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PubMed2006

Rapid development of secondary sclerosing cholangitis due to vancomycin-resistant enterococci.

Secondary sclerosing cholangitis is a rare complication of acute bacterial cholangitis. The natural course of the disease is a slow progression over years. Here we report about a patient in whom sclerosing cholangitis evolved much faster as a consequence of a persistent biliary infection with vancomycin-resistant enterococci. The infection could be treated successfully with prolonged therapy with linezolid and teicoplanin. This is the first report of sclerosing cholangitis caused by vancomycin-resistant enterococci. It illustrates the rapidity with which this disease can evolve as a fulminant and ultimately fatal complication of acute bacterial cholangitis.

Aged, 80 and over↗
PubMed2004

Effective antibiotic regime for postoperative acute cholangitis in biliary atresia--an evolving scene.

PURPOSE: The prompt use of empirical antibiotics is vital in managing post-Kasai cholangitis. The authors published findings of their clinical trial in 1991 and established the use of cefoperazone, with a response rate of 88.9%. Here its clinical use since its introduction is reviewed and the trend in its efficacy is assessed. METHODS: A retrospective review was carried out between 1997 and 2003. All episodes of acute cholangitis in patients who underwent Kasai procedure were recorded. Cholangitis was defined as unexplained fever with derangement of liver enzymes. Cefoperazone was started empirically according to the established protocol, and the response to treatment was analyzed. RESULTS: There were 19 patients with a total of 49 episodes of cholangitis. Cefoperazone was used as the first-line empirical antibiotic in 40 of these episodes. Only 30 showed successful response (75%). For the 10 unresponsive episodes, meropenem was used as second-line antibiotic with complete response in all. CONCLUSIONS: The efficacy of cefoperazone in the treatment of post-Kasai cholangitis has decreased over the last years. This suggests a need for a more effective first-line empirical antibiotic. From this review, meropenem seems to be a suitable candidate, and a future prospective clinical trial is warranted.

Acute Disease↗
PubMed1987

Bacterial cholangitis after surgery for biliary atresia.

We retrospectively studied the incidence of bacterial cholangitis in 129 infants operated on because of biliary atresia over 5 years. Forty-six of the 101 children who underwent hepatic portoenterostomy had a total of 105 episodes of cholangitis (range one to eight episodes per child). Most episodes occurred within 3 months of the operation. Factors associated with cholangitis included good or partial restoration of bile flow, abnormal intrahepatic bile ducts or cavities at the porta hepatis, and routine postoperative use of antibiotics. External jejunostomy was not effective in preventing cholangitis. In addition to fever and decreased bile flow, increased erythrocyte sedimentation rate and signs of shock were frequently observed. The responsible organisms, most often gram-negative bacteria, were identified in 79 (75%) episodes by blood or liver cultures. Most were susceptible to trimethoprim-sulfamethoxazole and third-generation cephalosporins during the first episode, but only to cephalosporins during later episodes. The incidence of signs of portal hypertension in children with normal serum bilirubin values at age 5 years was not higher in those who had previously experienced one or more episodes of cholangitis.

Biliary Atresia↗
PubMed1990

Primary sclerosing cholangitis.

Primary sclerosing cholangitis is a rare disease of unknown etiology. Sclerosis of the bile ducts may actually be the final result of multiple factors such as autoimmune, bacterial, congenital, drug, or viral injury. The most commonly associated diseases are ulcerative colitis and chronic pancreatitis. Except in the earliest stages of the disease, liver histologic findings are not specific. Most patients present with jaundice, pain, and pruritus, although an increasing number of asymptomatic patients with inflammatory bowel disease and abnormal liver function are being identified. Cholangiography is key to the diagnosis and is usually pathognomonic except in the unusual case where primary sclerosing cholangitis is confused with cholangiocarcinoma. Many forms of medical therapy have been tried, including antibiotics, azathioprine, cholestyramine, colchicine, cyclosporine, D-penicillamine, steroids, and ursodeoxycholic acid. To date, none of these medications has been proved to alter the course of this disease. Recent reports of ursodeoxycholic acid trials have been encouraging, but long-term results of ongoing randomized trials have yet to be published. In recent years, balloon dilatation of biliary strictures has been accomplished via endoscopic and percutaneous transhepatic approaches. However, in patients with primary sclerosing cholangitis, these nonoperative manipulations must be done repeatedly, may entail multiple general anesthetics, and are difficult to perform. We believe that a direct surgical approach to the biliary tree with long-term transhepatic stenting is indicated in selected patients with severe hilar or extrahepatic stricturing, persistent jaundice or recurrent cholangitis, and no evidence of cirrhosis. Hepatic transplantation should be reserved for patients with primary sclerosing cholangitis who have well-established cirrhosis and have not responded to other therapeutic measures.

