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PubMed1993

Distribution of B lymphocytes in nonsuppurative cholangitis in primary biliary cirrhosis.

Primary biliary cirrhosis is characterized by the immune-mediated, nonsuppurative destruction of intrahepatic small bile ducts, with significant T-cell involvement. To date, B cells in livers of primary biliary cirrhosis patients have been ignored in immunopathological evaluations of nonsuppurative cholangitis. This study aimed to correlate the distribution of activated T and B cells with the histopathology of nonsuppurative cholangitis in primary biliary cirrhosis, with emphasis on B cell infiltration. The distribution of activated T and B cells and the histopathology in primary biliary cirrhosis were heterogeneous in bile ducts showing nonsuppurative cholangitis; they were also heterogeneous in various parts of a given bile duct. Although activated T cells were significantly involved in the occurrence of nonsuppurative cholangitis, B cell-predominant reaction or cholangitis and B-cell infiltration into the biliary epithelial layer were also prominent in some nonsuppurative cholangitis. Many bile ducts with nonsuppurative cholangitis of various histological types showed intermixed activated T- and B-cell infiltration. These findings indicate that immune mechanism(s) involving B cells and, probably, humoral immunity are at work in the occurrence of nonsuppurative cholangitis and the destruction of bile ducts in primary biliary cirrhosis. Cell-to-cell interactions of activated T and B cells should therefore be evaluated in livers of patients with primary biliary cirrhosis.

Antibodies↗
PubMedDate not supplied

Counts of bacteria and pyocites of choledochal bile in controls and in patients with gallstones or common bile duct stones with or without acute cholangitis.

BACKGROUND/AIMS: The number of colonies of bacteria and the number of pyocites present per ml of choledochal bile was studied. PATIENTS AND METHODS: There were 42 controls, 100 patients with symptomatic gallstones, 42 patients with common duct stones without cholangitis and 24 patients with common duct stones and acute cholangitis. RESULTS: Control subjects had no bacteria present at gallbladder bile. Only 3% of patients with gallstones had more than 10(5) colonies per ml which increased to 36% in patients with common duct stones without cholangitis and to 84% among patients with acute cholangitis (p < 0.001). There were more polybacterial flora among patients with acute cholangitis and anaerobic bacteria were not seen in patients with gallstones. Patients with acute cholangitis had significantly more pyocites present at choledochal bile. CONCLUSION: There is a direct correlation between the number of colonies present per ml of choledochal bile and the severity of biliary tract disease. Patients with acute cholangitis had significantly more pyocites present at choledochal bile compared to gallstones or patients with CBD stones without cholangitis.

Acute Disease↗
PubMed1997

Concentrations of bile and serum endotoxin and serum cytokines after biliary drainage for acute cholangitis.

Endotoxin contributes to cholangitis. We measured concentrations of bile and serum endotoxin and serum cytokines after biliary drainage for obstructive jaundice with or without acute cholangitis. Patients who underwent percutaneous transhepatic cholangiodrainage (PTCD) in 1995 were classified as having acute cholangitis (group A; n = 11), having a history of acute cholangitis (group B; n = 5), or not having a history of acute cholangitis (group C; n = 13). Bile endotoxin was positive (above the cut-off value) in all patients in groups A and B, and in five patients in group C. The mean concentration of bile endotoxin was significantly higher in groups A and B than in group C. After PTCD, the bile endotoxin level decreased more slowly in group A than in the other groups. Before PTCD, the mean serum levels of IL-1 receptor antagonist (IL-1ra), IL-6, and IL-8 were higher in group A than the other groups. The serum levels of IL-1ra and IL-6 before PTCD were significantly higher when the acute cholangitis was more severe. The mean serum levels of cytokines increased just after PTCD and then decreased. In group A, the serum level of IL-6 at 5 h after PTCD was significantly correlated to the endotoxin level in bile at this time. Increases in cytokines may participate in the pathophysiological changes of acute cholangitis. Biliary drainage for acute cholangitis causes improvement by decreasing the bile endotoxin level addition to decreasing bile-duct pressure, thereby preventing excess production of inflammatory cytokines.

