Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis

被引：61

作者：

Sarcognato, Samantha ^{[1
]}

Sacchi, Diana ^{[1
]}

Grillo, Federica ^{[2
,3
]}

Cazzagon, Nora ^{[4
]}

Fabris, Luca ^{[5
]}

Cadamuro, Massimiliano ^{[5
]}

Cataldo, Ivana ^{[1
]}

Covelli, Claudia ^{[6
]}

Mangia, Alessandra ^{[7
]}

Guido, Maria ^{[1
,8
]}

机构：

[1] Azienda ULSS2 Marca Trevigiana, Dept Pathol, Treviso, Italy

[2] Univ Genoa, Anat Pathol Unit, Genoa, Italy

[3] Policlin San Martino Hosp, Genoa, Italy

[4] Univ Padua, Dept Surg Oncol & Gastroenterol DISCOG, Padua, Italy

[5] Univ Padua, Dept Mol Med DMM, Padua, Italy

[6] Fdn IRCCS Casa Sollievo della Sofferenza, Pathol Unit, San Giovanni Rotondo, Italy

[7] Fdn IRCCS Casa Sollievo della Sofferenza, Liver Unit, San Giovanni Rotondo, Italy

[8] Univ Padua, Dept Med DIMED, Padua, Italy

来源：

PATHOLOGICA | 2021年 / 113卷 / 03期

关键词：

immune-mediated cholangitis; autoimmune cholangitis; PBC; PSC; overlap variants; NONSUPPURATIVE DESTRUCTIVE CHOLANGITIS; REGULATORY T-CELLS; OVERLAP SYNDROME; NATURAL-HISTORY; HISTOLOGIC FEATURES; CLINICAL-FEATURES; GRADING SYSTEM; LIVER-BIOPSY; PLASMA-CELLS; HERING LOSS;

D O I：

10.32074/1591-951X-245

中图分类号：

R36 [病理学];

学科分类号：

100104 ;

摘要：

Autoimmune cholestatic liver diseases are rare hepato-biliary disorders characterized by a progressive, inflammatory destruction of bile ducts. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the main autoimmune cholestatic liver diseases. Both may evolve into secondary biliary cirrhosis and its complications. Therapeutic options are limited and liver transplantation remains the only definitive treatment for PBC and PSC. Most PBC and PSC patients have a typical presentation, which does not require liver biopsy. However, in routine clinical practice, important variants or specific subgroups that benefit from liver biopsy for proper management may be observed. Herein, we provide a general overview of clinical and pathological characteristic of PBC and PSC, highlighting the most important features for routine diagnostic practice.

引用

页码：170 / 184

页数：15

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