Cholangitis, Sclerosing↗
PubMed2000

A novel rat model of chronic fibrosing cholangitis induced by local administration of a hapten reagent into the dilated bile duct is associated with increased TNF-alpha production and autoantibodies.

BACKGROUND/AIM: The cholangiopathies represent hepatobiliary diseases in which bile-duct epithelial cells are targets for destructive processes, including immune-mediated damage. We describe a novel rat model of chronic fibrosing cholangitis induced by administration of the hapten reagent 2,4,6-trinitrobenzenesulfonic acid (TNBS) into the dilated bile duct. METHODS: The common bile duct was dilated due to a mild stenosis in 8-week-old female Lewis rats. TNBS (50 mg/kg) was injected during a second laparotomy. RESULTS: TNBS-treatment reproducibly resulted in chronic fibrosing cholangitis. In retrograde cholangiography the bile ducts showed irregularities, beading and strictures. Alkaline phosphatase levels remained abnormal throughout the study period. Immunohistochemical staining showed an increased number of macrophages, CD3+ T-lympbocytes and MHC class II antigen upregulation. The spontaneous interferon-gamma, tumor necrosis factor-alpha and interleukin-10 production of liver-derived mononuclear cells was increased. Anti-neutrophil cytoplasmic antibodies with specificity against myeloperoxidase, catalase and actin were found between 1 and 12 weeks after TNBS injection. CONCLUSIONS: We established a novel rat model of chronic fibrosing cholangitis with histologic, cholangiographic, serologic and immunologic similarities to human primary sclerosing cholangitis. This model may be used to study pathomechanisms of chronic cholangitis without concomitant inflammatory bowel disease.

Animals↗
PubMed1996

Evidence for an overlap syndrome of autoimmune hepatitis and primary sclerosing cholangitis.

BACKGROUND/AIMS: Autoimmune hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis are chronic liver diseases with probable autoimmune background. Overlapping features have been described for primary biliary cirrhosis and autoimmune hepatitis. In contrast, there have been only a few case reports on an overlap of autoimmune hepatitis and primary sclerosing cholangitis. METHODS: We describe three male patients with clinical and histological overlapping features of primary sclerosing cholangitis and autoimmune hepatitis. RESULTS: All initially asymptomatic patients had elevated levels of aminotransferases, alkaline phosphatase, gamma-glutamyltranspeptidase and IgG. Anti-nuclear antibodies and/or smooth muscle antibodies were positive and anti-neutrophil cytoplasmic antibodies were detected in all patients. Retrograde endoscopic cholangiography showed bile-duct strictures characteristic for primary sclerosing cholangitis. Histopathology showed necro-inflammatory activity of portal tracts with bridging necrosis in all patients at the time of first diagnosis. Aminotransferase levels and the necro-inflammatory activity responded well to immunosuppressive treatment. Predominant periductular fibrosis as a typical histopathological feature of primary sclerosing cirrhosis was seen to develop in all patients. Cholestatic serum parameters remained elevated and periductular fibrosis as endoscopic bile duct changes progressed despite immunosuppression. CONCLUSIONS: We suggest that these patients present an overlap syndrome of autoimmune hepatitis and primary sclerosing cholangitis as they fulfill the diagnostic criteria for both conditions.

Adolescent↗
PubMed1998

Abnormal accumulation of endotoxin in biliary epithelial cells in primary biliary cirrhosis and primary sclerosing cholangitis.