Acute Disease↗
PubMedDate not supplied

Cholangitis after endoscopic biliary drainage for hilar lesions.

BACKGROUND/AIMS: Cholangitis is a morbid complication following endoscopic biliary drainage. This study was undertaken to determine the subgroup of patients more likely to develop cholangitis after endoscopic drainage and to determine how to manage this complication. METHODOLOGY: Although we have used transhepatic biliary drainage as the procedure of choice for relieving obstructive jaundice, we recently encountered ten patients who underwent endoscopic drainage before referral. RESULTS: Three patients with a hilar obstruction developed cholangitis and cholangiolytic liver abscesses after endoscopic drainage, while seven with a distal obstruction did not develop cholangitis following drainage. Thus, the incidence of cholangitis in patients with a hilar obstruction (100%) was significantly higher than that (0%) in patients with a distal obstruction (p = 0.008). Cholangitis in the three patients was managed with percutaneous drainage without mortality, although this procedure was technically difficult because of the presence of collapsed intrahepatic bile ducts following the endoscopic drainage. CONCLUSIONS: Hilar lesions present a higher risk of cholangitis after endoscopic biliary drainage than distal lesions. Hence, percutaneous, rather than endoscopic, drainage is indicated for such lesions. Percutaneous drainage is the procedure of choice for the management of cholangitis after endoscopic drainage.

Aged↗
PubMed1997

Treatment of acute cholangitis due to choledocholithiasis in elderly and younger patients.

OBJECTIVE: To evaluate management strategies for acute cholangitis in elderly patients (age, > or = 80 years). DESIGN: Nonrandomized control trial. SETTING: A university hospital. PATIENTS: Patients (n = 191) who underwent urgent biliary drainage for acute cholangitis due to choledocholithiasis. Thirty-seven patients were elderly, and 154 were younger (age, < 80 years). INTERVENTIONS: Surgical (8 elderly and 48 younger patients), percutaneous transhepatic (11 elderly and 47 younger patients), or endoscopic drainage (18 elderly and 59 younger patients). MAIN OUTCOME MEASURES: Clinical features of acute cholangitis and outcomes of biliary drainage. RESULTS: The elderly patients had higher incidences of septic shock or mental confusion (acute severe cholangitis)(43.2%) and concomitant diseases (81.1%) than the younger patients (25.3% and 42.9%, respectively). The elderly patients had significantly greater morbidity (37.8%) and mortality (10.8%), compared with the younger patients (16.9% and 3.2%, respectively). Mortality was 18.8% in elderly patients with severe cholangitis and 4.8% in those with nonsevere cholangitis. In the elderly patients, endoscopic drainage yielded lower morbidity (16.7%) and mortality (5.6%) than surgical (87.5% and 25.0%, respectively) and percutaneous drainage (36.4% and 9.1%, respectively). No complications occurred after endoscopic nasobiliary drainage without sphincterotomy. CONCLUSIONS: Elderly patients with acute cholangitis have high incidence of severe disease and concomitant medical problems. They should undergo endoscopic biliary drainage, especially nasobiliary drainage without sphincterotomy, because of its safety and effectiveness.

Acute Disease↗
PubMed1989

Different immune mechanisms leading to autoimmunity in primary sclerosing cholangitis and autoimmune chronic active hepatitis of childhood.

