerative colitis. We conclude that bile duct carcinoma is significantly, and presumably consequentially, associated with ulcerative colitis but the nature of the association is uncertain.     

Primary sclerosing cholangitis. A report of nine cases and clinical review.

Nine cases of primary sclerosing cholangitis were reviewed as to methods of diagnosis, association with other disease states and clinical course of the patients. There were four cases of primary sclerosing cholangitis occurring alone, four cases associated with inflammatory bowel disease (three with chronic ulcerative colitis and one case with proctosigmoiditis) and one case associated with porphyria cutanea tarda. All cases of primary sclerosing cholangitis occurring alone, progressed to secondary biliary cirrhosis, however, none of the cases associated with chronic ulcerative colitis progressed to secondary biliary cirrhosis. In all cases, the diagnosis was established by operative findings and biopsy results. The mode of clinical presentation was similar in all cases and was characterized by slowly progressive jaundice. Intravenous and oral cholangiography were not useful in establishing a diagnosis but endoscopic retrograde cholangiography offers preoperative diagnostic hope and use for follow-up evaluation. One case with ulcerative colitis had a Strongyloides infection and the organism was found in the fibrotic duct and pericholedochal lymph nodes. The etiological considerations are reviewed and the classification of sclerosing cholangitis associated with ulcerative colitis, as primary, is discussed. Therapeutic modalities are discussed, though therapy is mainly empirical at present.     

Cystic dilatation of intrahepatic bile ducts in primary sclerosing cholangitis

A case of primary sclerosing cholangitis associated with cystic dilatations of intrahepatic bile ducts simulating Caroli's disease is described. The diagnosis of primary sclerosing cholangitis was based upon cholangiogram features, liver histologic examination and the association with chronic ulcerative colitis. It may be suggested that the cystic dilatation of intrahepatic bile duct represents an extreme form of the usual mild dilatations (cholangiectases) described in primary sclerosing cholangitis. We suggest that cystic dilatation of intrahepatic bile ducts could be included among the radiologic features of this disease.     

The course of colonic disease in ulcerative colitis patients with primary sclerosing cholangitis.

BACKGROUND/AIMS: Although there are many studies reporting that colonic dysplasia and cancer develop more frequently in ulcerative colitis patients with ulcerative colitis with primary sclerosing cholangitis, there are insufficient data on the course of the colonic disease. In this study, the course of the colonic disease in ulcerative colitis patients with primary sclerosing cholangitis was investigated. METHODS: Data obtained from ten patients with total colitis and accompanying primary sclerosing cholangitis (three females, seven males, mean age: 44.5+/-10.0 years) were compared with data obtained from 64 patients with pancolitis but without primary sclerosing cholangitis (27 females, 37 males; mean age: 42.3+/-17.1 years). RESULTS: The follow-up period was 6.4+/-6.2 years in patients without primary sclerosing cholangitis, 12.7+/-6.2 years in total and 5.1+/-4.0 years (after development of the condition) in patients with primary sclerosing cholangitis (p<0.01). The number of disease attacks (3.7 attacks/yr vs. 0.5 attacks/yr), duration of the active disease (12.9+/-8.0 months vs. 0,3+/-1.0 months), the number of patients in whom corticosteroids were used (47 patients vs. one patient), the number of patients hospitalized (50 patients vs. one patient) and duration of hospitalization (1.2+/-0.8 months vs. 0,1+/-03 months) were higher in patients with than without primary sclerosing cholangitis (after development of the condition) (p<0.001). There was no significant difference in data obtained from patients with and without primary sclerosing cholangitis before development of the disease. CONCLUSIONS: Colonic disease subsides when primary sclerosing cholangitis develops. The higher frequency of colonic dysplasia and cancer seen in patients with primary sclerosing cholangitis can be explained by the fact that most of them have a longer duration of total colitis and fewer need total colectomy. Even though it does not seem to cause clinical problems, the colonic disease should not be ignored in these patients.     

Increased risk of colorectal neoplasia in patients with primary sclerosing cholangitis and ulcerative colitis: a meta-analysis

