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PubMed2003

Surgical management of ulcerative colitis in the presence of primary sclerosing cholangitis.

INTRODUCTION: The surgical management of ulcerative colitis in the patient with primary sclerosing cholangitis is controversial. METHODS: This study was designed as a retrospective chart review of all patients with primary sclerosing cholangitis who were surgically treated for ulcerative colitis. RESULTS: Sixteen patients with primary sclerosing cholangitis and ulcerative colitis were identified. The indication for ulcerative colitis surgery was dysplasia in 7 patients (44 percent), cancer in 2 (13 percent), intractability in 4 (25 percent), and unknown in 1. Final colon pathology demonstrated cancer in three patients and dysplasia in four. Two patients had biliary cancer discovered at the time of orthotopic liver transplantation. Thirteen patients were known to have primary sclerosing cholangitis when they underwent surgery for ulcerative colitis; two patients with severe primary sclerosing cholangitis underwent simultaneous orthotopic liver transplantation/total abdominal colectomy and did well with subsequent ileal pouch reconstruction. Two patients had orthotopic liver transplantation first and then ileal pouch-anal anastomosis (1 patient) or total abdominal colectomy (1 patient) and did well. Seven patients had well-controlled primary sclerosing cholangitis on medication and underwent ileal pouch-anal anastomosis or total abdominal proctocolectomy without significant hepatic compromise. One patient with moderate primary sclerosing cholangitis underwent ileorectal anastomosis and had severe liver failure postoperatively but survived. Another patient with worsening primary sclerosing cholangitis after total abdominal colectomy has since developed persistent bleeding from peristomal varices. CONCLUSIONS: The overall cancer/premalignant lesion rate was high (50 percent in this study) in patients with primary sclerosing cholangitis and ulcerative colitis. Complications associated with the surgical management of ulcerative colitis are largely dictated by the degree of liver disease present at the time of surgery. Patients with significant primary sclerosing cholangitis that requires colectomy can undergo simultaneous orthotopic liver transplantation/total abdominal colectomy and then be candidates for subsequent ileal pouch-anal anastomosis reconstruction once liver function has improved. Patients with well-controlled primary sclerosing cholangitis can undergo ileal pouch-anal anastomosis surgery safely.

Adult↗

PubMed1987

Diagnosis of sclerosing cholangitis with technetium 99m-labeled iminodiacetic acid planar and single photon emission computed tomographic scintigraphy.

The purpose of this study was to determine whether 99mTc-iminodiacetic acid planar biliary scintigraphy combined with single photon emission computed tomography could detect sclerosing cholangitis and provide additional information regarding the extent and severity of disease. Thirteen patients with sclerosing cholangitis and 13 normal control subjects were studied. Scintigraphic results were also compared with previously reported studies of patients with isolated common bile duct obstruction and with primary biliary cirrhosis. The planar scintigraphy in patients with sclerosing cholangitis showed beading or bandlike constrictions of the biliary tract corresponding to lesions seen on cholangiography, and the image pattern was distinctly different from images obtained from patients with isolated common bile duct obstruction or primary biliary cirrhosis. The single photon emission computed tomography images of the liver in patients with sclerosing cholangitis demonstrated multiple focal areas of 99mTc-iminodiacetic acid retention, representing bile stasis in intrahepatic bile ducts. Compared to controls, the mean hepatic clearance half-time of 99mTc-iminodiacetic acid was markedly delayed in patients with sclerosing cholangitis (6-10 times normal). Individual patients with sclerosing cholangitis had wider variation in isotope clearance half-time from three regions of the liver than patients with isolated common bile duct obstruction, consistent with regional difference in disease severity and variable impairment of bile flow. In 4 patients with sclerosing cholangitis with incomplete filling of the right and left hepatic ducts at cholangiography, planar and single photon emission computed tomographic scintigraphy provided evidence of significant intrahepatic sclerosing cholangitis. In conclusion, combined 99mTc-iminodiacetic acid planar and single photon emission computed tomographic scintigraphy is a sensitive noninvasive test for the diagnosis of sclerosing cholangitis and reliably differentiates sclerosing cholangitis from isolated common bile duct obstruction or primary biliary cirrhosis. Measurement of isotope clearance half-time provides quantitative physiologic data that may be useful in the longitudinal follow-up of patients with sclerosing cholangitis.