Children with primary sclerosing cholangitis or autoimmune chronic active hepatitis have similar high levels of immunoglobulin G and non-organ-specific autoantibodies and may have similar histological features. To investigate a possible immunopathogenesis of primary sclerosing cholangitis, we have studied a series of regulatory and/or effector immune mechanisms in eight children with primary sclerosing cholangitis, comparing them to 14 children with autoimmune chronic active hepatitis and 24 healthy children as controls. Antibodies to a liver membrane protein preparation were found in all children with autoimmune chronic active hepatitis tested and in seven of eight with primary sclerosing cholangitis, whereas antibodies against the hepatic asialoglycoprotein receptor were present in three of six patients with autoimmune chronic active hepatitis and in two of the eight with primary sclerosing cholangitis. Lymphocyte cytotoxicity values to autologous hepatocytes were similarly elevated in primary sclerosing cholangitis (median: 50%; range: 38 to 83%) and in autoimmune chronic active hepatitis (median: 52%; range 37 to 87%) compared to controls (median: 8%; range: 0 to 27%) (p less than 0.01 for both). In contrast, T suppressor cell number and function were normal in patients with primary sclerosing cholangitis (median: 23%; range: 19 to 28%; and median: 54%; range: 44 to 61%), but significantly decreased in patients with autoimmune chronic active hepatitis (median: 15%; range: 9 to 21%; and median: 9%; range: -40 to +21%) when compared to controls (median: 24%; range: 16 to 29%; and median: 53%; range: 8 to 77%) (p less than 0.01 for both).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2000

Liver involvement in patients operated for ulcerative colitis, with special reference to the association of cholangitis with colorectal dysplasia and carcinoma.

This study classified liver changes found in patients undergoing proctocolectomy for ulcerative colitis and examined whether patients with cholangitis have an increased risk of colorectal dysplasia and carcinoma. The patients were 152 who underwent liver biopsy during surgery for ulcerative colitis. Prior surveillance colonoscopy specimens and operative liver and proctocolectomy specimens were examined histologically. Patients with dysplasia or carcinoma in colorectal specimens were pair-matched to patients without such neoplasia. Sixteen (10.5%) patients had histological features consistent with small-duct primary sclerosing cholangitis on liver biopsy, five of them showing normal liver function values. Of the 152 patients 4 were found to have colon carcinoma (2.6%) and another 4 low-grade dysplasia (2.6%) either upon colonoscopy or in colectomy specimens. The median duration of the colitis in the 8 patients with colorectal neoplasia was 12 years (range 2-29) and in the other 142 patients 4 years (0.1-33; P=0.007). The prevalence of primary sclerosing cholangitis (PSC) or cholangitis was 50% in cases with colorectal neoplasia and 13% in pair-matched controls without colorectal neoplasia. In this selected group of patients operated on for ulcerative colitis the prevalence of histological cholangitis was thus higher than that of PSC in previous epidemiological studies. In addition, the prevalence of PSC or cholangitis was much higher in cases with colorectal neoplasia than in pair-matched controls without colorectal neoplasia. Our results support the view that cholangitis constitutes an additional risk factor underlying colorectal dysplasia or carcinoma.

Adolescent↗
PubMed1999

Liver transplantation for primary sclerosing cholangitis.

Although the development of interventional radiology and biliary surgical techniques has prolonged the survival time of patients with primary sclerosing cholangitis, liver transplantation remains the only effective treatment for patients with primary sclerosing cholangitis with liver cirrhosis. Several prognostic survival models have been establised for this disease, and the efficacy of actual liver transplantations has been reported in comparison with these survival models. One- and 5-year actuarial patient survivals after liver transplantation for primary sclerosing cholangitis were shown to be greater than and approximately equal to 90%, respectively. An association with cholangiocarcinoma is the most adverse factor affecting survival after liver transplantation for primary sclerosing cholangitis, while the association of inflammatory bowel disease or previous bili-ary surgery does not adversely affect the outcome of the liver transplantation. Recurrent sclerosing cholangitis is an important issue for posttransplant patients with primary sclerosing cholangitis, and occurs in 10%-20% of such patients. Although our understanding of recurrent sclerosing cholangitis is still in the early stages, its potential occurrence indicates the need for a longer follow-up period after liver transplantation.

Bile Duct Neoplasms↗
PubMed2003

Outcome of patients undergoing liver transplantation for primary sclerosing cholangitis.

