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PubMed2000

Overlap syndrome between autoimmune hepatitis and primary sclerosing cholangitis in two cases.

The overlap syndrome between autoimmune hepatitis and primary sclerosing cholangitis is a rare condition and only few cases have been published, partly associated with ulcerative colitis, but not with Crohn's disease. We report an autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome in a female patient with Crohn's disease. In addition, a second case of overlap syndrome is reported in a man without inflammatory bowel disease. A 24-year-old woman was referred with a 10-month history of diarrhoea and biochemical changes including elevated serum levels of alkaline phosphatase, aspartate aminotransferase, alanine aminotransferase and immunoglobulin G. Enzyme linked immunosorbent assay showed that antinuclear autoantibodies were elevated. Immunofluorescence for perinuclear-staining antineutrophil cytoplasmatic antibodies was positive. Diagnostic criteria of definite autoimmune hepatitis according to the International Autoimmune Hepatitis Group were fulfilled. Liver biopsy simultaneously showed criteria of autoimmune hepatitis and primary sclerosing cholangitis. Endoscopic retrograde cholangiography demonstrated features of primary sclerosing cholangitis. Colonoscopy and colonoscopic biopsies indicated an active Crohn's disease affecting the terminal ileum and the ascending and transverse colon. Furthermore, we report the case of a 28-year-old man with known primary sclerosing cholangitis for the previous 6 years, and who developed jaundice and a marked increase of aspartate aminotransferase, alanine aminotransferase and immunoglobulin G, leading to the diagnosis of definite autoimmune hepatitis. A review of the literature revealed only 16 cases of an autoimmune hepatitis/primary sclerosing cholangitis syndrome in patients without inflammatory bowel disease or in association with ulcerative colitis. We report two additional cases, one case showing an association with Crohn's disease.

Adult↗
PubMed2002

Immunohistochemical differences in the portal tract and acinar infiltrates between primary biliary cirrhosis and autoimmune cholangitis.

BACKGROUND: Antimitochondrial antibody-negative primary biliary cirrhosis, or autoimmune cholangitis, may be indistinguishable clinically and histologically from antimitochondrial antibody-positive primary biliary cirrhosis. AIMS: We aimed to compare the phenotypic markers of the portal and acinar infiltrates in autoimmune cholangitis and antimitochondrial antibody-positive primary biliary cirrhosis. PATIENTS AND METHODS: Formalin-fixed, paraffin-embedded liver sections were identified from 32 patients with a clinical and histological diagnosis of primary biliary cirrhosis. Thirteen were antimitochondrial antibody-negative (autoimmune cholangitis group) and 19 were antimitochondrial antibody-positive. The groups were well matched for age, histological stage, liver biochemistry and drug treatment. Immunohistochemical staining was performed using monoclonal antibodies against CD3 (pan T cell), CD8 (cytotoxic), CD45RO (memory), CD45RA (naive), CD68 (macrophages) and against the secreted form of eosinophilic cationic protein (EG2). RESULTS: In autoimmune cholangitis, both portal and acinar CD3 cell counts were significantly higher than in antimitochondrial antibody-positive primary biliary cirrhosis (median portal count 421 vs 257 cells/graticule, P< 0.03; median acinar count 18 vs 9 cells/graticule, P< 0.02). There were no differences between the groups in portal or lobular CD8, CD45RO, CD45RA, CD68 or EG2. Of the total group (antimitochondrial antibody positives and negatives), there were significantly more CD45RA cells in early (stage 1) compared with cirrhotic (stage 4) disease (median 19.3 vs 14 cells/graticule, P< 0.03). EG2 staining was found in eight of the 32 sections overall, but not in the patients with stage 1 disease (P< 0.04). CONCLUSION: CD3 counts are higher in autoimmune cholangitis than in antimitochondrial antibody-positive primary biliary cirrhosis in both portal and acinar areas. However, there are no significant differences in memory/naïve T-cell subsets between both conditions and, in both, loss of naive T lymphocytes and secretion of eosinophilic cationic protein occur with disease progression. This implies that the effector pathways of bile duct destruction are similar in autoimmune cholangitis and primary biliary cirrhosis.

