Autoimmune hepatitis, one disease with many faces: Etiopathogenetic, clinico-laboratory and histological characteristics

Abstract

Autoimmune hepatitis (AIH) is an unresolving progressive liver disease of unknown etiology characterized by hypergammaglobulinemia, autoantibodies detection and interface hepatitis. Due to the absence of specific diagnostic markers and the large heterogeneity of its clinical, laboratory and histological features, AIH diagnosis may be potentially difficult. Therefore, in this in-depth review we summarize the substantial progress on etiopathogenesis, clinical, serological and histological phenotypes of AIH. AIH has a global distribution affecting any age, both sexes and all ethnic groups. Clinical manifestations vary from asymptomatic to severe or rarely fulminant hepatitis. Hypergammaglobulinemia with selective elevation of IgG is found in most cases. Autoimmune attack is perpetuated, possibly via molecular mimicry, and favored by the impaired control of T-regulatory cells. Histology (interface hepatitis, emperipolesis and hepatic rosette formation) and autoantibodies detection although not pathognomonic, are still the hallmark for a timely diagnosis. AIH remains a major diagnostic challenge. AIH should be considered in every case in the absence of viral, metabolic, genetic and toxic etiology of chronic or acute hepatitis. Laboratory personnel, hepato-pathologists and clinicians need to become more familiar with disease expressions and the interpretation of liver histology and autoimmune serology to derive maximum benefit for the patient.

Autoimmune hepatitis: review of histologic features included in the simplified criteria proposed by the international autoimmune hepatitis group and proposal for new histologic criteria

“…Using the original histologic criteria, simplified score of 46 was observed in 69% of autoimmune hepatitis cases, which is lower than observed in the original study but within the 65-95% range reported in literature. 3,4,6 When the total simplified score was calculated on the basis of the modified histologic criteria, there was an increase of autoimmune hepatitis cases with score 46 to 86%. In other words, 17% of autoimmune hepatitis cases that would have been labeled as non-autoimmune hepatitis based on the simplified criteria using the current histologic features would have been classified as probable or definite autoimmune hepatitis by the use of modified histologic features.…”

“…3,6 Multiple studies have established the key role of liver biopsy in autoimmune hepatitis, 3,10,16 and the practice guidelines of American Association for the Study of Liver Diseases as well as European Association for Study of Liver advocate the use of liver biopsy for diagnosis and treatment decisions. 25,26 The simplified criteria proposed by International Autoimmune Hepatitis Group mandate the presence of three histologic features in the same biopsy for the Autoimmune hepatitis: histologic criteria proposal case to qualify as a histologically 'typical' (2 points): interface lymphocytic/lymphoplasmacytic inflammatory activity, rosettes, and emperipolesis.…”

“…3,5 The sensitivity for diagnosis of autoimmune hepatitis is somewhat limited, with reported numbers ranging from 65 to 95% for probable autoimmune hepatitis (score 46) and from 15 to 87% for definite autoimmune hepatitis (score 47). 3,4,6 The International Autoimmune Hepatitis Group criteria do not perform well in making the diagnosis of autoimmune hepatitis in specific situations such as cases with acute presentation, 5,7,8 overlap syndromes, 5,9,10 and in the setting of concurrent diseases like nonalcoholic fatty liver disease. 11 Autoimmune hepatitis with acute presentation clinically present as acute hepatitis; a subset of these cases show histologic features of chronicity such as fibrosis on biopsy, while others resemble acute hepatitis on histology as well.…”

Bacteremia por Aeromonas caviae en paciente con hepatitis autoinmune: presentación de caso

“…A pesar de no conocerse la causa específica de la enfermedad, se considera que se desarrolla en personas con predisposición genética que se exponen a algún agente desencadenante como bacterias, virus, hongos u otras partículas externas al organismo 9,10 ; la caracterización del cuadro clínico es de amplio rango presentándose desde formas asintomáticas solo detectables por elevaciones moderadas de las aminotransferasas hasta casos de hepatitis severa fulminante 1, 11,12 . De esta forma, la mayoría de los pacientes son asintomáticos o presentan formas leves de la enfermedad, siendo los síntomas generales inespecíficos como astenia, fatiga y debilidad un motivo de consulta frecuente.…”

Role of Th1/Th2 cells and related cytokines in autoimmune hepatitis

“…The mechanisms underlying the onset of autoimmune hepatitis remain unclear. However, the main hypothesis postulates that disease susceptibility is affected by both genetic and environmental factors that lead to a loss of tolerance towards liver antigens (2).…”