BACKGROUNDS/AIMS: Previous studies have revealed the involvement of Kupffer cells and hepatocytes in the metabolism of endotoxin in the liver. The aim of this study was to investigate the in vivo localization of endotoxin in liver cells, including Kupffer cells, hepatocytes, and biliary epithelial cells, in primary biliary cirrhosis and primary sclerosing cholangitis. We also examined the effect of ursodeoxycholic acid on the intrahepatic distribution of endotoxin in primary biliary cirrhosis. METHODS: The immunohistochemical localization of endotoxin was examined in liver specimens from 30 cases of primary biliary cirrhosis and seven of primary sclerosing cholangitis using a monoclonal antibody against lipid A. Controls were seven cases of obstructive jaundice, ten of hepatitis C virus-related liver cirrhosis, 14 of chronic hepatitis C, and five histologically normal liver cases. Semi-quantitative analysis of endotoxin accumulation was performed to measure the intensity of fluorescence for endotoxin. Nine of the 30 patients with primary biliary cirrhosis underwent a second liver biopsy for evaluation of the ursodeoxycholic acid treatment. RESULTS: In primary biliary cirrhosis and primary sclerosing cholangitis, biliary epithelial cells showed strong immunostaining for endotoxin as well as hepatocytes and Kupffer cells. Biliary epithelial cells of primary biliary cirrhosis and primary sclerosing cholangitis showed more intense immunoreactivity than those of other controls. In primary biliary cirrhosis, ursodeoxycholic acid reduced the immunoreactivity to endotoxin in biliary epithelial cells, and increased the immunoreactivity to endotoxin in Kupffer cells, but did not affect that in hepatocytes. CONCLUSIONS: Our results revealed that in primary biliary cirrhosis and primary sclerosing cholangitis, endotoxin accumulates abnormally in biliary epithelial cells. In addition, we found that ursodeoxycholic acid treatment in primary biliary cirrhosis may provide a beneficial effect on the intrahepatic metabolism of endotoxin.

Adult↗
PubMed1998

Bile duct bacterial isolates in primary sclerosing cholangitis: a study of explanted livers.

BACKGROUND/AIMS: The pathogenesis of the inflammatory lesion in primary sclerosing cholangitis is unknown. The clinical picture is characterized by i.a. episodes of fever, the cause of which also remains speculative. Previous studies of bacterial isolates in the liver or bile ducts in primary sclerosing cholangitis have had the shortcoming of possible contamination associated with the sampling. The aim of this study was to investigate whether bile and bile duct tissue, obtained under sterile conditions in connection with liver transplantation, contain bacteria. METHODS: We studied bile from bile duct walls and bile collected from the explanted livers of 36 patients with primary sclerosing cholangitis and 14 patients with primary biliary cirrhosis. RESULTS: Positive cultures were obtained from 21 of 36 primary sclerosing cholangitis patients, but from none of the primary biliary cirrhosis patients. The number of bacterial strains was inversely related to the time after the last endoscopic retrograde cholangiography. Treatment with antibiotics or intraductal stent, or the occurrence of fever before liver transplantation did not seem to influence the culture results, whereas antibiotic treatment in connection with endoscopic retrograde cholangiography may possibly have reduced the number of isolates in the cultures. Alpha-haemolytic Streptococci were retrieved as late as 4 years after the last endoscopic retrograde cholangiography. Retrospective analysis of liver laboratory tests after endoscopic retrograde cholangiography did not indicate a deleterious effect of the investigation. CONCLUSIONS: The data suggest that antibiotics should be given routinely in connection with endoscopic retrograde cholangiography. They also raise the question of a possible role of alpha-haemolytic Streptococci in the progression of primary sclerosing cholangitis.

Adult↗
PubMed1999

Sclerosing cholangitis in adults with cystic fibrosis: a magnetic resonance cholangiographic prospective study.

