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

2019 ◽  
Vol 56 (2) ◽  
pp. 232-241 ◽  
Author(s):  
Cláudia Alves COUTO ◽  
Debora Raquel Benedita TERRABUIO ◽  
Eduardo Luiz Rachid CANÇADO ◽  
Gilda PORTA ◽  
Cynthia LEVY ◽  
...  

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.

2021 ◽  
pp. 52-56
Author(s):  
M. A. Livzan ◽  
O. V. Gaus ◽  
D. A. Gavrilenko

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.


2018 ◽  
Author(s):  
Albert J Czaja

When autoimmune hepatitis has features of primary biliary cholangitis or primary sclerosing cholangitis, these mixed clinical phenotypes constitute overlap syndromes. Diagnostic criteria have been promulgated, but clinical judgement and liver tissue examination remain cornerstones of diagnosis. Cholestatic laboratory and histological findings, concurrent inflammatory bowel disease, or non-response to conventional corticosteroid therapy compel practitioners to consider overlap in patients with autoimmune hepatitis. Laboratory indices of marked liver inflammation and histological findings of moderate to severe interface hepatitis, especially with lymphoplasmacytic infiltration, also warrant consideration of overlap in patients with primary biliary cholangitis or primary sclerosing cholangitits. Treatment recommendations to date have been based on weak clinical evidence, and disease management should be individualized and guided by the predominant disease component. Prednisone or prednisolone in combination with azathioprine has been used in patients with predominantly autoimmune hepatitis, whereas low dose ursodeoxycholic acid in conjunction with corticosteroid-based regimens has been recommended in syndromes with predominately cholestatic disease. All treatments have been variably effective, especially in patients with overlapping features of primary sclerosing cholangitis. Mycophenolate mofetil and calcineurin inhibitors have been used as salvage therapies in limited experiences, and liver transplantation has been associated with graft and overall survivals similar to those of the classical unmixed diseases.  This review contains 6 figures, 7 tables and 50 references Keywords: autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, overlap, cholestatic laboratory and histological features


2018 ◽  
Author(s):  
Albert J Czaja

When autoimmune hepatitis has features of primary biliary cholangitis or primary sclerosing cholangitis, these mixed clinical phenotypes constitute overlap syndromes. Diagnostic criteria have been promulgated, but clinical judgement and liver tissue examination remain cornerstones of diagnosis. Cholestatic laboratory and histological findings, concurrent inflammatory bowel disease, or non-response to conventional corticosteroid therapy compel practitioners to consider overlap in patients with autoimmune hepatitis. Laboratory indices of marked liver inflammation and histological findings of moderate to severe interface hepatitis, especially with lymphoplasmacytic infiltration, also warrant consideration of overlap in patients with primary biliary cholangitis or primary sclerosing cholangitits. Treatment recommendations to date have been based on weak clinical evidence, and disease management should be individualized and guided by the predominant disease component. Prednisone or prednisolone in combination with azathioprine has been used in patients with predominantly autoimmune hepatitis, whereas low dose ursodeoxycholic acid in conjunction with corticosteroid-based regimens has been recommended in syndromes with predominately cholestatic disease. All treatments have been variably effective, especially in patients with overlapping features of primary sclerosing cholangitis. Mycophenolate mofetil and calcineurin inhibitors have been used as salvage therapies in limited experiences, and liver transplantation has been associated with graft and overall survivals similar to those of the classical unmixed diseases.  This review contains 6 figures, 7 tables and 50 references Keywords: autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, overlap, cholestatic laboratory and histological features


2018 ◽  
Author(s):  
Albert J Czaja

When autoimmune hepatitis has features of primary biliary cholangitis or primary sclerosing cholangitis, these mixed clinical phenotypes constitute overlap syndromes. Diagnostic criteria have been promulgated, but clinical judgement and liver tissue examination remain cornerstones of diagnosis. Cholestatic laboratory and histological findings, concurrent inflammatory bowel disease, or non-response to conventional corticosteroid therapy compel practitioners to consider overlap in patients with autoimmune hepatitis. Laboratory indices of marked liver inflammation and histological findings of moderate to severe interface hepatitis, especially with lymphoplasmacytic infiltration, also warrant consideration of overlap in patients with primary biliary cholangitis or primary sclerosing cholangitits. Treatment recommendations to date have been based on weak clinical evidence, and disease management should be individualized and guided by the predominant disease component. Prednisone or prednisolone in combination with azathioprine has been used in patients with predominantly autoimmune hepatitis, whereas low dose ursodeoxycholic acid in conjunction with corticosteroid-based regimens has been recommended in syndromes with predominately cholestatic disease. All treatments have been variably effective, especially in patients with overlapping features of primary sclerosing cholangitis. Mycophenolate mofetil and calcineurin inhibitors have been used as salvage therapies in limited experiences, and liver transplantation has been associated with graft and overall survivals similar to those of the classical unmixed diseases.  This review contains 6 figures, 7 tables and 50 references Keywords: autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, overlap, cholestatic laboratory and histological features


2017 ◽  
Vol 95 (10) ◽  
pp. 940-945
Author(s):  
V. I. Podzolkov ◽  
Natal’ya A. Dragomiretskaya ◽  
E. V. Volchkova ◽  
O. I. Mitrokhina

Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC) comprise a group of autoimmune hepatic disorders. Sometimes, overlap syndromes occur, e.g. AIH/PBC or AIH/PSC. The authors consider autoimmune hepatic disorders with reference to pathogenesis, clinical picture, diagnostics, and treatment of overlap syndromes.


2018 ◽  
Vol 1 (2) ◽  
pp. 37-39
Author(s):  
Roongruedee Chaiteerakij

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.


Author(s):  
Gavin Spickett

This chapter covers the presentation, immunogenetics, immunopathology, diagnosis, treatment, and testing for a range of liver diseases. Primary biliary cirrhosis, autoimmune hepatitis, and primary sclerosing cholangitis are described.


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