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Primary Sclerosing Cholangitis (PSC)

Progressive Fibro-obliterative Bile Duct Disease

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

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What is Primary Sclerosing Cholangitis (PSC)?

Primary sclerosing cholangitis is a chronic progressive cholestatic disorder featuring inflammation and fibrosis of intrahepatic and/or extrahepatic bile ducts leading to multifocal stricturing.

Strong association with IBD: 60–80% of PSC patients have ulcerative colitis, often a mild but pancolonic form.

Diagnosis by magnetic resonance cholangiopancreatography (MRCP) showing multifocal strictures with beaded appearance; liver biopsy reserved for small-duct PSC or unclear cases.

Increased risk for cholangiocarcinoma (10–15% lifetime), gallbladder cancer, and colorectal cancer in PSC-IBD.

Symptoms

Asymptomatic elevated alkaline phosphatase on screening
Fatigue, pruritus, and right upper quadrant pain
Jaundice and dark urine with episodic cholangitis
Fever and rigors suggesting bacterial cholangitis
Weight loss and steatorrhea with fat-soluble vitamin deficiency
Features of advanced disease: ascites, variceal bleeding, encephalopathy

Risk Factors

Inflammatory bowel disease (especially ulcerative colitis)
Male sex (2:1 male predominance)
Age 30–50 years at diagnosis
Family history of PSC or autoimmune disease
HLA-B8, DR3 haplotypes
Northern European descent

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Unexplained cholestatic liver tests, especially with IBD
  • New jaundice, fever, or severe right upper quadrant pain (cholangitis)
  • IBD patient for baseline and periodic surveillance

Treatment Methods

01
No proven medical therapy to alter disease course; ursodeoxycholic acid controversial (moderate doses 15–20 mg/kg/day may be used)
02
Endoscopic treatment of dominant strictures: ERCP with balloon dilation, stent placement for biliary obstruction
03
Antibiotic prophylaxis and prompt treatment of bacterial cholangitis (ciprofloxacin, ceftriaxone)
04
Cholangiocarcinoma surveillance: annual MRI/MRCP plus CA 19-9; colonoscopy annually in PSC-IBD
05
Fat-soluble vitamin supplementation (A, D, E, K), calcium and bone density monitoring
06
Liver transplantation for end-stage disease, intractable pruritus, recurrent cholangitis, or early cholangiocarcinoma (Mayo protocol)

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.