Adult↗

PubMed2003

Atrophy and neoplastic transformation of the ileal pouch mucosa in patients with ulcerative colitis and primary sclerosing cholangitis: a case control study.

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.

Adult↗

PubMed1992

Ischemic cholangitis in hepatic allografts.

In this report, our objectives are to introduce the term "ischemic cholangitis" as an etiologic designation and to describe its manifestations. Herein we use the label "ischemic cholangitis" as a collective term for ischemic bile duct necrosis, cholangitis caused by ischemia but without necrosis, and biliary fibrosis as a manifestation of ischemic damage. The condition was observed in 12 allografts, either at the time of retransplantation (9 cases) or at autopsy (3 cases). Ischemic cholangitis involved primarily perihilar extrahepatic and intrahepatic bile ducts. The findings included duct necroses (eight cases), strictures (four cases), and cholangiectases (four cases); some of these features coexisted. In addition, complicating ascending cholangitis and cholangitic abscesses were noted in three cases. Ischemic cholangitis was caused by hepatic artery thrombosis (in nine patients) or stenosis (in one) or by occlusion of parabiliary arteries by fibrointimal proliferations, probably attributable to old thromboses (in two, in conjunction with associated foam cell arteriopathy in one). Biopsy specimens before retransplantation or autopsy were obtained in 11 patients, only 1 of whom had an infarct as direct evidence of ischemia. Nine patients had evidence of biliary obstruction or bile flow impairment; in two cases, specimens were normal or nondiagnostic relative to cholangitis. Features of cellular rejection associated with the manifestations of bile flow impairment and ischemia were noted in five cases. Thus, biopsy features that suggest biliary obstruction, with or without cellular rejection, may be a manifestation of ischemic cholangitis. We conclude that ischemic cholangitis is an important cause of cholestatic graft failure but that this type of cholangitis is difficult to diagnose because of its misleading biopsy manifestations.

Adult↗

PubMed2003

Cholangitis and liver abscess after percutaneous ablation therapy for liver tumors: incidence and risk factors.

PURPOSE: To determine the risk factors of cholangitis and liver abscess occurring after percutaneous ablation therapy for liver tumors. MATERIALS AND METHODS: Between October 1995 and September 2002, 358 patients with 455 liver tumors underwent a total of 683 ablation procedures, such as percutaneous ethanol injection (PEI), percutaneous microwave coagulation (PMC), and radiofrequency (RF) ablation therapy. With a retrospective review of medical records, the rates and outcomes of cholangitis and/or liver abscess occurring after ablation therapy were evaluated. The relationship between cholangitis and/or liver abscess and multiple variables (age, disease, Child-Pugh class, size of nodules, multiplicity of nodules, history of transcatheter arterial embolization, presence of bilioenteric anastomosis, and lack of prophylactic antibiotics administration) were statistically analyzed. RESULTS: Cholangitis and/or liver abscess occurred in 10 sessions (1.5%) in 10 patients: six sessions after PEI, three sessions after PMC, and one session after RF ablation. Both cholangitis and liver abscess were noted in seven sessions, cholangitis was noted in two, and liver abscess was noted in one. Six patients recovered, but two developed recurrent cholangitis and liver abscess, one developed lung abscess complicated with liver abscess, and one died of septic shock associated with cholangitis. On stepwise regression analysis, bilioenteric anastomosis was the sole significant predictor of cholangitis and/or liver abscess formation (P <.001; odds ratio = 36.4; 95% CI = 9.67-136.9). CONCLUSION: Bilioenteric anastomosis strongly correlated with the development of cholangitis and/or liver abscess after percutaneous ablation therapy. Close posttreatment attention should be paid to this subgroup of patients.

