Sclerosing cholangitis (MONDO:0018646) is defined in the packet as a chronic, autoimmune inflammatory liver disorder characterized by narrowing and scarring of the lumen of the bile ducts. Synonyms listed include primary sclerosing cholangitis (PSC) and fibrosing cholangitis. The packet's definition notes that it is often seen in patients with ulcerative colitis. Related entities listed under this term are neonatal ichthyosis-sclerosing cholangitis syndrome, isolated neonatal sclerosing cholangitis, primary sclerosing cholangitis, IgG4-related sclerosing cholangitis, and secondary sclerosing cholangitis. Prevalence is not certified in this packet.

The MONDO definition in the packet lists jaundice, fatigue, and malabsorption as signs and symptoms. No structured phenotype list with frequencies is certified in this packet, so the full range and frequency of features is unknown here.

The packet's definition describes the condition as autoimmune and inflammatory, affecting the bile ducts. No causative genes and no inheritance patterns are listed in the packet, and no GeneReviews chapter is attached. The packet does not describe further mechanistic detail.

No diagnostic methods, criteria, or GeneReviews diagnostic section are certified in this packet, so diagnostic approaches are unknown here.

The packet lists no certified foundational therapies, no GeneReviews management text, and no FDA-approved treatments for this condition, so treatment coverage is not certified in this packet. The packet records four orphan drug designations for primary sclerosing cholangitis: Beta-Lapachone (Curome Biosciences Co., Ltd.), a compound sponsored by Conatus Pharmaceuticals, a compound sponsored by Gilead Sciences, Inc., and 25HC3S (DURECT Corporation). An orphan designation is not an approval, and all four entries carry the status DESIGNATED.

The packet's definition states that the condition may lead to cirrhosis and liver failure. Further natural history information is not certified in this packet.

ClinicalTrials.gov records in the packet show active studies on sclerosing cholangitis. Examples include NCT07387549, a Phase 3 study of elafibranor in adults with primary sclerosing cholangitis sponsored by Ipsen (recruiting); NCT05627362, a Phase 2 elafibranor study sponsored by Ipsen (active, not recruiting); NCT07341282, a Phase 2 study of vancomycin in ulcerative colitis and primary sclerosing cholangitis sponsored by McMaster University (recruiting); and NCT06286709, a Phase 2 faecal microbiota transplantation study sponsored by the University of Birmingham (recruiting). Observational and mechanistic studies, including NCT06297993 (WIND-PSC, PSC Partners Seeking a Cure) and NCT04685200 (NIDDK), are also listed. Additional records can be searched on ClinicalTrials.gov.