Rare diseases · Sign or symptom
Cholangitis
Bile duct inflammation
HP:0030151
What it means
Inflammation of the biliary ductal system, affecting the intrahepatic or extrahepatic portions, or both.
Cholangitis is characterized by biliary epithelial damage with inflammatory cell infiltration. Some cholangitis is also associated with ductal and periductal fibrosis.
Rare diseases that can present with this14
Sometimes5–29%
7- Alveolar echinococcosis
- Autosomal recessive polycystic kidney disease
- Idiopathic hypereosinophilic syndrome
- Isolated mesenteric vein thrombosis
- Low phospholipid-associated cholelithiasis
- Syndromic multisystem autoimmune disease due to Itch deficiency
- X-linked lymphoproliferative disease due to XIAP deficiency
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.