Adolescent↗

PubMed2003

Dynamic CT of acute cholangitis: early inhomogeneous enhancement of the liver.

OBJECTIVE: Our objective was to describe the dynamic CT findings of acute cholangitis, especially early inhomogeneous enhancement of hepatic parenchyma. MATERIALS AND METHODS: Inhomogeneous enhancement of hepatic parenchyma was retrospectively evaluated on dynamic CT in 406 consecutive patients without irregular fatty liver or multiple hepatic tumors. Dynamic CT scans were obtained 30 sec (early phase) and 90 sec (late phase) after starting the contrast material injection. Thirteen patients were diagnosed as having acute cholangitis (cholangitis group), and the remaining 393 patients were classified as the control group. The frequency of inhomogeneous enhancement was compared between these two groups. In nine of the 13 patients in the cholangitis group, we also evaluated changes in inhomogeneous enhancement on follow-up dynamic CT scans obtained after the patients had undergone treatment for acute cholangitis. RESULTS: In the cholangitis group, 11 (85%) of 13 patients showed nodular, patchy, wedge-shaped, or geographic inhomogeneous enhancement throughout the liver in the early phase on dynamic CT. In the control group, 19 (5%) of 393 patients also showed inhomogeneous enhancement in the early phase on dynamic CT. The frequency of inhomogeneous enhancement was significantly higher in the cholangitis group than in the control group (p < 0.001). Follow-up dynamic CT performed after treatment for acute cholangitis showed decreased inhomogeneous enhancement or no inhomogeneous enhancement in seven (78%) of nine patients in the cholangitis group. CONCLUSION: Inhomogeneous enhancement in the early phase on dynamic CT is frequently seen in patients with acute cholangitis; this finding usually disappears after treatment.

Acute Disease↗

PubMedDate not supplied

Surgery for segmental primary sclerosing cholangitis.

Primary sclerosing cholangitis is an uncommon chronic hepatobiliary disorder, and the definitive surgical treatment for symptomatic primary sclerosing cholangitis is liver transplantation. Although some cases with primary sclerosing cholangitis treated with hepaticojejunostomy or partial hepatectomy have been reported, the indications for these procedures and their long-term results have not been well defined or studied. We present three patients with segmental primary sclerosing cholangitis, and discuss the indication of surgical treatment for primary sclerosing cholangitis excluding liver transplantation. Three patients with segmental primary sclerosing cholangitis of the common bile duct and the hepatic ducts were treated by hepaticojejunostomy with partial resection of the common bile duct. We investigated clinical features such as angiography, cholangiography, and surgical treatment outcomes. It was difficult to differentiate primary sclerosing cholangitis from a cholangioma preoperatively, despite the use of cytology, angiography, and cholangiography. Two of the three patients were preoperatively suspected to have cholangioma. Segmental primary sclerosing cholangitis was diagnosed operatively. All patients were treated hepaticojejunostomy and achieved long-term survival. Although liver transplantation is the treatment of choice for primary sclerosing cholangitis, in cases of segmental primary sclerosing cholangitis, local resection of the involved structures may be curative. Resection of a discrete lesion may provide many years of survival with a good quality of life, in some cases obviating the need for liver transplantation.

Aged↗

PubMed2002

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.

Journal Article↗

PubMedDate not supplied

Radiologic manifestations of sclerosing cholangitis with emphasis on MR cholangiopancreatography.