PURPOSE: The purpose of this study was to determine the outcome of patients with inflammatory bowel disease who underwent liver transplantation for primary sclerosing cholangitis. METHODS: All patients who underwent liver transplantation for primary sclerosing cholangitis at our institution were identified. A review of patients' hospital and office charts was performed; all patients were then contacted, and a detailed survey was administered by telephone. RESULTS: Sixty-nine patients were identified. There were 53 males (76.8 percent) and 16 females, with a mean age of 45.3 (+/- 13.3) years. Fifty-two (75.4 percent) of the 69 patients had documented inflammatory bowel disease; of these, 40 had ulcerative colitis (76.9 percent), 11 had Crohn's disease, and 1 had indeterminate colitis. Thirty-one patients (60 percent) were diagnosed with inflammatory bowel disease before primary sclerosing cholangitis, with a mean interval to diagnosis of primary sclerosing cholangitis of 10.8 (+/- 10.3) years. Seven patients had both diagnoses made at roughly the same time, and 14 patients initially were diagnosed with primary sclerosing cholangitis and subsequently were found to have inflammatory bowel disease, with a mean interval of 5.2 (+/- 4.4) years; 5 (35.7 percent) of those 14 patients were only diagnosed with inflammatory bowel disease after their liver transplant. The mean time from diagnosis of primary sclerosing cholangitis to liver transplantation was 6.1 (+/- 4.9) years. Since their transplant, 30.8 percent of patients rated their colitis as worse, 38.5 percent felt it was unchanged, and 30.8 percent felt that their colitis was better controlled. Eight (15.4 percent) of the 52 patients with inflammatory bowel disease denied having any knowledge of an increased risk of colorectal neoplasia. Four patients have required colectomy for colorectal neoplasia after liver transplantation, at a mean of 4.7 years after transplantation. Of the patients with inflammatory bowel disease, 42 (80.1 percent) had at least 1 posttransplant surveillance colonoscopy. Eight of the remaining ten patients had a colectomy, leaving only two patients (3.8 percent) who had not been surveyed. However, only 32 (61.5 percent) of the patients with inflammatory bowel disease have been on a surveillance regimen that would approximately conform to current screening recommendations. CONCLUSIONS: The activity of inflammatory bowel disease after transplantation is highly variable. Patients appeared to lack knowledge of their increased risk for colorectal neoplasia. Colorectal cancer is an uncommon but important complication in patients after liver transplantation for primary sclerosing cholangitis, and ongoing surveillance is required. Patients may require education to increase their awareness of the cancer risk and compliance with surveillance.

Chi-Square Distribution↗
PubMed2006

Placement of a metallic stent across the main duodenal papilla may predispose to cholangitis.

BACKGROUND: Cholangitis is a major complication after metallic stent placement to treat biliary obstruction, and it may impair quality of life. Whether transpapillary stent placement contributes cholangitis is still controversial. OBJECTIVES: The present study aimed to determine risk factors for acute cholangitis after metallic biliary stent placement. DESIGN: Retrospective cases series. PATIENTS: A total of 108 patients with malignant biliary obstruction were treated with metallic stents, resulting in 12 cases of cholangitis. MAIN OUTCOME MEASUREMENTS: A multiple logistic regression model was performed with clinical parameters, including stent placement across the main duodenal papilla. RESULTS: By multiple logistic regression model, age; lower location; and Wallstent, Ultraflex stent, and covered stent were negatively associated with cholangitis. Restenosis and placement across the main duodenal papilla were positively associated with the occurrence of cholangitis. Transpapillary stent placement was the most significant risk factor. In this logistic model, the area under a receiver operating characteristics curve was computed as 0.92: sensitivity, 0.92 (95% confidential interval (CI), 0.62-1.00); specificity, 0.86 (95% CI, 0.78-0.93); positive predictive value, 0.46 (95% CI, 0.26-0.67); and negative predictive value, 0.99 (95% CI, 0.94-1.00). CONCLUSIONS: Disruption of the sphincter mechanism by transpapillary placement may be the most important etiologic factor in the propensity for cholangitis after metallic stent placement for malignant biliary obstruction.

Acute Disease↗
PubMed2003

The intussusception antireflux valve is ineffective for preventing cholangitis in biliary atresia: a prospective study.

