[Immune-mediated cholangiopathies : Diagnostic and therapeutic challenges].
Autoimmunassoziierte Gallenwegserkrankungen : Diagnostische und therapeutische Herausforderungen.
IgG4-associated cholangitis
Magnetic resonance cholangiopancreatography
Primary biliary cholangitis
Primary sclerosing cholangitis
Ursodeoxycholic acid
Journal
Der Radiologe
ISSN: 1432-2102
Titre abrégé: Radiologe
Pays: Germany
ID NLM: 0401257
Informations de publication
Date de publication:
Apr 2019
Apr 2019
Historique:
pubmed:
16
3
2019
medline:
20
7
2019
entrez:
16
3
2019
Statut:
ppublish
Résumé
Immune-mediated cholangiopathies comprise primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and IgG4-associated cholangitis (IAC). A common feature is the progressive destruction of bile ducts leading to cholestasis with fibrosis and cirrhosis of the liver over time. The diseases are mostly identified during routine laboratory testing. Clinical signs and symptoms such as pruritus, fatigue or jaundice are infrequent in the early stage. The diagnostic work-up involves the patient's history, physical examination, serological tests, abdominal ultrasonography, magnetic resonance cholangiopancreatography (MRCP) and, where necessary, liver biopsy and genetic testing. Ursodeoxycholic acid (UDCA) is an effective treatment of PBC. Second-line therapies in addition to UDCA for incomplete UDCA responders are obeticholic acid (OCA) and bezafibrate, whereby only OCA has received approval for this indication from American (Federal Drug Administration) and European (European Medicines Agency) authorities. In PSC, UDCA improves prognostic markers; dominant bile duct strictures are treated with endoscopic balloon dilatation. Despite therapy, liver transplantation is frequently necessary for PSC. The risk of developing cholangiocarcinoma, colon cancer, and gallbladder cancer is increased for patients with PSC. In contrast to PBC and PSC, IAC responds well to corticosteroids. Disease relapse, however, is common, making long-term treatment with low-dose prednisolone or azathioprine necessary.
Sections du résumé
BACKGROUND
BACKGROUND
Immune-mediated cholangiopathies comprise primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and IgG4-associated cholangitis (IAC). A common feature is the progressive destruction of bile ducts leading to cholestasis with fibrosis and cirrhosis of the liver over time. The diseases are mostly identified during routine laboratory testing. Clinical signs and symptoms such as pruritus, fatigue or jaundice are infrequent in the early stage.
DIAGNOSIS
METHODS
The diagnostic work-up involves the patient's history, physical examination, serological tests, abdominal ultrasonography, magnetic resonance cholangiopancreatography (MRCP) and, where necessary, liver biopsy and genetic testing.
THERAPY
METHODS
Ursodeoxycholic acid (UDCA) is an effective treatment of PBC. Second-line therapies in addition to UDCA for incomplete UDCA responders are obeticholic acid (OCA) and bezafibrate, whereby only OCA has received approval for this indication from American (Federal Drug Administration) and European (European Medicines Agency) authorities. In PSC, UDCA improves prognostic markers; dominant bile duct strictures are treated with endoscopic balloon dilatation. Despite therapy, liver transplantation is frequently necessary for PSC. The risk of developing cholangiocarcinoma, colon cancer, and gallbladder cancer is increased for patients with PSC. In contrast to PBC and PSC, IAC responds well to corticosteroids. Disease relapse, however, is common, making long-term treatment with low-dose prednisolone or azathioprine necessary.
Identifiants
pubmed: 30874827
doi: 10.1007/s00117-019-0513-x
pii: 10.1007/s00117-019-0513-x
doi:
Substances chimiques
Ursodeoxycholic Acid
724L30Y2QR
Types de publication
Journal Article
Review
Langues
ger
Sous-ensembles de citation
IM
Pagination
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