Primary Sclerosing Cholangitis (PSC)

PSC is a chronic cholestatic liver disease characterized by progressive inflammation and fibrosis of intra- and extrahepatic bile ducts. It is strongly associated with ulcerative colitis and increases the risk of cirrhosis, cholangiocarcinoma, and colorectal cancer.

Symptoms

• Many patients asymptomatic initially
• Fatigue, pruritus, RUQ discomfort
• Jaundice, dark urine, pale stools
• Recurrent bacterial cholangitis (fever, chills, abdominal pain)

Diagnosis

• Elevated alkaline phosphatase ± bilirubin
• MRCP showing multifocal strictures/dilatations (“beading”)
• ERCP reserved for therapeutic dilation or brushing biopsies
• Autoimmune markers often present (p-ANCA); AMA usually negative (distinguishes from PBC)
• Liver biopsy if small-duct PSC suspected or overlap with autoimmune hepatitis
• Screen for IBD if not previously diagnosed

Management

Medical Therapy

• No curative medical therapy yet
• Ursodeoxycholic acid at moderate doses sometimes used for biochemical improvement (controversial)
• Manage pruritus (cholestyramine, rifampin, naltrexone)
• Antibiotics for cholangitis episodes
• Treat associated IBD aggressively

Surveillance

• Annual or semiannual imaging (MRCP/ultrasound) + CA 19-9 for cholangiocarcinoma
• Colonoscopy every 1–2 years in PSC-IBD for colorectal cancer risk
• Bone density scans for osteoporosis prevention

Interventional/Surgical

• Endoscopic dilation/stenting of dominant strictures
• Liver transplantation for decompensated cirrhosis, MELD ≥15, recurrent cholangitis, or intractable pruritus
• Post-transplant recurrence occurs in up to 25%

Lifestyle & Support

• Vaccinate against HAV/HBV
• Avoid alcohol; maintain healthy weight and nutrition
• Manage vitamin deficiencies (A, D, E, K)
• Mental health support; chronic monitoring causes anxiety

Complications

• Cholangiocarcinoma (lifetime risk 5–20%)
• Gallbladder carcinoma; portal hypertension, varices
• Dominant strictures causing recurrent cholangitis
• Osteoporosis, fat-soluble vitamin deficiency

Research & Future Directions

Emerging therapies target bile acid signaling (FXR agonists), antifibrotic pathways, and immune dysregulation.

Experimental & Emerging Treatments

• Non-steroidal FXR Agonists: E.g., cilofexor to reduce cholestasis.
• Farnesoid X/PPAR Dual Agonists: Aim to reduce inflammation and fibrosis simultaneously.
• Norursodeoxycholic Acid (norUDCA): Promising results in phase II trials.
• Microbiome-Based Therapies: Fecal transplant and selective antibiotics addressing PSC-IBD gut dysbiosis.

Track PSC with Diagnoza.care

Stay a Step Ahead of PSC – Log labs (ALP, bilirubin), imaging, ERCP procedures, IBD activity, symptoms, medications, vaccines, bone density, and transplant evaluations; capture side effects; and let the AI companion flag changes prompting hepatology follow-up.

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Medical Disclaimer: Informational only. Partner with your hepatologist and IBD team for surveillance schedules, ERCP decisions, and transplant planning. Sources: American Association for the Study of Liver Diseases, European Association for the Study of the Liver, PSC Partners Seeking a Cure