Antibodies, Monoclonal↗
PubMed2005

Sclerosing cholangitis.

PURPOSE OF REVIEW: Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by strictures of the biliary tree. It is immune mediated, although the precise cause remains unknown. Recent reports have shown a higher prevalence and burden of disease than was previously suspected. RECENT FINDINGS: The research into the etiopathogenesis, epidemiology, diagnosis of cholangiocarcinoma, medical and surgical therapy, and timing and outcome of liver transplantation is discussed. SUMMARY: Genetic heterogeneity among patients with primary sclerosing cholangitis is supported, and further gene polymorphisms associated with protection against primary sclerosing cholangitis have been elucidated. Bile duct injury seems to be a multistep process. Magnetic resonance cholangiopancreatography is a cost-effective and accurate way of diagnosing primary sclerosing cholangitis in comparison with endoscopic retrograde cholangiopancreatography. Ursodeoxycholic acid may have a role as a colorectal and hepatobiliary cancer chemopreventive agent. Liver transplantation remains the only treatment in end-stage disease. The 5-year and 10-year patient and graft survival rates are comparable with those in patients without primary sclerosing cholangitis, but there is a higher rate of retransplantation for primary sclerosing cholangitis in most centers. Hepatobiliary malignancy is found in a minority of patients at transplantation, although 5-year survival rates for these patients are still promising.

Cholangitis, Sclerosing↗
PubMed2006

Sclerosing cholangitis.

PURPOSE OF REVIEW: Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by strictures of the biliary tree complicated by cirrhosis and cholangiocarcinoma. It is immune mediated although the precise etiology remains unknown. RECENT FINDINGS: Research into etiopathogenesis, epidemiology, the relationship with inflammatory bowel disease, diagnosis of cholangiocarcinoma, medical therapy, and the outcome of liver transplantation are discussed. SUMMARY: It is likely that a number of patients previously diagnosed with primary sclerosing cholangitis have autoimmune pancreatitis in association with primary sclerosing cholangitis, a syndrome with distinct clinicopathological features including steroid responsiveness. Primary sclerosing cholangitis-inflammatory bowel disease probably represents a distinct inflammatory bowel disease phenotype, which has implications for colonoscopic surveillance of these patients. CA19-9 plays no surveillance role for the early detection of cholangiocarcinoma. The best-studied drug in primary sclerosing cholangitis is ursodeoxycholic acid, which, despite a range of potentially valuable actions on the cholestatic liver, has not yet been proved to make a substantial impression on the course of the disease. Orthotopic liver transplantation remains the only established long-term treatment for primary sclerosing cholangitis.

Bile Duct Neoplasms↗
PubMed2004

Endoscopic biliary drainage for severe acute cholangitis in biliary obstruction as a result of malignant and benign diseases.

