DISEASE SCANNER

Global Incurable Diseases Tracker

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Autoimmune Disease

Primary Sclerosing Cholangitis (PSC)

HIGH SEVERITY

A chronic cholestatic liver disease characterized by inflammation and fibrosis of bile ducts, eventually leading to cirrhosis. Strongly associated with inflammatory bowel disease (70% of patients). Increased risk of cholangiocarcinoma and colorectal cancer.

Global Affected

70.0K

Countries

15

Symptoms

Often asymptomatic (elevated ALP)
Fatigue
Pruritus (itching)
Jaundice
Cholangitis episodes (fever, RUQ pain)
Weight loss

Treatment Options

Corticosteroids
Immunosuppressants
Biologic agents
DMARDs
Plasmapheresis
IVIG
Physical therapy
Symptom management

Risk Factors

1Female gender
2Family history
3Genetic predisposition
4Environmental triggers
5Infections
6Hormonal factors
7Smoking

Diagnostic Methods

  • 1Autoantibody testing
  • 2Inflammatory markers
  • 3Imaging
  • 4Biopsy
  • 5Clinical criteria
  • 6Exclusion of other causes

Prognosis

Variable; many autoimmune diseases are chronic with relapsing-remitting courses. Early treatment and modern biologics have significantly improved outcomes.

Prevention

  • No specific prevention
  • Smoking cessation
  • Avoidance of triggers
  • Early diagnosis and treatment
  • Regular monitoring

Research Status

No FDA-approved medical therapy. Ursodiol may improve biomarkers but not outcomes. Liver transplant only definitive treatment. Norursodeoxycholic acid (norUDCA) in phase 3 trials. Surveillance for malignancy essential.

Affected Countries

AustraliaCanadaGermanySpainFinlandFranceUnited KingdomIsraelIndiaItalyJapanMalaysiaNorwaySwedenUnited States

Sources

  • https://www.ncbi.nlm.nih.gov/books/NBK519046
  • https://www.ncbi.nlm.nih.gov/books
  • https://www.mayoclinic.org/diseases-conditions
  • https://rarediseases.org/rare-diseases

Medical Disclaimer

This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.