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Pediatrics: General Medicine > Gastroenterology
Primary Sclerosing Cholangitis
Article Last Updated: Jun 26, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 10  
Author: Lynette Gillis, MD, Assistant Professor, Division of Pediatric Gastroenterology, Hepatology & Nutrition, Vanderbilt University
Lynette Gillis is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, and American Gastroenterological Association
Coauthor(s):
Julia Anderson, MD, Fellow, Division of Pediatric Gastroenterology, Vanderbilt Children's Hospital
Editors: Robert Baldassano, MD, Director, Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology and Nutrition, Associate Professor, Department of Pediatrics, The Children's Hospital of Philadelphia, University of Pennsylvania; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; David Piccoli, MD, Chief, Division of Gastroenterology and Nutrition, Department of Pediatrics, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine; Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, State University of New York, Downstate Medical Center College of Medicine; Professor of Clinical Pediatrics, St George's University School of Medicine; Distinguished Lecturer, New York Medical College, School of Public Health; Chair and Consulting Staff, Department of Pediatrics, Long Island College Hospital; Carmen Cuffari, MD, Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine
Author and Editor Disclosure
Synonyms and related keywords:
primary sclerosing cholangitis, PSC, liver disease, cirrhosis, portal hypertension, liver failure, end-stage liver disease, hepatomegaly
Background
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology that is recognized increasingly in children. The diagnosis is based on a combination of clinical features and cholestatic biochemical profile, along with typical cholangiographic abnormalities, and confirmed by liver histology findings. In the absence of underlying bile duct abnormalities, a generalized beading and stenosis of the intrahepatic and extrahepatic biliary tree characterize PSC. PSC is usually progressive, leading to cirrhosis, portal hypertension, and liver failure. Effective medical treatment modalities for childhood PSC are undetermined. Liver transplantation remains the only effective therapeutic option for patients with end-stage liver disease from PSC.
Pathophysiology
The mechanisms responsible for the development of PSC are unknown. The relationship with PSC and inflammatory bowel disease (IBD) offers several clues. The biliary injury may be initiated by an immune-mediated destruction of the hepatobiliary tract that is perhaps caused by transient infection or the absorption of bacterial byproducts in genetically predisposed individuals with colonic disease.
Frequency
United States
PSC is frequently seen in association with IBD. Seventy to eighty percent of patients who have PSC have coexistent IBD. PSC may precede the onset of ulcerative colitis (UC) or may develop following proctocolectomy. Conversely, 2.5-7.5% of patients with IBD develop PSC.
Sex
A male predominance appears to exist in PSC.
Age
Peak incidence of PSC occurs in the third and fourth decades of life. PSC has also been described in infancy.
History
The clinical presentation in children with primary sclerosing cholangitis (PSC) is highly variable and frequently lacks the obvious features of cholestasis. Patients may be asymptomatic with elevated liver function test findings or hepatomegaly, prompting further workup for PSC. Patients may also present with fatigue, pruritus, fever of unknown origin, intermittent jaundice, or weight loss. Some patients present with the stigmata of chronic liver disease and cirrhosis. The onset and progression tend to be insidious. Modes of presentation include the following:
- Asymptomatic patients present with incidental finding of hepatomegaly on examination or abnormal liver function test (LFT) results.
- Symptomatic patients may present with nonspecific complaints, including fatigue, pruritus, abdominal pain, fevers, weight loss, and intermittent jaundice.
- Patients with cholestasis present with complications of cholestasis, including pruritus, cholangitis, and fat malabsorption.
- Patients with cirrhosis present with complications of portal hypertension, including ascites, variceal bleeding, and splenomegaly.
Physical
Findings on physical examination vary with the degree of disease activity at the time of initial presentation. Approximately 55% of patients have hepatomegaly and 30% have splenomegaly at presentation.
Causes
PSC is a progressive disorder of unknown etiology. Bacteria, toxins, viral infections, and immunologic and genetic factors have been proposed as etiologic agents.
- The high degree of association of PSC with IBD suggests a common pathogenetic mechanism; however, no causal relationship has been established. An abnormal colonic mucosal barrier may lead to portal bacteremia or abnormal absorption of toxic metabolites or bile acids.
- Rats with experimental small-bowel bacterial overgrowth develop a hepatobiliary injury similar to PSC. The hepatobiliary injury is mediated by activation of Kupffer cells and by release of cytokines, such as tumor necrosis factor alpha.
- Reovirus and cytomegalovirus (CMV) are possible etiologic agents; PSC is analogous to a reovirus-induced cholestasis in mice.
- Immunologically mediated damage to the biliary tree remains the most likely etiology of PSC.