BACKGROUND AND AIMS: Endoscopic biliary drainage is an established mode of biliary decompression in patients with acute cholangitis as a result of biliary obstruction secondary to stones and benign strictures. However, there are no reports on endoscopic management of severe acute cholangitis caused by malignant conditions. We prospectively compared the efficacy of the endoscopic drainage for severe acute cholangitis in biliary obstruction as a result of malignant and benign diseases. METHODS: Forty-three patients with severe acute cholangitis requiring urgent biliary drainage were included. Sixteen patients (mean age 58.2 +/- 9.3 years; seven men, nine women) had biliary obstruction as a result of malignant diseases and 27 had benign biliary diseases (mean age 41.6 +/- 14.3 years; nine men, 18 women). Indications for urgent drainage included any one of the following: temperature >38 degrees C (n = 21), septic shock with systolic blood pressure <100 mmHg (n = 9), localized peritonism (n = 21), impaired consciousness (n = 6) and failure to improve within 72 h of conservative management (n = 13). After successful bile duct cannul degrees ation, patients received either a nasobiliary catheter (n = 38) or an in-dwelling stent (n = 5) with or without sphincterotomy for biliary drainage. Outcome measures included complications and clinical response. RESULTS: Endoscopic drainage was established successfully in all the patients in both the groups. Clinical improvement after biliary drainage occurred in 94% patients (15/16) in the malignant group compared with 96% patients (26/27) in the benign group (P = not significant [NS]). Fever subsided at a median of 2.2 days in the malignant group and at 1 day in the benign group (P = NS). Normalization of leukocyte count was seen at a median of 6 days (range 1-17) and 2 days (range 1-5) days in the malignant group and the the benign group, respectively (P = NS). There were no endoscopic retrograde cholangiopancreatography-related complications. The mortality rate as a result of cholangitis was 4.6%, that is two of 43 patients (6.2% of the malignant group vs 3.7% of the benign group; P = NS). CONCLUSIONS: Endoscopic biliary drainage is equally effective in patients with severe acute cholangitis caused by either malignant or benign biliary diseases.

Acute Disease↗
PubMed2006

New strategy to prevent ascending cholangitis in larger choledochoduodenal fistula.

BACKGROUND: Choledochoduodenal fistula (CDF) is a complication of common bile duct stones or cholangitis in Asia. It is unclear as to which type of the fistula needs surgical treatment. METHODS: To determine whether the sizes of CDF imply different clinical presentations and treatments, we reviewed 50 patients with CDF and their treatments during a recent 14-year period. For treatments of CDF, we applied the conventional methods, including removal of stone and complete decompression of biliary obstruction to treat the original bile lesions. In addition, according to the sizes of fistula and the frequencies of ascending cholangitis, we proposed the following strategies for fistula treatments: (i) for fistula orifices larger than 1 cm, a transection of common bile duct was applied to prevent the reflux of duodenal juice; (ii) for fistula orifices between 0.5 and 1.0 cm, an effective biliary drainage was applied; and (iii) for fistula orifices less than 0.5 cm, non-surgical treatments were applied. RESULTS: We found that hepatic biliary duct stones and hepatic biliary duct strictures were associated with more severe cholangitis (P = 0.037 and P = 0.009, respectively), but not with the episodes of cholangitis (P = 0.654 and P = 0.664, respectively). In contrast, the sizes of fistula >1 cm were associated with more frequent episodes of cholangitis (r = 0.774; P < 0.001). CONCLUSION: The larger fistula increases frequency of cholangitis episodes and needs surgical treatment for fistula itself.

Bile Duct Diseases↗
PubMed1990

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.

Antibody Specificity↗
PubMed1992

Sclerosing cholangitis and biliary tract calculi--primary or secondary?

The clinical features of 61 patients with sclerosing cholangitis were reviewed. This group included 23 patients with biliary tract calculi, commonly considered as excluding the diagnosis of primary sclerosing cholangitis. The aim of this study was to compare these 23 patients (group A) with 38 patients with sclerosing cholangitis free of calculi (group B). Both groups had the following features in common: (i) age at presentation, (ii) incidence of inflammatory bowel disease, (iii) extent of radiological disease, (iv) prevalence of HLA-B8 and DR3 haplotype, (v) incidence of cholangiocarcinoma, and (vi) progression to hepatic transplantation (mean follow up 49.9 months). All patients in group A were symptomatic at diagnosis compared with 23 of the 38 patients (61%) in group B. Recurrent ascending cholangitis occurred in 12 patients in group A (52%) and two patients (5%) in group B. The similarity between the two groups was maintained when the nine patients in group A who developed calculi after sclerosing cholangitis was diagnosed were excluded. It is concluded that choledocholithiasis is part of the spectrum of primary sclerosing cholangitis and that it is not necessary to invoke choledocholithiasis as the initial lesion of the bile ducts in such patients.