Magnetic resonance cholangiopancreatography (MRCP) is a relatively new, noninvasive cholangiographic technique that is comparable with invasive endoscopic retrograde cholangiopancreatography (ERCP) in the detection and characterization of extrahepatic bile duct abnormalities. The role of MRCP in evaluation of the intrahepatic bile ducts, especially in patients with primary or secondary sclerosing cholangitis, is under investigation. The key cholangiographic features of primary sclerosing cholangitis are randomly distributed annular strictures out of proportion to upstream dilatation. As the fibrosing process worsens, strictures increase and the ducts become obliterated, and the peripheral ducts cannot be visualized to the periphery of the liver at ERCP. In addition, the acute angles formed with the central ducts become more obtuse. With further progression, strictures of the central ducts prevent peripheral ductal opacification at ERCP. Cholangiocarcinoma occurs in 10%-15% of patients with primary sclerosing cholangitis; cholangiographic features that suggest cholangiocarcinoma include irregular high-grade ductal narrowing with shouldered margins, rapid progression of strictures, marked ductal dilatation proximal to strictures, and polypoid lesions. Secondary sclerosing and nonsclerosing processes can mimic primary sclerosing cholangitis at cholangiography. These processes include ascending cholangitis, oriental cholangiohepatitis, acquired immunodeficiency syndrome-related cholangitis, chemotherapy-induced cholangitis, ischemic cholangitis after liver transplantation, eosinophilic cholangitis, and metastases.

Bile Duct Neoplasms↗

PubMed2001

Cholangitis associated with cystic dilatation of the intrahepatic bile ducts after antireflux valve construction in biliary atresia.

An intussusception-type antireflux valve (ARV) has been introduced to prevent postoperative ascending cholangitis in the management of biliary atresia (BA). We investigated the characteristics of cholangitis in the management of BA using the ARV in 38 patients who had undergone an operation at our institution; 29 underwent ARV construction at the same time as portenterostomy (PEO) or hepaticojejunostomy. One patient underwent ARV construction for refractory cholangitis with cystic dilatation of the intrahepatic bile ducts (CDIB) long after the PEO. Five of 29 patients who had ARV construction developed CDIB complicated by severe, refractory cholangitis. One or two episodes of mild cholangitis were observed in 5 (20.8%) of 24 patients who did not show CDIB. An ARV created for postoperative recurrent cholangitis associated with CDIB was ineffective. Preoperative cholangitis associated with a type I choledochal cyst and CDIB was observed in 1 patient. In conclusion, the ARV was effective in preventing refractory cholangitis without CDIB, but ineffective in preventing cholangitis with CDIB. Our findings suggest that CDIB resulting from the ongoing process of BA could be a potential target of bacterial infection through other routes than bilioenteric reflux.

Bile Ducts, Intrahepatic↗

PubMed2001

Bacterial cholangitis in patients with biliary atresia: impact on short-term outcome.

Bacterial cholangitis (BC) is a common complication in patients with biliary atresia (BA) and is characterized by fever, acholic stools and positive blood cultures. The diagnosis is often empirical because the yield of blood cultures is low. It is difficult to differentiate BC from other febrile episodes. In order to characterize the clinical and laboratory features of BC in patients with BA, identify risk factors, and correlate cholangitis with outcome, 37 patients with BA from 1993 to 1998 who underwent a Kasai operation in our hospital were studied. The follow-up period ranged from 6 to 59 months. A total of 107 febrile episodes were documented in these patients. The diagnostic criteria for cholangitis were fever, increased jaundice, or acholic stools. The clinical features, laboratory data, results of bacterial cultures, and outcomes were analyzed retrospectively. A total of 107 febrile episodes, including 78 bouts of cholangitis and 29 non-cholangitis infections, were found in 34 patients. Patients with BC had higher postoperative bilirubin levels (P = 0.02) and less frequent use of prophylactic antibiotics (P = 0.05) than those with non-cholangitis infections. Abnormal white blood cell counts (> 12,000 or <4,000 mm3) tended to be present in patients with BC (P = 0.08). There were no statistical differences in the risk factors and laboratory data between culture-positive (n = 16) and -negative (n = 62) cholangitis cases. The occurrence of cholangitis significantly reduced survival in both patients with good (P = 0.03) and inadequate bile flow (P = 0.03). All 9 patients who had never had cholangitis survived during the follow-up period. Repeated attacks of BC further decreased survival probability. The responsive organisms were mainly enteric bacteria, including Escherichia coli, Enterobacter cloacae, Klebsiella pneumoniae, Pseudomonas aeruginosa, Acinetobacter baumanni, and Salmonella typhi. The sensitivity tests justified empirical therapy with ceftriaxone. The effectiveness of prophylactic trimethoprim-sulfamethoxazole or neomycin warrants further studies. BC was a highly prevalent postoperative complication in patients with BA, especially those with inadequate bile drainage. It significantly affected early mortality. Aggressive and complete treatment with empirical ceftriaxone was appropriate.

