Blog
Primary Sclerosing Cholangitis (PSC)
Chronic progressive cholestatic liver disease
Covenant Metabolic Specialists
Physician Reviewed
Dec 3, 2025
Primary Sclerosing Cholangitis is a chronic progressive cholestatic liver disease characterized by multifocal biliary strictures and fibrosis, ultimately leading to cirrhosis and biliary cancer. It frequently coexists with ulcerative colitis, suggesting an immune‑mediated gut–liver axis pathology. PSC lacks curative medical therapy, making surveillance and transplant timing pivotal.
Symptoms
Early PSC may be silent or noted only by elevated alkaline phosphatase. Patients develop pruritus, fatigue, jaundice, right‑upper‑quadrant pain, and recurrent cholangitis with fevers and chills. Progression culminates in portal hypertension, osteopenia, fat‑soluble‑vitamin deficiencies, and cholangiocarcinoma presenting with worsening jaundice or weight loss.
Causes
Exact pathogenesis remains elusive. Hypotheses involve aberrant immune responses to gut microbial antigens migrating via the portal vein, genetic susceptibility (HLA‑B8, DR3), and bile‑duct epithelial injury leading to fibrotic scarring. Environmental triggers or gut dysbiosis likely precipitate disease in genetically prone individuals.
Risk Factors
Risks include male sex, Northern European ancestry, coexisting ulcerative colitis, family history, smoking cessation in UC patients, and possibly recurrent bacterial cholangitis. Certain HLA haplotypes heighten susceptibility.
Diagnosis
Persistent cholestatic liver enzymes prompt MRCP showing beaded biliary strictures. ERCP with brushing rules out malignancy in dominant strictures. Liver biopsy confirms small‑duct PSC or overlaps with autoimmune hepatitis. Colonoscopy screens for IBD. CA19‑9 and imaging every 6–12 months surveil for cholangiocarcinoma.
Treatments
No drug halts progression. Ursodeoxycholic acid at moderate doses may improve labs, though survival benefit is unproven. Endoscopic dilation and stenting relieve dominant strictures. Rifampin, cholestyramine, or SSRIs treat pruritus. Recurrent cholangitis requires antibiotics. Orthotopic liver transplantation offers definitive cure and excellent survival.
Prevention
Controlling IBD inflammation, avoiding hepatotoxic substances, vaccinating against hepatitis viruses, and engaging in regular cancer surveillance mitigate complications. Timely transplant referral before decompensation or cancer improves outcomes.
Our Take
Covenant’s PSC program synchronizes hepatologists, colorectal surgeons, and radiologists, providing one‑stop surveillance, prompt stricture dilations, and early transplant listing discussions.
PSC demands vigilance. Through integrated care and timely transplantation, Covenant maximizes life expectancy and quality of life while research advances toward disease‑modifying treatments.