Adolescent↗
PubMed1993

The outcome of cholangitis after percutaneous biliary drainage in neoplastic jaundice.

The purpose of this paper is to evaluate factors affecting the outcome of cholangitis after PTBD in jaundiced cancer patients. Twenty nine patients with neoplastic jaundice (male/female ratio 13/16, median age 55 years) with full clinical data, were treated by PTBD and developed cholangitis at a median of 9 days later. Four patients (14%) died of biliary sepsis a median of one month after PTBD while the other 25 survived a median of 6 months, with one week median duration of cholangitis. The probability of the cholangitis resolving was analyzed by time to resolution and it was found that 50% and 100% of the recoveries occurred 5 and 9 months respectively from the onset of the complication. The series was analyzed to determine the role of several variables (disease/patient/treatment related) in the resolution of cholangitis. Only a low stricture site, a large initial drainage catheter (10F) and a temperature increase exceeding 39 degrees C were correlated with a positive outcome. We conclude that PTBD-related cholangitis has, in our experience, a good chance of cure, low mortality rate and satisfactory 6 months median survival.

Acute Disease↗
PubMed2002

Primary biliary cirrhosis and autoimmune cholangitis are not associated with coeliac disease in Crete.

BACKGROUND: An increased prevalence of coeliac disease in patients with primary biliary cirrhosis has been recently reported. However, in other studies the association has not been confirmed. There have been no formal attempts to systematically evaluate patients with autoimmune cholangitis for coeliac disease. METHODS: Sera from 62 patients with primary biliary cirrhosis, 17 with autoimmune cholangitis and 100 blood donors were screened for anti-gliadin, anti-endomysial, anti-reticulin, and IgA class antibodies to guinea pig liver-derived tissue transglutaminase. Eighteen untreated coeliacs served as methodological controls. Analyses were performed by using the chi2 and Fischer's exact tests. RESULTS: Anti-gliadin antibodies were detected in 21% of patients with primary biliary cirrhosis, 35% of patients with autoimmune cholangitis, and 3% of controls (p < 0.001). IgA class gliadin antibodies positivity was more pronounced in patients with Scheuer's stage III-IV disease (p < 0.05). Anti-transglutaminase antibodies were detected in 10% and in 18% of patients with primary biliary cirrhosis and autoimmune cholangitis respectively (p < 0.001). Anti-reticulin and anti-endomysial antibodies were negative in all patients. Duodenal biopsies were performed in 59% and 71% of patients with primary biliary cirrhosis and autoimmune cholangitis respectively, tested positive for at least one antibody class. No histological features of coeliac disease were found. CONCLUSIONS: We were unable to demonstrate an increased risk of coeliac disease in patients with primary biliary cirrhosis and autoimmune cholangitis. Our results confirm the previously reported high prevalence of false-positive anti-gliadin and guinea pig liver-derived anti-tissue transglutaminase antibodies in patients with chronic liver disease.

Adult↗
PubMed1996

Piperacillin to prevent cholangitis after endoscopic retrograde cholangiopancreatography. A randomized, controlled trial.

