J 2021

Differentiating Primary Sclerosing Cholangitis from Similar Diseases of Autoimmune Origin

KUNOVSKÝ, Lumír, Petr DITE, Ľubomíra HORŇÁKOVÁ, Jiří DOLINA, Magdalena UVIROVA et. al.

Základní údaje

Originální název

Differentiating Primary Sclerosing Cholangitis from Similar Diseases of Autoimmune Origin

Autoři

KUNOVSKÝ, Lumír (203 Česká republika, garant, domácí), Petr DITE (203 Česká republika), Ľubomíra HORŇÁKOVÁ (703 Slovensko, domácí), Jiří DOLINA (203 Česká republika, domácí), Magdalena UVIROVA (203 Česká republika), Vladimír KOJECKÝ (203 Česká republika, domácí), Arnost MARTINEK (203 Česká republika) a Petr JABANDŽIEV (203 Česká republika, domácí)

Vydání

Journal of Gastrointestinal and Liver Diseases, CLUJ-NAPOCA, MEDICAL UNIV PRESS, 2021, 1841-8724

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30219 Gastroenterology and hepatology

Stát vydavatele

Rumunsko

Utajení

není předmětem státního či obchodního tajemství

Odkazy

Impakt faktor

Impact factor: 2.142

Kód RIV

RIV/00216224:14110/21:00122809

Organizační jednotka

Lékařská fakulta

UT WoS

000701282100014

Klíčová slova anglicky

autoimmune diseases;   IgG4-related sclerosing cholangitis;   primary sclerosing cholangitis;   primary sclerosing cholangitis with increased IgG4

Příznaky

Mezinárodní význam, Recenzováno
Změněno: 1. 3. 2022 07:20, Mgr. Tereza Miškechová

Anotace

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.