Bacterial Infections↗

PubMed2004

Imaging of primary sclerosing cholangitis: preliminary results by two new non-invasive techniques.

BACKGROUND: Magnetic resonance cholangiography is a new technique which has already gained a role in primary sclerosing cholangitis. Computerised tomographic cholangiography is another non-invasive technique which has been used in assessing abnormal biliary tree, but has never been applied to evaluating primary sclerosing cholangitis. AIMS: To evaluate the ability of both magnetic resonance cholangiography and computerised tomographic cholangiography to detect bile duct changes in primary sclerosing cholangitis. PATIENTS AND METHODS: Magnetic resonance cholangiography and computerised tomographic cholangiography were performed in 16 primary sclerosing cholangitis patients. The computerised tomographic cholangiography data set was transferred to a processing workstation to obtain tridimensional reconstructions. Magnetic resonance cholangiography and computerised tomographic cholangiography images were analysed blind by two radiologists to assess: primary sclerosing cholangitis involvement, quality of imaging and the radiologist's certainty in determining the presence and location of the disease. RESULTS: Mean imaging quality was significantly better with computerised tomographic cholangiography compared with magnetic resonance cholangiography. Primary sclerosing cholangitis was identified in 15 cases with computerised tomographic cholangiography and 10 with magnetic resonance cholangiography (P < 0.05). Sensitivity in diagnosing primary sclerosing cholangitis was 94% with computerised tomographic cholangiography versus 63% with magnetic resonance cholangiography. Intrahepatic location was found in 14 cases, definitely present in 10 cases with computerised tomographic cholangiography and five with magnetic resonance cholangiography. Extrahepatic location was found in 13 cases, definitely present in 11 cases with computerised tomographic cholangiography and four with magnetic resonance cholangiography (P < 0.05). Computerised tomographic cholangiography also offered dynamic information about biliary excretion. CONCLUSIONS: Computerised tomographic cholangiography enables more accurate detection and location of primary sclerosing cholangitis than magnetic resonance cholangiography. Since computerised tomographic cholangiography offers additional information about biliary excretion, it may be proposed as an integrative technique in the diagnosis and follow-up of patients with primary sclerosing cholangitis.

Adult↗

PubMed2002

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.

Cholangitis, Sclerosing↗

PubMed2002

Role of cholecystectomy in preventing recurrent cholangitis.

BACKGROUND: Whether cholecystectomy should be performed after an episode of acute cholangitis is still unresolved. The purpose of this study was to analyze the role of elective cholecystectomy in preventing recurrent acute cholangitis in Asian patients. METHODS: Two hundred ten consecutive Asian patients with acute cholangitis caused by choledocholithiasis with coexisting cholelithiasis were studied prospectively. RESULTS: Forty-one patients (19.5%, Group 1) agreed to elective cholecystectomy whereas 169 patients (80.5%, Group 2) did not. Mean (+/- SEM) follow-up for Groups 1 and 2 were, respectively, 110.2 +/- 6.6 and 96.8 +/- 2.9 months. Endoscopic papillotomy was performed in 120 patients, 22 (53.7%) in Group 1 and 98 (58%) in Group 2. Recurrent acute cholangitis developed in 31 patients (14.8%), 9 in Group 1 and 22 in Group 2. There was no significant difference in the Kaplan-Meier estimates of the cumulative probability of occurrence of recurrent acute cholangitis between the 2 groups (p = 0.90). Recurrent acute cholangitis developed in 10 patients (8.3%) who underwent endoscopic papillotomy and in 21 (23.3%) patients who did not. There was a significant difference in the Kaplan-Meier estimates of the cumulative probability of occurrence of recurrent acute cholangitis between the patients with endoscopic papillotomy versus those without endoscopic papillotomy (p = 0.001). CONCLUSION: Cholecystectomy did not prevent recurrent acute cholangitis in Asian patients. In these patients, early endoscopic papillotomy lowered the frequency of recurrent acute cholangitis.