BACKGROUND/AIMS: Liver disease is a leading cause of morbidity in adult patients with cystic fibrosis. Diagnosis of limited liver involvement in asymptomatic patients is important since a safe and effective treatment with ursodeoxycholic acid can be used. We carried out a prospective open study to describe the intrahepatic biliary lesions using magnetic resonance cholangiography. METHODS: Twenty-seven adult patients with cystic fibrosis were prospectively enrolled, whatever their hepatobiliary status. All patients underwent liver function tests, ultrasonography and magnetic resonance cholangiography. Magnetic resonance cholangiograms were acquired on a Philips 1.5 Tesla unit using a 3D TSE MR sequence. Acquisition parameters (120 slices, 1.6 mm thickness, interslice overlap 0.8 mm) were followed by MIP reconstruction in two orthogonal planes. Magnetic resonance cholangiography images were assessed for the presence of stenosis, dilatations and rigidity corresponding to current criteria of cholangitis. Among the 27 cystic fibrosis patients, 18 (Group I) fulfilled none of the clinical, biological or ultrasonographic criteria of liver disease; the remaining nine (Group II) fulfilled the criteria for liver disease. In every patient, current causes of secondary sclerosing cholangitis had been excluded. RESULTS: All the Group II patients had abnormal magnetic resonance cholangiograms with features resembling those of primary sclerosing cholangitis in five, and simple biliary lesions in four. Nine Group I patients had abnormal magnetic resonance cholangiograms with primary sclerosing cholangitis-like lesions in five and simple biliary lesions in four. Magnetic resonance cholangiography anomalies were always dilatations, either isolated or associated with strictures and rigidity, both resembling those seen in cholangitis. They were seen in all the patients with known liver disease and in half the patients without evidence of liver disease. CONCLUSION: This study confirms the high frequency of intrahepatic biliary abnormalities in CF patients, which is probably underestimated by clinical, biological and ultrasonographic evaluation. The magnetic resonance cholangiography technique could be useful to detect early intrahepatic biliary tract involvement in cystic fibrosis patients.

Adult↗
PubMed2006

[Acute cholangitis: imaging diagnosis and management].

Cholangitis is an infection of the biliary ductal system that results from biliary obstruction. Choledocholithiasis has been the leading cause of acute cholangitis. Acute cholangitis remains a life-threatening complication of biliary obstruction that needs emergency diagnosis and treatment. Ultrasound (US) is the primary imaging modality for assessment of patients with suspected acute cholangitis. US is both sensitive and specific in demonstrating biliary dilatation. However, biliary dilatation is not always present at the early stage of bile duct obstruction and the performance of US in demonstrating choledocholithiasis is poor. Computed tomography (CT) without contrast injection is more sensitive than US in demonstrating choledocholithiasis. Magnetic resonance cholangiopancreatography (MRCP) and endoscopic sonography (EUS) are the most sensitive techniques to correctly determine the underlying cause and level of biliary obstruction in patients with acute cholangitis. In patients without previous cholecystectomy, clinical, biological, and US results allow to determine patients with high probability of having choledocholithiasis even if the stone is not directly visible on US. Patients undergoing cholecystectomy require laparoscopic common bile duct exploration, especially if the common bile duct clearance is not checked before surgery by MRCP or EUS.

Acute Disease↗
PubMed2003

[Sclerosing cholangitis as a complication of Langerhans'cell histiocytosis].

INTRODUCTION: Langerhans'cell histiocytosis is a rare and mainly pediatric disease. Patients with hepatic involvement usually have a disseminated form of the disease, with poor prognosis. Sclerosing cholangitis can occur in 10 to 15% of multivisceral Langerhans'cell histiocytosis. We report the case of a 56-years-old patient who developed sclerosing cholangitis 12 years after the diagnosis of Langerhans'cell histiocytosis. EXEGESIS: A 56-years-old man was admitted because of rapid general health impairment with epigastric pain. He was diagnosed as having Langerhans'cell histiocytosis 12 years ago because of a diabetes insipidus. Lungs were involved and during follow-up vertebral osteocondensation also developed. However, Langerhans'cell histiocytosis was clinically silent at the time of admission, without any treatment. Biologically, cholestasis and inflammation were noted. Digestive radiological investigations (echo-endoscopy, CT-scan, MRI) showed homogenous hepatomegaly and a diffuse infiltration of intra and extrahepatic bile ducts. Liver biopsy yielded the diagnosis of sclerosing cholangitis. Clinical and biological improvement occurred with oral corticosteroids (at 12 months after sclerosing cholangitis diagnosis). CONCLUSION: Sclerosing cholangitis is a potential complication of Langerhans'cell histiocytosis, mainly in its multivisceral form. It can occur at a median of 2 years after diagnosis in children, but occasionally much later in adults, whereas Langerhans'cell histiocytosis seems quiescent. Diagnosis is supported by radiological investigations and liver biopsy. As no drug therapy appears clearly effective, liver transplantation must frequently be considered in these patients.