- A high prevalence of the perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) is seen in PSC and UC.
- Autoimmune disorders are more frequent in patients with PSC than in patients with IBD without liver disease; 25% of patients with PSC have at least one autoimmune disorder outside of the liver and colon.
- In children, PSC is commonly associated with markers suggestive of an autoimmune process. Some patients have elevated levels of circulating immune complexes, immunoglobulins, and autoantibodies that are not organ specific. Histologic and clinical overlap (ie, overlap syndrome) with autoimmune hepatitis may exist.
- The close association between PSC and various human leukocyte antigen (HLA) haplotypes is well established.
- An increased frequency of HLA-B8 and HLA-DR3 is observed in patients with PSC. HLA-B8 is also associated with other autoimmune disorders.
- These lend support to the theory that immunologic and genetic mechanisms may be involved in the pathogenesis.
- Significant alcohol consumption is never advisable in patients with chronic liver disease. Alcohol consumption has been shown to be an independent risk factor for the development of cholangiocarcinoma in patients with PSC.
Autoimmune Chronic Active Hepatitis
Cholestasis
Histiocytosis
Human Immunodeficiency Virus Infection
Other Problems to be Considered
Chronic hepatitis
Infectious hepatitis
Idiopathic autoimmune hepatitis (can coexist with PSC, termed overlap syndrome)
Autoimmune Hepatitis Causes of sclerosis secondary to bile duct abnormalities
Choledocholithiasis
Cholangiocarcinoma
Biliary Trauma
Congenital anomalies of the biliary tract
Lab Studies
- Liver function tests
- The most common abnormality is an elevated alkaline phosphatase or g-glutamyltransferase (GGT) level.
- Serum transaminase levels may be normal or elevated to several times normal.
- The serum bilirubin level is elevated in advanced stages of primary sclerosing cholangitis (PSC).
- Results from hepatic synthetic function tests (eg, serum albumin, prothrombin time [PT]) become abnormal with advanced disease activity.
- Serum cholylglycine is often markedly elevated, out of proportion to the elevation of serum bilirubin.
- Immunoglobulin G (IgG) and immunoglobulin M (IgM) levels are elevated in 48% and 80% of cases of PSC, respectively. The serum globulin fraction may also be elevated in some patients with PSC.
- p-ANCAs have been found to be present in 60-82% of patients with PSC but lack diagnostic specificity for PSC; frequency in UC is similar. Assessment of the target antigens for ANCA (eg, catalase, alpha-enolase, lactoferrin) does not significantly contribute to their clinical importance. The presence of ANCA is associated with a more severe course of autoimmune liver disease. The anti–Saccharomyces cerevisiae antibody is also found in some patients with PSC, independent of IBD status.
- A serum carbohydrate antigen 19-9 (CA 19-9) level greater than 100 U/mL has 75% sensitivity and 80% specificity in identifying PSC patients with cholangiocarcinoma.
Imaging Studies
- Magnetic resonance cholangiography
- Peripheral wedge-shaped areas of high T2 signal intensity and dilatation of bile ducts are characteristic magnetic resonance (MR) findings in PSC.
- Pathologic correlation of magnetic resonance cholangiography (MRC) findings suggests that these features may be related to underlying perfusion changes and bile duct inflammation.
- The overall diagnostic accuracy of MRC in patients with PSC is 90%, compared to 97% for endoscopic retrograde cholangiography (ERCP) or percutaneous transhepatic cholangiography (PTC).
- The advantages of MRC include less risk for complications as compared with ERCP. In addition, MRC has the advantage of visualizing bile ducts proximal to obstructed areas.
- Ultrasonography
- Ultrasonography may reveal intrahepatic and extrahepatic ductal dilatation, increased echogenicity and heterogeneity observed with cirrhosis, and splenomegaly and ascites observed with portal hypertension.
- Ultrasonographic findings may be normal in as many as 50% of patients.
Procedures
- Endoscopic retrograde cholangiopancreatography
- Cholangiography remains the criterion standard for establishing the diagnosis of PSC.
- Cholangiography demonstrates irregularly distributed areas of segmental bile duct fibrosis interposed by areas of saccular dilation that result in the characteristic appearance of beading. As many as 40% of children with PSC lack extrahepatic duct involvement at the time of diagnosis. Serial cholangiograms have shown that, in most patients with disease limited to the intrahepatic ducts, the disease progresses to involvement of the extrahepatic ducts.