PURPOSE: Cholangitis after Kasai portoenterostomy for biliary atresia (BA) strongly influences outcome, and an intussusception antireflux valve (IAV) in the Roux-en-Y limb has been advocated for the prevention of cholangitis without definitive evidence. The authors report a prospective study to evaluate the effectiveness of IAV in BA. METHODS: Twenty-one consecutive patients with uncorrectable-type BA were the subjects for this study. IAV was included based on informed parental request to give 2 groups: IAV- (n = 11) and IAV+ (n = 10). The incidence/duration of episodes of cholangitis (temperature > 38 degrees C, elevated serum bilirubin level, and leukocytosis) during the first 6 months after Kasai were statistically compared between the 2 groups (P >.05 was regarded as not significant). RESULTS: Technical variations in surgical technique were minimized because the same surgeon supervised all procedures. There were no significant differences between the 2 groups for age and weight at the time of Kasai, mean size of bile ductules at the porta hepatis, and degree of liver cirrhosis present. Protocols for antibiotic and steroid therapy were the same for both groups. The total number of patients who had cholangitis in the IAV- group was 6 of 11 and 5 of 10 in the IAV+ group (P = NS). The total number of episodes of cholangitis was 16 in the IAV- group versus 17 in the IAV+ group (P value, not significant). The mean duration of an episode of cholangitis (number of days per number of episodes) was 12 +/- 6 days in the IAV- group versus 11 +/- 7 days in the IAV+ group (P value, not significant). CONCLUSIONS: IAV does not make any significant impact on the incidence or duration of cholangitis in the early post-Kasai period.

Anastomosis, Roux-en-Y↗
PubMed1998

Cholangitis in viral disease.

This review of biliary manifestations of viral diseases includes aspects of morphologic diagnosis, therapeutic implications, prognostic effect, and natural history. The viral causes of cholangitis are reviewed, with subclassification on the basis of primary hepatic versus systemic infections and immune competence of the host. Special attention is given to the histopathologic and clinical features of viruses affecting the biliary tree. Among hepatotropic viruses, hepatitis C more frequently is associated with cholangitis than is hepatitis B. In both hepatitis B and hepatitis C, the lymphocytic cholangitis duct damage is reversible and does not adversely influence the course of disease or response to therapy. Hepatitis A and hepatitis E, despite causing clinical cholestasis, do not result in severe cholangitis. The effect of systemic viruses on the biliary tree is primarily dependent on the status of the host immune system. Infants and severely immunosuppressed patients (such as those who have undergone liver transplantation) are at risk for cytomegalovirus cholangitis, whereas patients with late-stage acquired immunodeficiency syndrome (AIDS) are at risk for cholangitis due to numerous organisms. Overall, cholangitis attributable to viral disease encompasses a wide spectrum of clinicopathologic scenarios, depending on the etiologic virus and the immune competence of the host.

AIDS-Related Opportunistic Infections↗
PubMed1990

Acute cholangitis.

Cholangitis is an infection of the biliary ductal system that results from the combination of bactibilia and biliary obstruction. Choledocholithiasis has been the leading cause of cholangitis. However, in recent years, especially at tertiary referral centers, nonoperative biliary manipulations, often in patients with unresectable malignancies, have become the most common cause of cholangitis. As a result, the complete triad of fever and chills, jaundice, and abdominal pain, as originally described by Charcot, is now seen less frequently. Most patients still have leukocytosis and abnormal liver function tests, but many patients with indwelling tubes may develop cholangitis without significant jaundice. E. coli, Klebsiella species, and the enterococci remain the most frequently isolated organisms, and anaerobes including Bacteroides fragilis are recovered in 15% to 30% of patients. However, Enterobacter and Pseudomonas species, as well as yeasts, are now being isolated more frequently from patients with indwelling tubes, who often have been treated previously with antibiotics. Computed cholangiography usually is necessary to determine the cause and site of biliary obstruction. In the majority of patients with cholangitis, cholangiography can be delayed until the patient has been afebrile for a minimum of 24 to 48 hours. Initial therapy includes bowel rest, intravenous fluids, and antibiotics. Many antibiotic regimens are now available to cover the gram-negative aerobes, the enterococcus, and the anaerobes that are likely to be causing the biliary infection. The combination of a penicillin and an aminoglycoside has been the gold standard. However, recent studies suggest that the newer broad-spectrum penicillins provide adequate therapy for these patients. Only a small percentage (5%-10%) of patients with toxic cholangitis require emergency biliary decompression. The choice of percutaneous or endoscopic drainage should be made on the basis of the presumed site and cause of obstruction as well as local expertise. The nature of the biliary obstruction may be the most important determinant of outcome. At present, patients with end-stage malignant obstruction account for most of the deaths, whereas approximately 95% of patients survive an episode of cholangitis.