Abdominal Pain↗
PubMed1997

An atypical presentation for primary sclerosing cholangitis.

We describe the clinical course of a group of patients with primary sclerosing cholangitis who at presentation were diagnosed to have autoimmune hepatitis. The history of one such patient is described in detail. We also compare this atypical sclerosing cholangitis (group I) to typical sclerosing cholangitis (group II) and to autoimmune hepatitis with (group III) and without (group IV) cholestasis. At presentation, mean AST in groups I and III was similar and significantly higher than in group II (P < 0.05). Mean ALP was higher in sclerosing cholangitis than in autoimmune hepatitis but not significantly so. Triaditis was present in all patients in groups I, III, and IV. Piecemeal necrosis and multilobular collapse/fibrosis were equally frequent in groups I, III, and IV. Only the response to corticosteroids helped differentiate among groups. Groups III and IV responded by normalizing AST. In group I, AST improved, but never became normal. As ALP became disproportionately abnormal (ALP-predominant pattern), cholangiography was performed, and the diagnosis of primary sclerosing cholangitis was made in all group I patients. We recommend that cholangiography be performed early in patients with suspected autoimmune hepatitis who partially respond to corticosteroids and develop an ALP-predominant pattern.

Adrenal Cortex Hormones↗
PubMed2003

Review article: is there an optimal therapeutic regimen for antimitochondrial antibody-negative primary biliary cirrhosis (autoimmune cholangitis)?

Testing for antimitochondrial antibodies is the most useful laboratory procedure in the diagnosis of primary biliary cirrhosis; nevertheless, 5-10% of patients with typical features of primary biliary cirrhosis do not have detectable antimitochondrial antibodies, their condition being referred to as antimitochondrial antibody-negative primary biliary cirrhosis or "autoimmune cholangitis". Uncertainty exists whether antimitochondrial antibody-positive and -negative primary biliary cirrhosis represent distinct entities. We reviewed studies that compared: (i) the clinical, laboratory and histological characteristics of antimitochondrial antibody-positive and -negative primary biliary cirrhosis; (ii) the response to treatment of both conditions; and (iii) the response of autoimmune cholangitis to ursodeoxycholic acid and immunosuppressive therapy. Antimitochondrial antibody-positive and -negative primary biliary cirrhosis were characterized by similar clinical, laboratory and histological abnormalities, clinical course and survival. Antimitochondrial antibody status did not seem to affect the response to ursodeoxycholic acid. At present, the efficacy of therapies for autoimmune cholangitis has not been established in controlled trials. Of 52 patients with autoimmune cholangitis treated with ursodeoxycholic acid in 13 uncontrolled studies, 83% had serum biochemical improvement. Also, a favourable effect of immunosuppressive drugs occurred in 57% of 54 patients with autoimmune cholangitis in 17 uncontrolled studies. Each of these trials included very few patients and most evaluated the effects of treatment on surrogate markers of disease only. No marker that consistently distinguished patients who would respond favourably to ursodeoxycholic acid or immunosuppression was apparent. Consequently, treatment is, at present, empirical. However, ursodeoxycholic acid may be given when histology reveals bile duct lesions, whereas immunosuppressive therapy should probably be reserved for patients exhibiting interface hepatitis.

Antibodies↗
PubMed1999

Primary sclerosing cholangitis in children.