Acute Disease↗

PubMed1998

Chronic inflammatory changes in the pouch mucosa are associated with cholangitis found on peroperative liver biopsy specimens at restorative proctocolectomy for ulcerative colitis.

BACKGROUND: The clinical syndrome of primary sclerosing cholangitis (PSC), diagnosed in about 5% of patients with ulcerative colitis (UC), has been shown to be associated with pouchitis after ileal pouch-anal anastomosis. The aim of this study was to ascertain whether UC patients with cholangitis on liver biopsy at proctocolectomy, with or without the clinical syndrome of PSC, have an increased risk of inflammatory changes in the ileal reservoir mucosa and clinical pouchitis. METHODS: Of the consecutive 81 UC patients treated with restorative proctocolectomy with ileal J reservoir at Tampere University Hospital between 1985 and 1991, 73 with peroperative liver biopsy were included. A peroperative liver biopsy was obtained during proctocolectomy. After a median follow up of 64 months, pouch biopsy specimens were obtained. Periods of clinical pouchitis were diagnosed by means of clinical criteria alone or by clinical criteria combined with the results of previous pouch endoscopies in all patients. RESULTS: Ten patients (14%) showed histologic features consistent with small-duct PSC on liver biopsy. Endoscopic retrograde cholangiography had previously been performed on four of these patients, and all four had large-duct PSC. Patients with cholangitis had significantly more severe chronic, but not acute, inflammation in the pouch mucosa than patients without cholangitis. At least one episode of pouchitis occurred in 30% of the patients without cholangitis as compared with 90% of the patients with cholangitis. Chronic pouchitis was more frequent in the group with cholangitis than in the group without it (70% versus 11%). CONCLUSIONS: The only means of detecting all UC patients with cholangitis is a liver biopsy. Cholangitis, either with the clinical syndrome of PSC or found on liver biopsy, seems to be a risk factor for chronic-type inflammatory changes in the pouch mucosa and for the development of pouchitis.

Adult↗

PubMed1995

Diagnosis and prognosis of AIDS-related cholangitis.

OBJECTIVE: To determine more precisely the clinical and biological characteristics of AIDS-related cholangitis, and to investigate prognostic variables of this disease. DESIGN: Retrospective clinical and prognostic study. SETTING: Biliary unit, Bicêtre Hospital, France. PATIENTS: HIV-positive patients (n = 52) referred to the unit between December 1986 and June 1993 for biliary symptoms leading to the suspicion of AIDS-related cholangitis, (42 men; 10 women; mean age, 37 +/- 8 years). INTERVENTION: Endoscopic retrograde cholangiopancreatography (ERCP) was performed in order to determine the cause of the biliary symptoms. MAIN OUTCOME MEASURE: Clinical features and evolution of the cholangitis. RESULTS: Among the 52 patients, 45 met the ERCP criteria of AIDS-related cholangitis (36 men; nine women). The diagnosis of cholangitis was strongly suggested by abdominal ultrasonography in 47% of the cases. ERCP showed papillary stenosis, diffuse cholangitis, extrahepatic cholangitis alone, and intrahepatic cholangitis alone in 60, 67, 7 and 27%, respectively. Endoscopic sphincterotomy was performed in 28 patients. Pain was relieved by sphincterotomy in nine patients, but the other clinical or biological features were not influenced. One-year and 2-year survival rates were 41 +/- 7% and 8 +/- 4%, respectively. Multidimensional analysis using a Cox model showed that a lymphocyte count > 500 x 10(6)/l was the only independent predictive factor of better survival. CONCLUSION: AIDS-related cholangitis is a disease which leads preferentially to papillary stenosis or diffuse abnormalities of the biliary tract. Prognostic factors depend on the stage of the HIV infection. Another diagnosis of cholestasis was found in approximately 15% of the patients who showed biliary symptoms.