Acute Disease↗
PubMed2000

Serum autoantibodies in patients with primary sclerosing cholangitis.

BACKGROUND/AIM: Primary sclerosing cholangitis is a chronic cholestatic syndrome with a presumed autoimmune basis frequently associated with inflammatory bowel disease. The aim of this study was to determine the profile and significance of serum autoantibodies in patients with primary sclerosing cholangitis. METHODS: Serum samples taken from 73 untreated patients (32 female and 41 male, median age 45 years) with well-defined primary sclerosing cholangitis, and from 75 healthy age- and sex-matched controls were assayed for 20 different autoantibodies. RESULTS: Of 73 patients, 71 (97%) were positive for at least 1 autoantibody; whereas 59/73 patients (81%) were positive for > or =3 antibodies. Patients with primary sclerosing cholangitis had a significantly greater rate of positivity than controls for antinuclear, anticardiolipin, antineutrophil cytoplasmic, and antithyroperoxidase antibodies as well as rheumatoid factor. The rate of positivity and serum levels of any of these 20 autoantibodies were not significantly different between patients with primary sclerosing cholangitis and inflammatory bowel disease and those without inflammatory bowel disease. Anticardiolipins were the single group of antibodies that had a significant correlation with the Mayo risk score (r=0.49, p<0.001) and histologic stage of disease (r=0.30, p<0.01). CONCLUSIONS: Primary sclerosing cholangitis is associated with a high proportion of non-organ specific autoantibodies. Anticardiolipin antibodies appear to be related to the severity of primary sclerosing cholangitis and may be a useful prognostic marker.

Adult↗
PubMed1997

Preferential V beta3 usage by hepatic T lymphocytes in patients with primary sclerosing cholangitis.

BACKGROUND/AIMS: Primary sclerosing cholangitis and primary biliary cirrhosis are two biliary destructive disorders characterized by prominent T lymphocyte infiltrates in areas of portal destruction. The specificity of the T cell is determined by the T cell receptor for antigens. The aim of this study was to investigate the preference by which certain V alpha and V beta gene segments are expressed by peripheral and hepatic T cells in primary sclerosing cholangitis and primary biliary cirrhosis. METHODS: The usage of the alpha/beta T cell receptor (TcR) V gene of liver infiltrating lymphocytes and peripheral blood lymphocytes from 12 primary sclerosing cholangitis patients, 10 primary biliary cirrhosis patients and healthy controls was investigated, using alpha/beta TcR V gene product-specific monoclonal antibodies. HLA class II antigen typing with genomic typing technique was done in 11/12 primary sclerosing cholangitis patients. RESULTS: A significant difference between the studied groups of patients was an increase in the expression of V beta3+ T cells in liver tissue from patients with primary sclerosing cholangitis compared to patients with primary biliary cirrhosis and healthy controls (p<0.01). No significant differences were found in the peripheral blood between the three groups. Furthermore, no relation between the different TcR V alpha/beta cells and histological staging and class II antigen association was observed. CONCLUSIONS: Predominant TcR V beta3 gene usage in liver tissue in primary sclerosing cholangitis may indicate the presence of a specific antigen in this tissue with the capacity of selectively driving T cells, utilizing the V beta3 gene segment product, in primary sclerosing cholangitis patients.

Adult↗
PubMed2004

[Early cholangitis complicating percutaneous biliary drainage].

