Overlap syndromes of autoimmune hepatitis: diagnosis and treatment

Aguilar-Nájera, O.; Velasco-Zamora, J.A.; Torre, Aldo

doi:10.1016/j.rgmxen.2015.07.001

Cited by 11 publications

(9 citation statements)

References 55 publications

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“…Fibrogenesis in Ad‐ Mdr2 −/− mice may be driven by two distinct activation pathways, which may potentiate each other, as in AIH only HSCs differentiate into myofibroblasts, whereas in SC both myofibroblastic HSCs and PFs are involved. These strong fibrotic changes detected in Ad‐ Mdr2 −/− mice match with the observation that patients with overlap syndromes tend to develop a more aggressive form of autoimmune liver disease with poorer treatment responses and higher rates of cirrhosis The hCYP2D6‐specific B cell response is a parameter that was similar in Ad‐FVB and Ad‐ Mdr2 −/− mice.…”

Section: Discussionsupporting

confidence: 79%

Effects of adenovirus‐induced hepatocyte damage on chronic bile duct inflammation in a sclerosing cholangitis mouse model

Fuchs

Bayer

Taubert

et al. 2019

Liver International

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Background & Aims: Four major autoimmune diseases target the liver. They develop because of bile duct destruction, leading to chronic cholestasis or result from hepatocyte damage like autoimmune hepatitis (AIH). Interestingly, some patients simultaneously show features of both cholangitis and AIH. Our goal was to mimic such concurrent characteristics in a mouse model that would help deciphering mechanisms possibly involved in an inflammatory crosstalk between cholestatic disease and hepatitis.Methods: Mdr2 −/− mice, which spontaneously develop sclerosing cholangitis because of accumulation of toxic bile salts, were infected with adenovirus (Ad) encoding human Cytochrome P4502D6 (hCYP2D6), the major target autoantigen in type-2 AIH, to trigger hepatocyte injury. Wild type FVB mice were controls. Results:Resulting Ad-Mdr2 −/− mice presented with cholangitis, fibrosis and cellular infiltrations that were higher than in Mdr2 −/− or Ad-FVB mice. Increased levels of antineutrophil cytoplasmic antibodies but similar anti-hCYP2D6 antibody titres were detected in Ad-Mdr2 −/− compared to Mdr2 −/− and Ad-FVB mice respectively. IFNγexpressing hCYP2D6-specific CD4 T cells declined, whereas hCYP2D6-specific CD8 T cells increased in Ad-Mdr2 −/− compared to Ad-FVB mice. The overall T cell balance in Ad-Mdr2 −/− mice was a combination of a type 17 T cell response typically found in Mdr2 −/− mice with a type 1 dominated T cell response characteristic for Ad-FVB mice.Simultaneously, the type 2 T cell compartment was markedly reduced. Conclusions:Experimental hepatitis induction in a mouse with sclerosing cholangitis results in a disorder which represents not simply the sum of the individual characteristics but depicts a more complex entity which urges on further analysis. K E Y W O R D S autoimmune hepatitis, autoimmune liver disease, primary sclerosing cholangitis S U PP O RTI N G I N FO R M ATI O N Additional supporting information may be found online in the Supporting Information section at the end of the article. How to cite this article: Fuchs S, Bayer M, Taubert R, et al. Effects of adenovirus-induced hepatocyte damage on chronic bile duct inflammation in a sclerosing cholangitis mouse model.

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Section: Discussionsupporting

confidence: 79%

Effects of adenovirus‐induced hepatocyte damage on chronic bile duct inflammation in a sclerosing cholangitis mouse model

Fuchs

Bayer

Taubert

et al. 2019

Liver International

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“…A menudo representan un desafío diagnóstico y terapéutico, ya que no está claro si son estos síndromes forman entidades distintas o son solo variantes o consecuencia de las principales enfermedades hepáticas autoinmunes 5 . Su importancia radica en que sus patrones bioquímicos y serológicos no son los clásicos o la respuesta terapéutica no sea la esperada 6 . La frecuencia de superposición de la hepatitis autoinmune con cirrosis biliar primaria es de 7-13%, mientras la superposición con colangitis esclerosante primaria es de 8-17% 1 .…”

Section: Discussionunclassified

Hepatitis Autoinmune y Colangitis Biliar Primaria en Paciente con Crioglobulinemia

Rocabado¹

2021

Gac Med Bol

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Paciente mujer de 55 años con antecedente de crioglobulinemia mixta con compromiso renal glomerulonefritis membranoproliferativa, enfermedad renal crónica (ERC) en estadio G5, presenta cuadro de ictericia, coluria los exámenes complementarios muestran incremento de las pruebas hepáticas con hipertransaminasemia, hiperbilirrubinemia y colestasis, serologías hepáticas virales negativas, anticuerpo antimitocondrial (AMA) +, se realiza biopsia hepática identificando hepatitis de interfase y en el interior de los septos fibrosos, reacción ductal focal y leve proliferación de ramas de la vena porta que están distorsionadas entre gruesas fibras de colágeno, llegando al diagnóstico de síndrome de superposición, se inicia tratamiento con corticoides 1 mgr/Kgr/día y ácido ursodeoxicólico 13 mgr/kgr/día con buena evolución, logrando normalizar las enzimas hepáticas. Ambas patologías “crioglobulinemia mixta y hepatopatías autoinmunes”, son entidades poco frecuentes que presentan buenas respuestas al tratamiento inmunosupresor e inmunomodulador.

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“…Its characteristic histopathological image is a florid bile duct lesion (degeneration of the biliary ductal epithelium with focal ductal obliteration and formation of granulomas with predominant portal distribution). 2,7,30 Currently, the only approved therapy is ursodeoxycholic acid. 28…”

Section: Primary Biliary Cholangitismentioning

confidence: 99%

Granulomatous liver disease

Ortíz-Brizuela¹,

Azamar-Llamas²,

Mora³

et al. 2023

GMM

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Granulomas are mainly composed of macrophages and T cells. The immune phenotype that triggers them and their histological appearance differ according to their etiology and to the host immune status. 1,4,5 For example, exposure to weakly antigenic agents results in the formation of persistent low-turnover granulomas (e.g., sutures), also called foreign-body granulomas (Figure 1). On the other hand, exposure to highly antigenic stimuli (e.g., Mycobacterium tuberculosis) results in additional activation of adaptive immune responses and in the formation of more complex granulomas (immune granulomas) (Figure 2). 1,6 Both, the innate and adaptive immune systems, are involved in their formation and maintenance. 7 In the case of Mycobacterium tuberculosis, its recognition through various macrophage receptors (e.g., complement receptor 4), among others, allows the T-helper cells activation into Th1, Th2, Th17 or regulatory T cells, as well as recruitment of other inflammatory cells (macrophages, neutrophils, B-cells). This results in the formation of the granuloma structure: a center composed

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Overlap syndromes of autoimmune hepatitis: diagnosis and treatment

Cited by 11 publications

References 55 publications

Effects of adenovirus‐induced hepatocyte damage on chronic bile duct inflammation in a sclerosing cholangitis mouse model

Effects of adenovirus‐induced hepatocyte damage on chronic bile duct inflammation in a sclerosing cholangitis mouse model

Hepatitis Autoinmune y Colangitis Biliar Primaria en Paciente con Crioglobulinemia

Granulomatous liver disease

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