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Primary sclerosing cholangitis

Background

Overview

Definition
PSC is a chronic autoimmune liver disease characterized by progressive biliary inflammation and fibrosis, resulting in multifocal biliary strictures.
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Pathophysiology
Risk factors include family history, genetic susceptibility (HLA class, I, II, and III loci, IL-2 pathway), environmental factors (exposure to farm animals, contraceptive pills, UTIs, diet), and microbial dysbiosis due to IBD.
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Epidemiology
The incidence and prevalence of PSC is estimated at 0-0.92 per 100,000 person-years, and 0-13.6 persons per 100,000 population, respectively.
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Disease course
Injury to cholangiocytes leads to biliary inflammation and fibrosis, which may further progress to chronic cholestasis, liver cirrhosis, bile duct strictures, bacterial cholangitis, metabolic bone disease, colon cancer, and cholangiocarcinoma.
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Prognosis and risk of recurrence
PSC is associated with increased risk of cholangiocarcinoma (400 times) and colon cancer (10 times) as compared to the general population.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of primary sclerosing cholangitis are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL 2024,2022), the World Federation for Ultrasound in Medicine and Biology (WFUMB 2024), the European Association for the Study of the Liver (EASL/ILCA 2023), the American Association for ...
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Screening and diagnosis

Diagnosis: as per EASL 2022 guidelines, diagnose large-duct PSC in adult patients presenting with elevated serum markers of cholestasis in the presence of typical findings of sclerosing cholangitis on high-quality cholangiography (preferably with MRCP) and after exclusion of secondary causes.
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  • Differential diagnosis (IgG4-SC)

  • Differential diagnosis (cholangiocarcinoma)

Classification and risk stratification

Risk stratification: as per AASLD 2022 guidelines, obtain risk stratification and fibrosis staging at diagnosis of PSC and regularly during follow-up. Consider using clinical risk tools for this purpose but interpret specific probabilities of events with caution in individual patients.
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Diagnostic investigations

Laboratory tests: as per EASL 2022 guidelines, consider obtaining serum IgG4 at the time of diagnosis in all adult patients with large-duct sclerosing cholangitis.
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  • Diagnostic imaging

  • Evaluation for liver fibrosis

  • Evaluation for osteoporosis

  • Evaluation for AIH

  • Evaluation for cholangiocarcinoma

  • Nutritional assessment

  • Evaluation of fatigue

  • Assessment of QoL

Diagnostic procedures

Liver biopsy: as per AASLD 2022 guidelines, consider performing liver biopsy to rule out small-duct PSC in patients with suspected PSC and a normal, high-quality MRI with cholangiopancreatography.
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  • ERCP

  • Upper gastrointestinal endoscopy

  • Lower gastrointestinal endoscopy

Medical management

General principles: as per AASLD 2022 guidelines, consider offering enrollment in clinical trials in all patients with PSC.
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  • Management of pruritus

  • UDCA

  • Corticosteroids

  • Immunosuppressants (pre-transplant)

  • Immunosuppressants (post-transplant)

  • Vancomycin

Nonpharmacologic interventions

Vitamin supplements: as per BSG 2019 guidelines, consider setting a low threshold for empirical vitamin replacement in patients with advanced PSC, as poor nutrition and fat-soluble vitamin deficiencies are relatively common in these patients.
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  • Psychosocial support

Therapeutic procedures

Endoscopic biliary dilatation: as per AASLD 2022 guidelines, decide on the choice between biliary balloon dilation with or without stenting at the discretion of the endoscopist. Remove stent within 4 weeks following placement if a plastic biliary stent is placed.
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Surgical interventions

Liver transplantation: as per AASLD 2022 guidelines, consider offering liver transplantation in all patients with PSC and complications of end-stage liver disease, recurrent cholangitis, intractable pruritus, or early-stage hepatobiliary cancers.
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Specific circumstances

Pediatric patients: as per EASL 2022 guidelines, use the same diagnostic workup in pediatric patients as in adult practice, based on MRCP as the diagnostic modality of choice.
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  • Pregnant patients

  • Patients with ABCB4 deficiency

  • Patients with gallbladder polyps

  • Patients with IBD

  • Patients with acute bacterial cholangitis

  • Patients with bacterial cholangitis

  • Patients with AIH

  • Critically ill patients with secondary sclerosing cholangitis

Patient education

Preconception counseling: as per EASL 2022 guidelines, consider providing preconception counseling in female patients with PSC wishing to become pregnant.
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Follow-up and surveillance

Indications for referral: as per AASLD 2022 guidelines, refer patients with an equivocal MRI with cholangiopancreatography to an experienced center for consideration of a repeat high-quality MRI with cholangiopancreatography or liver biopsy.
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  • Follow-up

  • Surveillance for IBD

  • Surveillance for gallbladder polyps

  • Surveillance for malignancy (cholangiocarcinoma)

  • Surveillance for malignancy (HCC)

  • Surveillance for malignancy (gallbladder carcinoma)

  • Surveillance for malignancy (CRC)