Primary sclerosing cholangitis (PSC), a chronic inflammatory process affecting the extrahepatic and/or medium to large bile ducts, is not rare in children. It has features suggesting an autoimmune pathogenesis, although the mechanism of tissue damage remains unknown. The clinical presentation of childhood primary sclerosing cholangitis is highly variable and frequently without obvious features of cholestasis. Clinical similarity to autoimmune hepatitis is common. Association with chronic colitis is less common than in adults. Cholangiography is essential for the diagnosis and examination of the medium to large intrahepatic ducts is mandatory, as 40% of children lack extrahepatic duct involvement. Histological findings may help to distinguish childhood PSC from autoimmune hepatitis. In children, sclerosing cholangitis may also develop secondary to other disease processes, notably Langerhans histiocytosis, congenital immunodeficiencies and cystic fibrosis. Neonatal sclerosing cholangitis is chronic inflammatory disease of bile ducts which presents initially with neonatal cholestasis; its pathogenesis remains uncertain and may not be the same as for primary sclerosing cholangitis. Effective treatment modalities for childhood PSC remain undetermined. Liver transplantation is required for children who progress to biliary cirrhosis and hepatic decompensation.

Adolescent↗
PubMed1998

The role of surveillance endoscopic retrograde cholangiopancreatography in preventing episodic cholangitis in patients with recurrent common bile duct stones.

BACKGROUND AND STUDY AIMS: Approximately 2-7% of patients who have undergone previous removal of bile duct stones have recurrence often presenting as ascending cholangitis. The aim of this study was to identify the incidence, clinical presentation, and objective findings in this group of patients. Additionally, the effect of surveillance endoscopic retrograde cholangiopancreatography (ERCP) in preventing cholangitis, was studied. PATIENTS AND METHODS: Two thousand and ninety-six patients who underwent ERCP for cholelithiasis were studied with 45 of these patients being identified as having recurrent common bile duct stones. Of the 45, 13 had two or more recurrences without having any obvious predisposing factors. The mean age of the 13 patients was 57 years. The characteristics of 13 patients were reviewed, including sphincterotomy size, liver function tests, and contrast drainage time. RESULTS: All 13 patients with recurrent stones presented with ascending cholangitis. Stones were found to be soft, brown and accompanied by a large amount of sludge. The common bile duct in all 13 patients was noted to be dilated and had notable, widely patent sphincterotomes. There was significant delayed drainage in 77% of these patients. Yearly surveillance ERCPs were performed in the 13 patients, the incidence of acute cholangitis episodes per patient decreased from 2 to 0.6 with a four-year follow-up. CONCLUSION: In a subgroup of patients with multiple common bile duct stone recurrences, annual surveillance ERCP with stone removal decreases the incidence of recurrent episodes of ascending cholangitis as well as its associated morbidity and mortality.

Adult↗
PubMed1990

Association of primary sclerosing cholangitis with HLA-DRw52a.

We sought to determine whether there are specific HLA haplotypes in patients with either primary sclerosing cholangitis or primary biliary cirrhosis. Surprisingly, 100 percent of the 29 patients with primary sclerosing cholangitis had the HLA-DRw52a antigen, which is normally present in 35 percent of the population (relative risk, 109.5; P less than 0.00001). Fifteen of these patients had a single common haplotype: A1,B8,Cw7,DRw17,DQw2,DRw52a. In the remaining 17 patients there was a loss of at least one of these antigens. Of the 15 patients with the common haplotype, 12 also had ulcerative colitis, thereby linking the occurrence of ulcerative colitis in patients with primary sclerosing cholangitis to the presence of this haplotype. Although there was no association in 35 patients between primary biliary cirrhosis and specific HLA haplotypes, there was a significant association of the disease with DRw8 (relative risk, 3.1; P = 0.02). We conclude that the development of primary sclerosing cholangitis involves a strong genetic predisposition. Since the association of primary sclerosing cholangitis with HLA-DRw52a appears to be total, HLA typing should be helpful in differentiating this disease from primary biliary cirrhosis.

Cholangitis, Sclerosing↗
PubMed2000

Effectiveness of ERCP in cholangitis: a community-based study.

