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| Disease found: | Primary Sclerosing Cholangitis |
| Current as of: | October 8, 2024 |
| Disease Overview: | PSC is an immune-mediated inflammatory disease causing fibrosis of intra- and extra-hepatic bile ducts [more]. Many patients have co-occurring inflammatory bowel disease, and progression to liver cirrhosis is common [more]. |
| Signs and Symptoms: | Fatigue and pruritus are common initial signs; jaundice and ascites indicate progressive liver damage. Patients may have hepatosplenomegaly and RUQ pain [more]. Elevated alkaline phosphatase is very common; GGT elevation is also common but aminotransferases are only mildly elevated [more] [more]. |
| Diagnosis: | U/S of the RUQ is recommended to rule out biliary obstruction; if no obstruction, MRCP is the preferred initial imaging modality for diagnosis of PSC [more]. Liver biopsy can be confirmatory but is rarely required unless MRCP is unsuccessful in establishing diagnosis. All patients with PSC should be tested for IgG4 autoantibodies. |
| Treatment: | Ursodeoxycholic acid therapy ranging from 3-12 months, avoiding doses above 20mg/kg/day, is the current standard of care, though clinical trials have shown mixed/controversial results [more]. Immunosuppressive therapies are under study. Many patients will progress to end-stage liver disease and require transplant. For dominant strictures felt to be symptomatic, balloon dilation is preferred to stenting [more]. Patients with IgG4+ disease may have a more rapid course that is less steroid-responsive [more]. |
| Clinical Management: | Patients will need frequent monitoring and likely consideration for liver transplant with disease progression; patients are also at high risk for cholangiocarcinoma [more]. If a prior diagnosis of IBD has not been made, colonoscopy may be indicated given the frequent overlap between PSC and IBD [more]. |
| Referral: | PSC Partners is a patient support organization maintaining a map of providers with PSC experience, including hepatologists and gastroenterologists. Referral to Medical Genetics Department, if available. Initial virtual care is also available through organizations like TeleRare Health. |
| Clinical Trials: | A large number of trials are currently recruiting in multiple countries. |