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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 a 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, roč.~30, č.~3, s.~398-403. ISSN~1841-8724. Dostupné z: https://dx.doi.org/10.15403/jgld-3849.
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