Non-viral aspects

Autoimmuneliver diseases, particularly autoimmune hepatitis and primary biliary cholangitis,are not uncommon among the Thai population. This article summarizes main findings of studies of autoimmune liver diseases published during the past year, which included natural history and long-termoutcomes of primary biliary cholangitis treatment, a promising result of the new treatment for primary sclerosing cholangitis and outcomes of a second-line therapy of autoimmune hepatitis.

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Manifest hyperthyroidism in patient with autoimmune hepatitis (clinical observation)

Medical alphabet ◽

10.33667/2078-5631-2021-35-52-56 ◽

2021 ◽

pp. 52-56

Author(s):

M. A. Livzan ◽

O. V. Gaus ◽

D. A. Gavrilenko

Keyword(s):

Autoimmune Diseases ◽

Autoimmune Hepatitis ◽

Clinical Guidelines ◽

Thyroid Disease ◽

Liver Diseases ◽

Sclerosing Cholangitis ◽

Clinical Observation ◽

Primary Biliary Cholangitis ◽

Autoimmune Thyroid Diseases ◽

Autoimmune Thyroid

Among patients with autoimmune hepatitis concomitant autoimmune diseases occur in 40 % of cases. The most commonly associated autoimmune hepatitis is primary biliary cholangitis, primary sclerosing cholangitis and thyroid disease. With regard to the association of autoimmune liver diseases with each other there is experience in management and this is reflected in the clinical guidelines. Information on the features of comorbidity in autoimmune hepatitis and autoimmune thyroid diseases is very limited. This article presents our own clinical observation of the manifestation of thyrotoxicosis against the background of autoimmune hepatitis.

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

Digestive Diseases ◽

10.1159/000444472 ◽

2016 ◽

Vol 34 (4) ◽

pp. 334-339 ◽

Cited By ~ 8

Author(s):

Christina Weiler-Normann ◽

Ansgar W. Lohse

Keyword(s):

Bile Duct ◽

Autoimmune Hepatitis ◽

Sclerosing Cholangitis ◽

Immunosuppressive Treatment ◽

Overlap Syndrome ◽

Liver Histology ◽

Primary Biliary Cholangitis ◽

The Past ◽

Complete Work ◽

Work Up

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.

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Selected transgenic murine models of human autoimmune liver diseases

Pharmacological Reports ◽

10.1007/s43440-021-00351-y ◽

2022 ◽

Author(s):

Katarzyna Trzos ◽

Natalia Pydyn ◽

Jolanta Jura ◽

Jerzy Kotlinowski

Keyword(s):

Liver Diseases ◽

Sclerosing Cholangitis ◽

Molecular Mechanisms ◽

Treatment Strategies ◽

Primary Biliary Cholangitis ◽

Murine Models ◽

Transgenic Models ◽

New Treatment ◽

Transgenic Murine Models

AbstractMurine models of human diseases are of outmost importance for both studying molecular mechanisms driving their development and testing new treatment strategies. In this review, we first discuss the etiology and risk factors for autoimmune liver disease, including primary biliary cholangitis, autoimmune hepatitis and primary sclerosing cholangitis. Second, we highlight important features of murine transgenic models that make them useful for basic scientists, drug developers and clinical researchers. Next, a brief description of each disease is followed by the characterization of selected animal models.

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UPDATE OF THE BRAZILIAN SOCIETY OF HEPATOLOGY RECOMMENDATIONS FOR DIAGNOSIS AND MANAGEMENT OF AUTOIMMUNE DISEASES OF THE LIVER

Arquivos de Gastroenterologia ◽

10.1590/s0004-2803.201900000-43 ◽

2019 ◽

Vol 56 (2) ◽

pp. 232-241 ◽

Cited By ~ 2

Author(s):

Cláudia Alves COUTO ◽

Debora Raquel Benedita TERRABUIO ◽

Eduardo Luiz Rachid CANÇADO ◽

Gilda PORTA ◽

Cynthia LEVY ◽

...

