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@article{1802366,
author = {Kunovský, Lumír and Dite, Petr and Horňáková, Ľubomíra and Dolina, Jiří and Uvirova, Magdalena and Kojecký, Vladimír and Martinek, Arnost and Jabandžiev, Petr},
article_location = {CLUJ-NAPOCA},
article_number = {3},
doi = {http://dx.doi.org/10.15403/jgld-3849},
keywords = {autoimmune diseases; IgG4-related sclerosing cholangitis; primary sclerosing cholangitis; primary sclerosing cholangitis with increased IgG4},
language = {eng},
issn = {1841-8724},
journal = {Journal of Gastrointestinal and Liver Diseases},
title = {Differentiating Primary Sclerosing Cholangitis from Similar Diseases of Autoimmune Origin},
url = {https://www.jgld.ro/jgld/index.php/jgld/article/view/3849/1760},
volume = {30},
year = {2021}
}
TY - JOUR
ID - 1802366
AU - Kunovský, Lumír - Dite, Petr - Horňáková, Ľubomíra - Dolina, Jiří - Uvirova, Magdalena - Kojecký, Vladimír - Martinek, Arnost - Jabandžiev, Petr
PY - 2021
TI - Differentiating Primary Sclerosing Cholangitis from Similar Diseases of Autoimmune Origin
JF - Journal of Gastrointestinal and Liver Diseases
VL - 30
IS - 3
SP - 398-403
EP - 398-403
PB - MEDICAL UNIV PRESS
SN - 18418724
KW - autoimmune diseases
KW -
KW - IgG4-related sclerosing cholangitis
KW -
KW - primary sclerosing cholangitis
KW -
KW - primary sclerosing cholangitis with increased IgG4
UR - https://www.jgld.ro/jgld/index.php/jgld/article/view/3849/1760
N2 - 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.
ER -
KUNOVSKÝ, Lumír, Petr DITE, Ľubomíra HORŇÁKOVÁ, Jiří DOLINA, Magdalena UVIROVA, Vladimír KOJECKÝ, Arnost MARTINEK and Petr JABANDŽIEV. Differentiating Primary Sclerosing Cholangitis from Similar Diseases of Autoimmune Origin. \textit{Journal of Gastrointestinal and Liver Diseases}. CLUJ-NAPOCA: MEDICAL UNIV PRESS, 2021, vol.~30, No~3, p.~398-403. ISSN~1841-8724. Available from: https://dx.doi.org/10.15403/jgld-3849.
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