primary sclerosing cholangitis
MONDO:0013433Mondo
Findings
No curated finding names primary sclerosing cholangitis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Primary sclerosing cholangitis (PSC) is a rare, slowly progressive liver disease characterized by inflammation and destruction of the intra- and/or extra-hepatic bile ducts that lead to cholestasis, liver fibrosis, liver cirrhosis and ultimately liver failure.
Definition from the Mondo Disease Ontology (MONDO:0013433), read 2026-09-29. CC BY 4.0.
Features
51 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abnormal biliary tract morphologyHPOHP:0012440
- Very frequent (80% to 99% of cases)
- AutoimmunityHPOHP:0002960
- Very frequent (80% to 99% of cases)
- CholestasisHPOHP:0001396
- Very frequent (80% to 99% of cases)
- Abnormal large intestine physiologyHPOHP:0012700
- Frequent (30% to 79% of cases)
- AscitesHPOHP:0001541
- Frequent (30% to 79% of cases)
- CirrhosisHPOHP:0001394
- Frequent (30% to 79% of cases)
- Dilated superficial abdominal veinsHPOHP:0030168
- Frequent (30% to 79% of cases)
- Elevated alkaline phosphatase of hepatic originHPOHP:0010638
- Frequent (30% to 79% of cases)
- Elevated circulating hepatic transaminase concentrationHPOHP:0002910
- Frequent (30% to 79% of cases)
- FeverHPOHP:0001945
- Frequent (30% to 79% of cases)
- Hepatic fibrosisHPOHP:0001395
- Frequent (30% to 79% of cases)
- HepatomegalyHPOHP:0002240
- Frequent (30% to 79% of cases)
Reported absent (4)
- Abnormal eosinophil morphologyHPOHP:0001879
- Amyloid depositionHPOHP:0011034
- HistiocytosisHPOHP:0100727
- Recurrent systemic pyogenic infectionsHPOHP:0005429
Show the remaining 39
- HepatosplenomegalyHPOHP:0001433
- Frequent (30% to 79% of cases)
- Palmar telangiectasiaHPOHP:0100869
- Frequent (30% to 79% of cases)
- Portal hypertensionHPOHP:0001409
- Frequent (30% to 79% of cases)
- Spider hemangiomaHPOHP:0012522
- Frequent (30% to 79% of cases)
- SplenomegalyHPOHP:0001744
- Frequent (30% to 79% of cases)
- Ulcerative colitisHPOHP:0100279
- Frequent (30% to 79% of cases)
Where it sits
- A kind of
Other names
1 name
Resolves to: primary sclerosing cholangitis
- Also called
- PSC