BACKGROUND: Published data on the risk of colorectal neoplasia in patients with ulcerative colitis with and without primary sclerosing cholangitis are conflicting. A meta-analysis was performed to synthesize available publications and to compare the risk of colorectal neoplasia in patients with ulcerative colitis with and without primary sclerosing cholangitis. METHODS: By using MEDLINE and manual search methods, studies were identified that compared the risk of colorectal neoplasia (dysplasia and carcinoma) in patients with ulcerative colitis with and without primary sclerosing cholangitis. In addition, citations were reviewed in relevant articles and proceedings from gastroenterology meetings, and investigators were contacted when data were incomplete. The summary odds ratio (OR) was then calculated for the risk for patients with ulcerative colitis and primary sclerosing cholangitis of having colorectal neoplasia develop compared with that of patients with ulcerative colitis without primary sclerosing cholangitis. RESULTS: Eleven studies met all eligibility criteria for the meta-analysis. Patients with ulcerative colitis and primary sclerosing cholangitis are at increased risk of colorectal dysplasia and carcinoma compared with patients with ulcerative colitis alone; OR 4.79: 95% CI [3.58, 6.41] with the Mantel-Haenszel method, and OR 5.11: 95% CI [3.15, 8.29] with the Der Simonian and Laird method. This increased risk is present even when the risk of colorectal carcinoma alone is considered; OR 4.09: 95% CI [2.89, 5.76] and OR 4.26: 95% CI [2.80, 6.48] by using, respectively, the Mantel-Haenszel and the Der Simonian and Laird methods. CONCLUSIONS: Patients with ulcerative colitis and primary sclerosing cholangitis have a significantly higher risk for the development of colorectal neoplasia than patients with ulcerative colitis but not primary sclerosing cholangitis. More intensive colonoscopic surveillance should be considered for patients with ulcerative colitis and primary sclerosing cholangitis.     

Primary sclerosing cholangitis and low-dose oral pulse methotrexate therapy. Clinical and histologic response

Two patients with sclerosing cholangitis responded to low-dose methotrexate treatment. A 40-year-old man who had previously undergone total colectomy for ulcerative colitis presented with refractory erythroderma and sclerosing cholangitis. Both disorders were alleviated and have remained in remission on methotrexate, 5 mg every 12 hours three times each week (15 mg/wk). Liver function improved, bile duct scarring did not worsen, and repeat liver biopsy samples have shown striking improvement. A 60-year-old man with long-standing ulcerative colitis and repeated exacerbations of sclerosing cholangitis had a similar response to low-dose methotrexate, 2.5 mg every 12 hours three times each week (7.5 mg/wk), during a 6-year period. Recurrent episodes of cholangitis have disappeared, liver function has become normal, bile duct scarring has not worsened, and liver histologic findings have become normal. Because of the potential hepatotoxicity of methotrexate, we suggest that a prospective, randomized trial be done.     

Bile duct antibodies crossreacting with blood group antigens in primary sclerosing cholangitis.

Indirect immunoperoxidase histochemistry was used to localise and determine the disease, species, and tissue specificity of bile duct antibodies in primary sclerosing cholangitis. Serum was collected from: 29 patients with primary sclerosing cholangitis, 18 patients with ulcerative colitis alone, 19 patients with extrahepatic biliary obstruction of other causes, and 42 healthy control subjects. Bile duct antibodies reacted with an antigen localised to the small and large intrahepatic bile ducts. When blood group A human liver was used they were detected in 34% of patients with primary sclerosing cholangitis. They were not detected when blood group O human liver was used. Bile duct antibodies that reacted with obstructed and normal rabbit liver were detected in 34% and 17% respectively of patients with primary sclerosing cholangitis but were also present in similar proportions of control subjects. Colon antibodies that reacted with human and rabbit colon were found in 52% and 24% respectively of patients with primary sclerosing cholangitis. Absorption studies using blood group substances A and B abolished the reactivity of bile duct antibodies with human and rabbit liver and that of colon antibodies' with rabbit colon. Colon antibodies that reacted with human colon were not absorbed. Absorption studies using isolated peripheral white blood cells did not affect reactivity of bile duct or colon antibodies. We conclude that bile duct antibodies are disease, species, and tissue non-specific and react with blood group A/B antigens present in human and rabbit bile ducts and rabbit colon. This suggests that they do not play a role in the pathogenesis of primary sclerosing cholangitis.     

Ulcerative colitis, primary sclerosing cholangitis, bile duct carcinoma, and generalized sarcoidosis. Report of a unique association

We describe a young man with a hitherto unreported association of chronic ulcerative colitis, primary sclerosing cholangitis (PSC), bile duct carcinoma, and generalized sarcoidosis with features of high-intensity alveolitis. This finding suggests that common immunological mechanisms may be involved in the pathogenesis of these diseases.     

Primary sclerosing cholangitis in Japan—analysis of 192 cases

Primary sclerosing cholangitis is very rare in Japan. The aim of the present study was to identify the characteristics of such patients in Japan. A questionnaire was sent to the members of the Japanese Society of Gastroenterology and responses for 192 cases were analyzed. There was male predominancy (61%), and two peaks in the age distribution at diagnosis (20–30 years and 50–70 years). Bile duct damage was mainly intra + extrahepatic (69%) versus intrahepatic (17%) or extrahepatic (14%). The incidences of eosinophilia and positivity for anti-nuclear antibody were 27% and 30%, respectively. The incidence of associated inflammatory bowel disease was 21 % (38 ulcerative colitis and 2 Crohn's disease). Chronic pancreatitis, gallstones, and biliary cancers occurred in 15%, 12%, and 4%, respectively, of the 192 patients. Patients less than 40 years of age had a higher incidence than the patients 40 years old or more of damage intra + extrahepatic bile ducts (89% vs 56%) and of associated ulcerative colitis (36% vs 9%), whereas the incidence of chronic pancreatitis was lower in patients aged less than 40 years (4%). The characteristics of patients with primary sclerosing cholangitis in Japan differ from those in other countries in regard to age distribution and the incidence of complications, and at least two different groups of patients seem to exist in terms of the degree of bile duct damage and the incidence of complications.     