PURPOSE: To evaluate the prevalence, severity and outcome of cholangitis within two months after percutaneous transhepatic biliary drainage. MATERIALS AND METHODS: One hundred and nineteen patients with obstructive jaundice but no septic symptoms underwent biliary drainage for biliary decompression. Biliary obstruction was malignant in 87 patients and benign in 32 patients. Clinical and biological criteria were developed to confirm and grade the cholangitis. Based on these, the prevalence, severity and outcome of cholangitis was determined and analysed through review of clinical records. RESULTS: All patients had successful biliary drainage and internal drainage. Nine patients (7.5%) developed cholangitis after biliary drainage, three (2.5%) being of the severe type. Of these, 8 had malignant obstruction. All three patients with severe cholangitis had malignant obstruction. All patients with cholangitis except one (0.8%) responded to antibiotic and supportive therapy. CONCLUSION: Severe cholangitis occurring within two months following PBD for obstructive jaundice was uncommon. It tended to occur in patients with malignant obstruction. Although most responded favorably to medical treatment, post procedural sepsis must be handled with great care since it is life-threatning.

Adult↗
PubMed2003

Different immunosuppressive regimens and recurrence of primary sclerosing cholangitis after liver transplantation.

Primary sclerosing cholangitis (PSC) is the fourth leading diagnosis in liver transplant recipients in the United States. The disease is known to recur in 15% to 30% of liver transplant recipients. We set out to investigate how different immunosuppression regimens affected natural history of PSC after liver transplantation at our center. We reviewed records of all patients who underwent a liver transplantation at our institution in between 1988 and 2000 and had a diagnosis of PSC at the time of liver transplantation. Primary sclerosing cholangitis recurred in 15 of 71 patients (21.1%) who had complete records and survived more than 30 days after liver transplantation. Although recurrence of primary sclerosing cholangitis was most often seen (but not statistically significantly so) in patients who received maintenance corticosteroids, the time to recurrence was not significantly different between those who were treated with maintenance, those who were not successfully weaned, and those who successfully weaned off corticosteroids within 3 months after liver transplantation. Orthoclone (OKT3) therapy (Ortho-Biotech, Inc., Raritan, NJ) was associated with a higher risk of primary sclerosing cholangitis recurrence (29% versus 10%, P <.05). Recurrence was not influenced by immunosuppression with either cyclosporine or tacrolimus. Coexistent inflammatory bowel disease was a cause of failure to wean off corticosteroids, was associated with a shorter time to recurrence of sclerosing cholangitis, and was responsible for significant comorbidity (colon cancer in 7.3%). Primary sclerosing cholangitis recurrence is commonly seen after liver transplantation. More immunosuppression seems to be detrimental to the outcome of our patients with sclerosing cholangitis: use of OKT3 was associated with a greater incidence of recurrence. Length of corticosteroid use did not affect timing or risk of recurrence, and because it has been proven that early corticosteroid withdrawal after liver transplantation is beneficial, we continue to recommend this practice.

Adult↗
PubMed1991

Cholangiocarcinoma complicating primary sclerosing cholangitis.

Cholangiocarcinoma is more likely to develop in patients with primary sclerosing cholangitis. Our aims were to describe the clinical presentation, course, and management of patients afflicted with both cholangiocarcinoma and primary sclerosing cholangitis and to estimate the prevalence of cholangiocarcinoma in patients with primary sclerosing cholangitis. A retrospective analysis was conducted of 30 patients with both primary sclerosing cholangitis and cholangiocarcinoma managed at our institution during an 8-year period. Development of cholangiocarcinoma was heralded by rapid clinical deterioration with jaundice, weight loss, and abdominal discomfort. Cholangiocarcinoma complicating primary sclerosing cholangitis often was detected at an advanced tumor stage, which precluded effective therapy, and overall median survival was 5 months. Earlier recognition and treatment of cholangiocarcinoma in such patients will be necessary to increase survival rates. Seventy patients with primary sclerosing cholangitis were followed prospectively in a clinical trial of medical therapy for an average of 30 months. Twelve patients died and five were found at autopsy to have cholangiocarcinoma. The potential for cholangiocarcinoma to develop in patients with primary sclerosing cholangitis may indicate that liver transplantation should be considered earlier in the course of the disease.

Adenoma, Bile Duct↗