- Therapeutic interventions, such as dilation of strictures or placement of an endoprosthesis, can be performed during ERCP, but they carry an increased complication rate. A role may exist for short-term stenting for symptomatic dominant strictures in PSC, with a decrease in cholestatic complaints and improvement of the cholestatic biochemical profile (alkaline phosphatase, GGT, conjugated bilirubin) for several years.
- Liver biopsy
- Certain liver histologic findings are highly suggestive of PSC. More often, liver biopsy findings are nonspecific and infrequently diagnostic. Nevertheless, liver histology remains important to exclude other causes of chronic cholestasis and to stage the disease.
- Sampling variability exists in needle or core liver biopsies secondary to the heterogeneous distribution of biliary lesions.
Histologic Findings
A wide range of histologic findings exists in PSC. Nonspecific features include a periductal concentration of mononuclear cells and ductular proliferation. Less specific liver histology can present with a picture resembling chronic active hepatitis. One series of childhood cases described consistent periportal copper-associated protein (orcein stain) positivity. The most characteristic findings of PSC are periductal fibrosis with inflammation, bile duct proliferation, and ductopenia. This pathognomonic fibro-obliterative cholangiopathy has been occasionally observed in children with PSC.
Staging
The hepatic progression of PSC is divided into 4 histologic stages. These stages are used to document histologic progression and may help evaluate treatment effect in clinical trials. At present, these stages have limited value in predicting the natural history of the disease, most likely because of the high degree of sampling variability in the hepatic pathology of PSC.
Researchers at the Mayo Clinic have developed a multivariate statistical survival model from long-term survival data (Mayo risk score). The Mayo natural history model of PSC computes the score on the basis of the patient's age, history of variceal bleeding, and serum levels of albumin, bilirubin, and aspartate aminotransferase. This has been a major step in identifying patients at low, moderate, and high risk of dying while early in the course of PSC. In an age-adjusted multivariate analysis, each unit increase in the Mayo risk score was associated with a 2.5-fold increased risk of death, whereas the Child-Pugh classification for advanced cirrhosis had no significant impact on survival rate. The histologic stage of disease has consistently been useful in predicting survival rate, most likely because of a large sampling variability with liver biopsies.
Medical Care
- Treatment of patients with primary sclerosing cholangitis (PSC) should be directed at managing the following:
- Cirrhosis and portal hypertension
- Chronic cholestasis with pruritus and malabsorption
- Ductular complications, such as dominant strictures, cholelithiasis, and ascending bacterial cholangitis
- Other associated diseases, such as IBD or other autoimmune diseases
- Pharmacologic therapies that have been evaluated in adult patients with PSC include prednisone, azathioprine, budesonide, methotrexate, cyclosporine, pentoxifylline, tacrolimus, bezafibrate, and antibiotics.
- To date, none of the available treatment modalities has been shown to affect patient survival or need for liver transplantation.
- Choleretic therapy with ursodeoxycholic acid (UDCA) reportedly improves symptoms and biochemical abnormalities in adult patients with PSC. Some observational and controlled trial data also suggest a reduction in colonic dysplasia rates with UDCA.
- Children with PSC experience significant improvements in their liver biochemical indices when treated with UDCA; however, the long-term effect of UDCA on the clinical outcome of PSC has not been determined.
- Dominant strictures of the extrahepatic biliary tree, most often at the bifurcation of the hepatic ducts, are major problems for patients with PSC.
- Transhepatic or endoscopic balloon dilatation of strictures has been shown to be useful in children with PSC.
- Short-term stenting of strictures has also demonstrated clinical improvement of symptomatic strictures.
- Surgical, endoscopic, and interventional radiologic procedures to relieve symptomatic dominant strictures have been demonstrated to prolong survival time of the native liver in patients with PSC. None of these interventions have altered the ultimate rate of progression of PSC to end-stage liver disease.
- Bacterial cholangitis, which can occur spontaneously, is more common after endoscopic or surgical manipulation of the biliary tree. Episodes of cholangitis require prompt antibiotic therapy.
Surgical Care
- Surgical drainage procedures (eg, portoenterostomy, choledochoenterostomy) are insignificant in the management of PSC.
- These procedures may provide palliation but do not alter the natural history of the disease because of the consistent involvement of the intrahepatic biliary tree.
- Surgical drainage procedures are associated with an increased risk of cholangitis postoperatively, and subsequent liver transplantation may become technically more difficult.
- Orthotopic liver transplantation (OLT) has been proven successful in treating children with PSC.
- Data from numerous liver transplant centers demonstrate excellent long-term patient and graft survival for patients with end-stage PSC.
- Actuarial patient survival rates after OLT for PSC at 1 and 5 years have been shown to be greater than and approximately equal to 90%, respectively.