Acquired Immunodeficiency Syndrome↗

PubMed1986

Serum autoantibodies, ulcerative colitis and primary sclerosing cholangitis.

The aetiology of primary sclerosing cholangitis is unknown, but it is closely associated with ulcerative colitis. Serum anticolon antibodies, crossreacting with portal tracts, have been reported in patients with ulcerative colitis but no studies have been carried out in primary sclerosing cholangitis. The frequency of serum anticolon antibodies and portal tract antibodies have been measured in 24 patients with primary sclerosing cholangitis and ulcerative colitis; 15 patients with primary sclerosing cholangitis without ulcerative colitis; 77 patients without primary sclerosing cholangitis: 25 patients with Crohn's colitis; 10 patients with primary biliary cirrhosis; 22 patients with extrahepatic biliary obstruction and 20 normal controls. Serum anticolon and portal tract antibodies were detected using immunoperoxidase techniques on normal colon and obstructed human liver. Tissue typing was undertaken using a standard microcytotoxicity technique. The frequency of anticolon antibodies was markedly increased in primary sclerosing cholangitis patients with ulcerative colitis (62.5%) compared with patients with ulcerative colitis (17%) and Crohn's colitis (16%) (chi 2 = 17.9; p less than 0.001). The antibodies were almost entirely of IgG and IgA classes in all groups. Anticolon antibodies were not found in sera from any other group. Sera from eight of 15 patients with primary sclerosing cholangitis, ulcerative colitis and anticolon antibody reacted with portal tracts of human obstructed liver. This reaction was also seen in four of nine patients with ulcerative colitis and primary sclerosing cholangitis and in three of 15 patients with primary sclerosing cholangitis alone. Portal tract antibody was of IgG class and was not present in sera from any other groups. Unlike anticolon antibody, there was a close relationship between HLA-B8 phenotype and the portal tract antibody (p<0.02; chi 2 = 6.04). Absorption studies confirmed that the anticolon antibody is distinct from portal tract antibody.

Autoantibodies↗

PubMed1991

Significance and specificity of antibodies to neutrophils detected by western blotting for the serological diagnosis of primary sclerosing cholangitis.

Antibodies against neutrophils have been detected in sera from patients with primary sclerosing cholangitis and inflammatory bowel diseases either by immunofluorescence or by enzyme-linked immunosorbent assay. To assess primary sclerosing cholangitis-specific antibodies, we examined sera from 30 patients with clinically and morphologically well-established primary sclerosing cholangitis by Western blotting against neutrophils and compared these results with those obtained by testing sera from patients with inflammatory bowel diseases. By Western blot using sonified neutrophils, 24 (80%) of 30 primary sclerosing cholangitis sera were positive. Five antigenic determinants at 95, 60, 55, 40 and 30 kD were visualized. Twenty-eight of the primary sclerosing cholangitis sera also showed the characteristic perinuclear fluorescence pattern by immunofluorescence on neutrophils. Thus a serological diagnosis of primary sclerosing cholangitis could be made in 80% of patients based on these two methods. In contrast, only 9% of 23 patients with ulcerative colitis and 10% of 60 patients with Crohn's disease were positive by Western blot, and these patients also showed positive perinuclear fluorescence pattern by immunofluorescence, suggesting an overlap between inflammatory bowel diseases and primary sclerosing cholangitis. Although some patients with classical primary biliary cirrhosis and autoimmune chronic active hepatitis had antibodies against primary sclerosing cholangitis epitopes, none of the patients with obstructive bile duct disorders, collagen diseases, Wegener's granulomatosis or other hepatic and nonhepatic disorders were positive by Western blot, indicating the specificity of these five primary sclerosing cholangitis-related neutrophilic epitopes.

Adolescent↗