BACKGROUND: Although experts have demonstrated the efficacy of endoscopic retrograde cholangiopancreatography (ERCP) in cholangitis, the effectiveness of ERCP in unselected patients has not been measured. The aim was to investigate the clinical impact of ERCP performed at any time and of early ERCP (within 24 hours of admission) in patients with a primary discharge diagnosis of cholangitis. METHODS: A retrospective record review of patients admitted to eight area hospitals with an International Classification of Diseases (ICD)-9 diagnosis consistent with cholangitis was performed. Extracted data included clinical characteristics, ERCP findings, and patient outcome. The associations of ERCP overall and early ERCP with length of stay were examined. Confounding factors including severity of illness, etiology of cholangitis, and hospital type were adjusted for in a multivariate analysis. RESULTS: A total of 116 patients were studied. ERCP was performed in 71 patients with endoscopic therapy administered in 57 (80%). ERCP overall was not associated with any change in length of hospital stay. However, compared with other invasive biliary procedures, ERCP was associated with a shorter hospital stay (median 5 vs. 9.5 days, p = 0.01) and a 36% (95% CI [5%, 57%]) reduction in severity-adjusted length of stay. Patients who had early ERCP had a significantly shorter hospital stay than those who had delayed ERCP (median 4 vs. 7 days, p < 0.005) and early ERCP was associated with a 34% (95% CI [11%, 48%]) reduction in severity-adjusted length of stay. CONCLUSION: Early ERCP may be an effective strategy for shortening the length of stay in patients hospitalized with cholangitis.

Cholangiopancreatography, Endoscopic Retrograde↗
PubMed2002

A possible mouse model for spontaneous cholangitis: serological and histological characteristics of MRL/lpr mice.

AIMS: MRL/Mp-lpr/lpr (MRL/lpr) mice spontaneously develop lymphadenopathy, hypergammaglobulinaemia, serum auto-antibodies, and a generalised auto-immune disease including glomerulonephritis and arthritis, and have been used as a model for the study of systemic lupus erythematosus. Recently, MRL/lpr mice were also reported as a potentially suitable animal model of primary biliary cirrhosis (PBC). The aim of this study was to determine the suitability of MRL/Mp-lpr/lpr (MRL/lpr) mice as an experimental auto-immune-mediated cholangitis model for PBC. METHODS: We investigated the serum hepatobiliary enzymes, histopathological findings, and the target antigen of antimitochondrial antibodies (AMA) in MRL/lpr mice. RESULTS: Serum levels of total bilirubin and hepatobiliary enzymes including alanine aminotransferase (ALT), leucine aminopeptidase (LAP), and gamma-glutamyl transpeptidase (G-GTP) in older-aged (over 20 weeks old) MRL/lpr or MRL/Mp-+/+ (MRL/+) mice were not significantly higher than those in younger (8-12 weeks old) MRL/lpr, MRL/+, or older-aged control mice (C3H/HeJ and BALB/C mice). Histopathologically, 24 of 47 (51%) older-aged MRL/lpr mice showed evidence of cholangitis, compared with two of 20 (10%) younger MRL/lpr mice. Especially, epithelioid granuloma and/or bile duct loss were seen in 11 out of 47 (23%) older-aged MRL/lpr mice, whereas such findings were seen in only one of 20 (5%) younger MRL/lpr mice. None of the MRL/+, C3H/HeJ, and BALB/C mice developed cholangitis. The target antigens of AMA were not pyruvate dehydrogenase complex but 2-oxoglutarate dehydrogenase complex and/or branched-chain oxo-acid dehydrogenase complex as confirmed by immunoblotting. There was no significant correlation between the presence of AMA and severity of histological lesions in older-aged MRL/lpr mice, and there were no significant differences in these biochemical data, the proportion of mice with portal inflammation, cholangitis and AMA between male and female MRL/lpr mice. CONCLUSION: Although several clinical features were incompatible with PBC, the serological and histopathological features of MRL/lpr mice indicate that these mice can be used as an experimental immune-mediated cholangitis model for PBC.

Animals↗