BACKGROUND: Cholangitis does not often occur after endoscopic retrograde cholangiopancreatography (ERCP), but it can be a serious complication of this procedure. Antibiotic prophylaxis is therefore frequently used in patients having ERCP, but existing data are insufficient to allow evaluation of the effectiveness of this practice. OBJECTIVE: To determine the efficacy of single-dose antibiotic prophylaxis with piperacillin for ERCP-induced cholangitis. DESIGN: Randomized, double-blind, placebo-controlled clinical trial. SETTING: Tertiary referral center for ERCP. PATIENTS: Patients who had ERCP for suspected biliary tract stones or distal common bile duct stricture were eligible. Major exclusion criteria were previous ERCP within 7 days, biliary endoprosthesis in situ, and use of antimicrobial agents or presence of fever within 7 days before the procedure. INTERVENTION: Piperacillin, 4 g, or placebo was given intravenously approximately 30 minutes before ERCP. MEASUREMENTS: Duration of follow-up was 1 week. Acute cholangitis was diagnosed if a patient had a body temperature greater than 38 degrees C, a clinically apparent need for antibiotic treatment, and no symptoms indicating infection outside of the biliary tree. RESULTS: 551 consecutive patients were enrolled. During ERCP, stones were found in 147 patients, malignant distal strictures were found in 203 patients, other pathologic findings were seen in 88 patients, and normal biliary tracts were seen in 113 patients. Seventeen of the 281 patients who received placebo (6.0%) and 12 of the 270 patients who received piperacillin (4.4%) developed acute cholangitis (relative risk, 0.73 [95% CI, 0.36 to 1.51]). The absolute risk reduction was 1.6% (CI, -5.3% to 2.1%). All cases of cholangitis (with the exception of one case seen in a patient in the piperacillin group) were mild or moderate in severity. CONCLUSION: Single-dose prophylaxis with piperacillin is not associated with a clinically significant reduction in the incidence of acute cholangitis after ERCP in patients suspected of having biliary tract stones or distal common bile duct stricture.

Adult↗
PubMed2001

Ursodiol use is associated with lower prevalence of colonic neoplasia in patients with ulcerative colitis and primary sclerosing cholangitis.

BACKGROUND: Patients with ulcerative colitis and primary sclerosing cholangitis are at high risk for colonic dysplasia and cancer. This risk approaches 50% after 25 years of colitis. Ursodiol has been shown to protect against development of colorectal neoplasia in animal models. OBJECTIVE: To assess the relationship between ursodiol use and colonic dysplasia, the precursor to colon cancer, in patients with ulcerative colitis and primary sclerosing cholangitis. DESIGN: Cross-sectional study. SETTING: University medical center. PATIENTS: 59 patients with ulcerative colitis and primary sclerosing cholangitis who were undergoing colonoscopic surveillance for colonic dysplasia. MEASUREMENTS: Use of ursodiol was assessed in all patients. The presence or absence of colonic dysplasia was evaluated by colonoscopic surveillance. Other variables assessed were age at onset and duration of ulcerative colitis; duration of primary sclerosing cholangitis; Child-Pugh classification; and use of sulfasalazine, other 5-aminosalicylic acid preparations, prednisone, cyclosporine, azathioprine, and methotrexate. RESULTS: Ursodiol use was strongly associated with decreased prevalence of colonic dysplasia (odds ratio, 0.18 [95% CI, 0.05 to 0.61]; P = 0.005). The association between dysplasia and ursodiol use remained after adjustment for sex, age at onset of colitis, duration of colitis, duration of sclerosing cholangitis, severity of liver disease, and sulfasalazine use (adjusted odds ratio, 0.14 [CI, 0.03 to 0.64]; P = 0.01). Younger age at onset of colitis was associated with an increased risk for dysplasia. CONCLUSIONS: Ursodiol use appears to be associated with a lower frequency of colonic dysplasia in patients with ulcerative colitis and primary sclerosing cholangitis. A randomized trial investigating the chemoprotective effect of ursodiol in patients with ulcerative colitis may be warranted.

Adult↗
PubMedDate not supplied

Three cases of primary sclerosing cholangitis associated with ulcerative colitis; diagnostic usefulness of magnetic resonance cholangiopancreatography.

