Detailed Information on Publication Record
2021
Differentiating Primary Sclerosing Cholangitis from Similar Diseases of Autoimmune Origin
KUNOVSKÝ, Lumír, Petr DITE, Ľubomíra HORŇÁKOVÁ, Jiří DOLINA, Magdalena UVIROVA et. al.Basic information
Original name
Differentiating Primary Sclerosing Cholangitis from Similar Diseases of Autoimmune Origin
Authors
KUNOVSKÝ, Lumír (203 Czech Republic, guarantor, belonging to the institution), Petr DITE (203 Czech Republic), Ľubomíra HORŇÁKOVÁ (703 Slovakia, belonging to the institution), Jiří DOLINA (203 Czech Republic, belonging to the institution), Magdalena UVIROVA (203 Czech Republic), Vladimír KOJECKÝ (203 Czech Republic, belonging to the institution), Arnost MARTINEK (203 Czech Republic) and Petr JABANDŽIEV (203 Czech Republic, belonging to the institution)
Edition
Journal of Gastrointestinal and Liver Diseases, CLUJ-NAPOCA, MEDICAL UNIV PRESS, 2021, 1841-8724
Other information
Language
English
Type of outcome
Článek v odborném periodiku
Field of Study
30219 Gastroenterology and hepatology
Country of publisher
Romania
Confidentiality degree
není předmětem státního či obchodního tajemství
References:
Impact factor
Impact factor: 2.142
RIV identification code
RIV/00216224:14110/21:00122809
Organization unit
Faculty of Medicine
UT WoS
000701282100014
Keywords in English
autoimmune diseases; IgG4-related sclerosing cholangitis; primary sclerosing cholangitis; primary sclerosing cholangitis with increased IgG4
Tags
International impact, Reviewed
Změněno: 1/3/2022 07:20, Mgr. Tereza Miškechová
Abstract
V originále
Background & Aims: Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease. Differential diagnostics can confuse it with immunoglobulin (Ig) G4-related sclerosing cholangitis (SC), an IgG4-related disease with clearly proven autoimmune origin. Differential diagnosis is made even more challenging because PSC with increased IgG4 levels (PSC-increased IgG4) also occurs. In order to facilitate their differential diagnosis, we reviewed recent literature regarding the etiologies, identifying characteristics, the most useful diagnostics, treatment, and the progression of these partially similar diseases. It is clear that PSC's pathogenesis differs from that of IgG4-related SC. In any differential diagnosis between PSC and PSC-increased IgG4, high IgG1 and low or normal IgG2 levels are characteristic for patients with PSC. Histological examination of the biliary tree wall in patients with IgG4-related SC typically reveals such changes as storiform fibrosis, obliterative phlebitis, and venulitis. These are absent in PSC-increased IgG4, which is characterized by a typical circular thickness in different parts of the biliary ducts. Finally, PSC is associated with inflammatory bowel disease, which is rare in IgG4-related SC, and more frequently is associated with cholangiocarcinomas and colon cancers. As distinct from IgG4-related SC, PSC is not a primary autoimmune disease.