Variant Syndromes of Autoimmune Liver Diseases: Classification, Diagnosis and Management

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Variant Syndromes of Autoimmune Liver Diseases: Classification, Diagnosis and Management. / Weiler-Normann, Christina; Lohse, Ansgar W.

in: DIGEST DIS, Jahrgang 34, Nr. 4, 2016, S. 334-9.

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@article{d3fe0f7ce8694572867653ea751459a9,
title = "Variant Syndromes of Autoimmune Liver Diseases: Classification, Diagnosis and Management",
abstract = "The term 'overlap syndrome' has been used to describe the presence of both autoimmune hepatitis and primary biliary cholangitis or primary sclerosing cholangitis in the past. As this term is misleading, the term 'variant syndrome' should be used preferably. Laboratory features, serology, histology and bile duct imaging contribute to the diagnosis of 'variant syndromes'. Patients with a suspected variant syndrome should receive a complete work-up with liver histology, serology and - if not conclusive, bile duct imaging. Liver histology is usually reliable to recognize secondary autoimmune hepatitis in patients with primary cholestatic disease. An histological activitiy index of >4 usually is commonly seen in patients with variant syndrome. Identification of variant syndrome is very important, as appropriate - in most cases additional - immunosuppressive treatment is necessary and most patients will respond promptly.",
keywords = "Journal Article",
author = "Christina Weiler-Normann and Lohse, {Ansgar W}",
note = "{\textcopyright} 2016 S. Karger AG, Basel.",
year = "2016",
doi = "10.1159/000444472",
language = "English",
volume = "34",
pages = "334--9",
journal = "DIGEST DIS",
issn = "0257-2753",
publisher = "S. Karger AG",
number = "4",

}

RIS

TY - JOUR

T1 - Variant Syndromes of Autoimmune Liver Diseases: Classification, Diagnosis and Management

AU - Weiler-Normann, Christina

AU - Lohse, Ansgar W

N1 - © 2016 S. Karger AG, Basel.

PY - 2016

Y1 - 2016

N2 - The term 'overlap syndrome' has been used to describe the presence of both autoimmune hepatitis and primary biliary cholangitis or primary sclerosing cholangitis in the past. As this term is misleading, the term 'variant syndrome' should be used preferably. Laboratory features, serology, histology and bile duct imaging contribute to the diagnosis of 'variant syndromes'. Patients with a suspected variant syndrome should receive a complete work-up with liver histology, serology and - if not conclusive, bile duct imaging. Liver histology is usually reliable to recognize secondary autoimmune hepatitis in patients with primary cholestatic disease. An histological activitiy index of >4 usually is commonly seen in patients with variant syndrome. Identification of variant syndrome is very important, as appropriate - in most cases additional - immunosuppressive treatment is necessary and most patients will respond promptly.

AB - The term 'overlap syndrome' has been used to describe the presence of both autoimmune hepatitis and primary biliary cholangitis or primary sclerosing cholangitis in the past. As this term is misleading, the term 'variant syndrome' should be used preferably. Laboratory features, serology, histology and bile duct imaging contribute to the diagnosis of 'variant syndromes'. Patients with a suspected variant syndrome should receive a complete work-up with liver histology, serology and - if not conclusive, bile duct imaging. Liver histology is usually reliable to recognize secondary autoimmune hepatitis in patients with primary cholestatic disease. An histological activitiy index of >4 usually is commonly seen in patients with variant syndrome. Identification of variant syndrome is very important, as appropriate - in most cases additional - immunosuppressive treatment is necessary and most patients will respond promptly.

KW - Journal Article

U2 - 10.1159/000444472

DO - 10.1159/000444472

M3 - SCORING: Journal article

C2 - 27170386

VL - 34

SP - 334

EP - 339

JO - DIGEST DIS

JF - DIGEST DIS

SN - 0257-2753

IS - 4

ER -