BACKGROUND/AIMS: Primary sclerosing cholangitis is often accompanied by inflammatory bowel disease in western countries. However, the incidence of primary sclerosing cholangitis in patients with ulcerative colitis appears to be much lower in Japan. METHODOLOGY: Between 1980 and 1998, a total of 402 patients with ulcerative colitis were seen in our department. The patients were evaluated by abdominal ultrasonography, endoscopic retrograde cholangiopancreatography, and/or magnetic resonance cholangiopancreatography when persisting abnormalities of biochemical findings suggested the presence of hepatobiliary diseases. RESULTS: Of the 402 patients with ulcerative colitis, 3 patients with primary sclerosing cholangitis were found. There were 2 men and 1 woman. One patient had left-sided colitis while 2 had total colitis. Magnetic resonance cholangiopancreatography was done in 2 of these 3 patients and demonstrated diagnostic features of primary sclerosing cholangitis. All 3 patients had intra- and extrahepatic involvement by primary sclerosing cholangitis. One male patient died due to progressive hepatic failure. The other male patient was treated with ursodeoxycholic acid, but serum alkaline phosphatase level remained above the normal range. The female patient maintained normal serum alkaline phosphatase levels without specific medication. CONCLUSIONS: Magnetic resonance cholangiopancreatography is the most safe and convincing tool for the diagnosis of coexistent primary sclerosing cholangitis in the patients with ulcerative colitis.

Adult↗
PubMedDate not supplied

Endoscopic sphincterotomy in patients with "acalculus" cholangitis associated with juxtapapillary diverticula.

BACKGROUND/AIMS: The aim of this study was to investigate patients who underwent endoscopic sphincterotomy for "acalculus" cholangitis associated with juxtapapillary diverticula. METHODOLOGY: In a retrospective study we analyzed 87 patients who underwent endoscopic sphincterotomy for cholangitis; the cholangitis considered "acalculus", when outlining the extra- and intrahepatic bile ducts, we could not observe any intraluminal defect or stricture, and during the clearing of the bile ducts with the balloon, after endoscopic sphincterotomy, there was no evidence of stones, fragments of stones or sludge. Patients who had undergone previous endoscopic sphincterotomy, or who had additional pancreatobiliary diseases were excluded from this study. There were 11 patients with "acalculus" cholangitis associated with juxtapapillary diverticula, and sufficient clinical data available for this study. RESULTS: Nine patients presented pain, fever, and jaundice. In two patients diagnosis was established via the test of abnormal liver biochemistry. Seven patients had positive blood cultures and three of them developed confusion and hypotension. Endoscopic sphincterotomy succeeded in all cases; no evidence of stones, fragments of stones or sludge was recorded during the clearing of bile ducts, after endoscopic sphincterotomy, with the balloon. Five patients presented mild post-endoscopic sphincterotomy complications successfully treated. In the follow-up period, from 4 months to 7 years after endoscopic sphincterotomy, none of the patients developed symptoms of cholangitis. CONCLUSIONS: We recommend endoscopic sphincterotomy in patients with "acalculus" cholangitis associated with juxtapapillary diverticula, despite the absence of obvious obstruction, and the possible morbidity which is inherent with an invasive procedure like endoscopic sphincterotomy.

Aged↗
PubMedDate not supplied

Non-calculus suppurative cholangitis in Danish patients with inflammatory bowel disease.

BACKGROUND/AIMS: We examined the risk of non-calculus suppurative cholangitis in patients with inflammatory bowel disease in the entire Danish population. METHODOLOGY: The study included all patients discharged from Danish hospitals with a diagnosis of Crohn's disease or ulcerative colitis as registered in the Danish National Registry of Patients from January 1, 1977 to December 31, 1992. We compared the observed number of patients hospitalized with suppurative cholangitis with expected numbers on the basis of age, gender, and calendar-specific incidence rates in the general population. RESULTS: Overall, 15,317 eligible patients with inflammatory bowel disease were discharged during the study period. Among these were 52 cases of non-calculus suppurative cholangitis. The incidence rate of non-calculus suppurative cholangitis in the cohort with inflammatory bowel disease was 46.1 per 100,000 person-years. The standardized incidence ratio (SIR) for suppurative cholangitis was increased similarly for patients with Crohn's disease [SIR=6.7, 95% confidence interval (CI): 3.1-12.7] and for patients with ulcerative colitis (SIR=6.6, 95% CI: 4.7-9.1). The highest relative risk was found in male patients younger than 40 years of age, for both Crohn's disease and ulcerative colitis (SIR=70.5 and 78.7, respectively). CONCLUSIONS: Patients with inflammatory bowel disease have an increased risk of non-calculus suppurative cholangitis.