Keyword(s):

Autoimmune Diseases ◽

Autoimmune Hepatitis ◽

Primary Sclerosing Cholangitis ◽

Liver Diseases ◽

Sclerosing Cholangitis ◽

Primary Biliary Cholangitis ◽

Web Based ◽

Overlap Syndromes ◽

Brazilian Society ◽

Consensus Document

ABSTRACT New data concerning the management of autoimmune liver diseases have emerged since the last single-topic meeting sponsored by the Brazilian Society of Hepatology to draw recommendations about the diagnosis and treatment of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), overlap syndromes of AIH, PBC and PSC and specific complications and topics concerning AIH and cholestatic liver diseases. This manuscript updates those previous recommendations according to the best evidence available in the literature up to now. The same panel of experts that took part in the first consensus document reviewed all recommendations, which were subsequently scrutinized by all members of the Brazilian Society of Hepatology using a web-based approach. The new recommendations are presented herein.

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Efficacy and Safety of Mycophenolate Mofetil and Tacrolimus as Second-line Therapy for Patients With Autoimmune Hepatitis

Clinical Gastroenterology and Hepatology ◽

10.1016/j.cgh.2017.06.001 ◽

2017 ◽

Vol 15 (12) ◽

pp. 1950-1956.e1 ◽

Cited By ~ 44

Author(s):

Cumali Efe ◽

Hannes Hagström ◽

Henriette Ytting ◽

Rahima A. Bhanji ◽

Niklas F. Müller ◽

...

Keyword(s):

Mycophenolate Mofetil ◽

Autoimmune Hepatitis ◽

Second Line ◽

Efficacy And Safety ◽

Second Line Therapy ◽

Line Therapy

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Obeticholic acid—a new therapy in PBC and NASH

British Medical Bulletin ◽

10.1093/bmb/ldaa006 ◽

2020 ◽

Vol 133 (1) ◽

pp. 95-104 ◽

Cited By ~ 3

Author(s):

Roger W Chapman ◽

Kate D Lynch

Keyword(s):

Clinical Trials ◽

Side Effects ◽

Ursodeoxycholic Acid ◽

Farnesoid X Receptor ◽

Primary Biliary Cholangitis ◽

Second Line ◽

Obeticholic Acid ◽

Second Line Therapy ◽

Line Therapy

Abstract Introduction Obeticholic acid (OCA) is a semi-synthetic hydrophobic bile acid (BA) analogue that is highly selective agonist of farnesoid X receptor (FXR), a key nuclear BA receptor, which induces expression of gut-derived hormones, in particular fibroblast growth factor 19. The resulting beneficial effects of OCA on glucose and lipid metabolism and particularly hepatic inflammation make it a candidate for the treatment of a variety of conditions including primary biliary cholangitis (PBC) and nonalcoholic steatohepatitis (NASH). Sources of data In PBC patients who have not initially responded to ursodeoxycholic acid, OCA has been shown in double-blind controlled clinical trials to significantly reduce serum alkaline phosphatase. To date, OCA is the only therapy licensed by the FDA, EMA and endorsed by NICE as second line therapy for PBC. No medications are currently approved in Europe or the USA for the treatment of NASH. In recent clinical trials, OCA has been shown encouraging results by improving liver blood tests and reducing liver fibrosis with no worsening of NASH. Areas of agreement OCA is the established second line therapy for PBC in those patients who fail to adequately respond to ursodeoxycholic acid. Areas of controversy The main side effects of OCA treatment in both PBC and NASH is that of dose-dependent pruritis which can lead to treatment discontinuation in ~1–10% of patients. In addition, OCA-treated patients may also exhibit (reversible) alterations in serum lipid levels; most notably a small decrease in high density lipoprotein cholesterol. It is not yet known whether these changes carry a long-term cardiovascular risk in NASH. In addition, the relatively high cost of OCA may limit its use in cash-limited health systems. Growing Points Additional clinical trials are in progress to ascertain the long-term effects of OCA on survival in PBC and NASH. Areas timely for developing research New FXR agonists with a lower rate of side effects are being developed and trialed. Combination therapy with other agents may offer increased efficacy.

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