Forty-six patients with primary sclerosing cholangitis: radiological bile duct changes in relationship to clinical course and concomitant inflammatory bowel disease

The clinical features of primary sclerosing cholangitis were studied in 46 consecutive patients. Jaundice was the most common symptom (57%), followed by pruritus (28%), pain (24%), and fever (15%). Thirty-three per cent of the patients had no symptoms, merely laboratory changes. No significant relationship was observed between a numerical score of radiological bile duct changes at diagnosis and the clinical picture, or the clinical course during follow-up. If clinical deterioration occurred, this seemed to happen within the first eight years after the clinical presentation. Patients with only intra-hepatic bile duct changes (n = 10) did not differ clinically from those with extrahepatic changes as well. Forty-three out of 44 patients examined had inflammatory bowel disease, usually ulcerative colitis, with total colitis in 84%. Radiological bile duct changes had a significantly higher score in patients who had to be treated with a combination of sulfasalazine and steroids, suggesting a weak relationship between severity of bowel disease and bile duct disease.     

Cytokine production from colonic T cells in patients with ulcerative colitis with and without primary sclerosing cholangitis

PURPOSE: Only five percent of all patients with ulcerative colitis develop primary sclerosing cholangitis. T cells accumulate at the sites of the colonic and bile duct inflammation in both ulcerative colitis and primary sclerosing cholangitis. T helper cell populations comprise functionally distinct subsets characterized by the cytokines they produce. Several alterations in cytokine production have been described in patients with ulcerative colitis. The aim of this study was to investigate possible differences in T helper subsets and cytokine production in peripheral blood and colonic mucosa among ulcerative colitis patients with and without primary sclerosing cholangitis. METHODS: Eleven patients with primary sclerosing cholangitis and extensive ulcerative colitis, 11 patients with extensive ulcerative colitis and no liver disease, and 5 patients without any history of liver disease who underwent routine colonoscopy because of previous polypectomy were included in the study. Colonoscopy with multiple biopsies was performed on all patients. Lamina propria mononuclear cells and peripheral blood mononuclear cells were isolated. A modified version of solid-phase enzyme-linked immunospot assay was used for the separate counting of cells producing interferon-, interleukin-2 (T helper 1), and interleukin-4 (T helper 2). RESULTS: No differences in spontaneous production of cytokines from peripheral blood mononuclear cells was found among the three groups. Patients with primary sclerosing cholangitis compared with patients with ulcerative colitis without liver disease showed a significant increase in the number of cells secreting interferon- after purified protein derivative stimulation (P<0.02). More cells secreting interferon- were found in the two ulcerative colitis groups than in the cell populations from healthy controls (P<0.03). The number of cells secreting interferon- in the primary sclerosing cholangitis group was significantly lower than in the ulcerative colitis group without liver disease (P<0.04). The number of cells secreting interleukin-4 was lower in the primary sclerosing cholangitis group than among the patients with ulcerative colitis only (P=0.05). CONCLUSION: Isolated lymphocytes from colonic mucosa differ in cytokine production in patients with ulcerative colitis with and without primary sclerosing cholangitis.This study was supported by grants from The Swedish Medical Research Council (7129), foundations of the Karolinska Institute, the Nanna Svartz foundation, the Swedish Society of Medicine, and the Ruth and Richard Juhlin foundation.     

Isolated granulomatous hepatitis-A histopathological surprise mimicking cholangiocarcinoma in ulcerative colitis

A 63-yr-old woman, known case of ulcerative colitis, was diagnosed with sclerosing cholangitis 2 years back. She was admitted for investigation of abdominal discomfort, fatigue with elevated alkaline phosphatase and deranged liver function test. Imaging studies (computerised tomography and magnetic resonance imaging) demonstrated a normal biliary tree with focal hepatic lesion which was showing features of cholangiocarcinoma. Ultrasound guided biopsy of the lesion surprisingly revealed non caseating granulomata. Granulomatous hepatitis occurs in less than 1 percent of cases of inflammatory bowel disease. A clinical diagnosis of isolated granulomatous hepatitis was established as the lesion remained stable on follow up and no other cause for it was identified on further investigation. Although the differential diagnosis of focal hepatic lesion in patients with ulcerative colitis with sclerosing cholangitis is wide, granulomatous hepatitis presenting as focal mass lesion mimicking cholangiocarcinoma has never been described previously.