Adult↗
PubMed1991

[Surgical tactics in cholangitis in middle-aged and elderly patients].

The authors analysed the results of examination and treatment of 399 elderly and old-aged patients in the period between 1971 and 1981. At that time cholangitis was managed in 146 (36.6%) patients mainly by surgical intracavitary operations, in 21.2% with a fatal outcome. The results showed the high risk of surgical methods for treatment of cholangitis. Since 1982 the authors have introduced into every-day practice endoscopic methods for the management of this disease. It was found that endoscopic papillosphincterotomy (EPST) is the most physiological and safe method for the treatment of choledocholithiasis, stenosis of the major duodenal papilla, and acute cholangitis. EPST undertaken in 253 (63.4%) patients ensured adequate decompression of the bile ducts in 187 (73.9%) of them and facilitated removal of cholangitis and recovery. With the use of EPST in the treatment of acute cholangitis not only was the number of surgical operations on patients of the older age group reduced, but the mortality decreased to 5.5% (which was 3.9 times lower than the mortality in management of cholangitis by surgical operations).

Age Factors↗
PubMed1988

[Clinical and experimental study on reconstruction after pancreatectomy--Part I. Cholangitis and its relationship to reconstruction].

The purpose of the present study is to clarify pathogenesis of cholangitis after pancreatectomy. The clinical study consisted of follow-up survey and hepatobiliary scintigraphy for the patients with type-I reconstruction (e.g. Imanaga procedure) and type-II reconstruction (e.g. Child procedure) after pancreatoduodenectomy. The experimental study was performed in dogs by creating three types of biliary reconstruction, namely, cholecystoduodenostomy (C-D), cholecystojejunostomy (C-J) and Roux-Y cholecystojejunostomy (R-Y). In the follow-up survey, cholangitis occurred in 19.0% of 21 type-I patients and in 33.3% of 18 type-II patients. In the hepatobiliary scintigraphy, type-I demonstrated smooth transit of bile along the reestablishing intestine. Type-II, on the other hand, showed marked stagnation of bile in the excluded loop, which could even trigger cholangitis. The experimental study showed that the results obtained from R-Y were no better than those obtained from C-D and C-J with respect to prevention of cholangitis based on histological, biochemical and bacteriological point of view. These studies suggest that type-I reconstruction carries little risk of causing cholangitis, whereas type-II reconstruction is not effective in preventing cholangitis.

Anastomosis, Roux-en-Y↗
PubMed1983

Primary sclerosing cholangitis.

Primary sclerosing cholangitis is now being recognized as the underlying cause of liver disease with increasing frequency. This is due to the widespread use of cholangiography especially endoscopic retrograde cholangiopancreatography. Hepatic histology shows a spectrum of changes and is diagnostic in only a minority of patients. Several distinct clinical presentations of primary sclerosing cholangitis are now recognized. While most patients present with a cholestatic illness and acute cholangitis, a proportion simulate chronic active hepatitis, cryptogenic cirrhosis or primary biliary cirrhosis. Some patients are entirely asymptomatic. Primary sclerosing cholangitis is associated with inflammatory bowel disease (predominantly ulcerative colitis) and a variety of fibrosing diseases. Approximately 3 percent of patients with ulcerative colitis develop primary sclerosing cholangitis. The prognosis of primary sclerosing cholangitis is variable; it can no longer be regarded as an invariably progressive cholestatic disease. Patients may remain asymptomatic or enjoy remissions lasting many years. Treatment for the disease remains unsatisfactory despite attempts with numerous medical and surgical measures.

Adult↗