Consultations
Despite progress in early recognition, optimal treatment of patients with PSC remains a challenge and requires a multidisciplinary approach among hepatologists, endoscopists, surgeons, and interventional radiologists.
Diet
As with other cholestatic disorders, provide fat-soluble vitamin supplementation (vitamins A, D, E, K) and nutritional support to ensure adequate growth.
No effective medical therapies exist for primary sclerosing cholangitis (PSC). Choleretic therapy with ursodiol (ie, ursodeoxycholic acid [UDCA]) has been reported to improve symptoms and biochemical abnormalities, but the long-term effect on clinical outcome remains undetermined.
Drug Category: Choleretic agents
These agents enhance bile salt-dependent biliary flow. They may prove to be a valuable addition to therapy in repeated and refractory cholangitis.
| Drug Name | Ursodiol (URSO, Actigall) |
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| Description | Also called UDCA. Choleretic therapy to improve bile flow, treat cholestasis, and dissolve gallbladder stones. Shown to promote bile flow in cholestatic conditions associated with a patent extrahepatic biliary system. |
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| Adult Dose | 250-500 mg PO bid |
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| Pediatric Dose | 15-30 mg/kg/d PO divided bid/tid |
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| Contraindications | Documented hypersensitivity; cholesterol, radiopaque, or bile pigment gallstones; extrahepatic biliary tree obstruction |
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| Interactions | Aluminum-based antacids, cholestyramine, or oral contraceptives may decrease absorption |
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| Pregnancy | B - Usually safe but benefits must outweigh the risks.
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| Precautions | May affect CNS (eg, fatigue, headache, anxiety, depression), dermal system (eg, pruritus, rash, hair thinning), GI system (eg, nausea, vomiting, dyspepsia, metallic taste, abdominal pain, biliary pain, diarrhea, flatulence), liver (eg, rarely increased liver enzymes), and neuromuscular system (eg, arthralgias, myalgia, back pain) |
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Complications
- Cholangiocarcinoma (CCA) develops in 10-15% of adult patients with primary sclerosing cholangitis (PSC).
- Early detection of CCA is limited by a lack of reliable serologic, radiologic, and endoscopic findings.
- CCA often is cholangiographically indistinguishable from a benign dominant stricture.
- Brush cytology of dominant strictures has not shown predictive value.
- Serum CA 19-9 recently appears useful (75% sensitivity, 80% specificity) in discriminating PSC patients with and without CCA.
- Indices of disease severity, such as the Mayo risk or Child-Pugh score, do not correlate with the likelihood of CCA development in patients with PSC.
- Risk factors are poorly understood, but alcohol consumption has been shown to be an independent risk factor for development of CCA in patients with PSC. An association between previous or current smoking status and CCA has also been suggested.
- The risk of colorectal cancer or dysplasia is increased in patients with UC and PSC.
- Chronically active disease may be a risk factor, whereas folate may have a protective effect.
- Colorectal cancers associated with PSC are more likely to be proximal, diagnosed at a more advanced stage, and fatal.
- Colectomy in patients with UC and PSC does not alter the natural history of PSC.
- Patients who have undergone transplantation are susceptible to a wide array of complications secondary to chronic immunosuppression.
- The incidence of acute cellular and chronic ductopenic rejection is higher in patients with PSC than in individuals of a non-PSC control group.
- Chronic ductopenic rejection adversely affects patient and graft survival.
- Biliary strictures, both anastomotic and nonanastomotic, can occur.
- Recurrent sclerosing cholangitis occurs in 10-20% of patients with PSC who have undergone transplantation.
- Data from the Mayo Clinic's review of 150 consecutive PSC patients who received 174 liver allografts suggests that postoperative biliary strictures or recurrence of PSC does not impact patient survival.
Prognosis
- PSC is characterized by a slow insidious progression to cirrhosis. In adult patients, the median period of survival from the time of diagnosis is 9-11 years. The median period of survival is shorter for patients who are symptomatic at the time of diagnosis.
- The identification of abnormal LFTs in patients with IBD has led to earlier diagnosis of PSC, with apparent survival times that are likely longer.
- Despite progress in early recognition, optimal treatment of patients with PSC remains a challenge, requiring a multidisciplinary approach among hepatologists, endoscopists, surgeons, and interventional radiologists.
- The coexistence of UC is not predictive of an increased risk of death in PSC. UC may be associated with an increased posttransplantation survival.
| Media file 1:
Fibro-obliterative cholangiopathy. Image courtesy of Dr. Kay Washington. |
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Media type: Image
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Primary Sclerosing Cholangitis excerpt Article Last Updated: Jun 26, 2006
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