Evaluation of Patients With Autoimmune Hepatitis in a Specialized Outpatient Clinic in Southern Brazil

Abstract

BACKGROUND: Autoimmune hepatitis (AIH) is a chronic liver disease, characterized by necroinflammation and autoimmune etiology. Studies evaluating the characteristics of patients with AIH are scarce in Brazil. OBJECTIVE: Our objective was to evaluate the profile of patients with AIH in a specialized center in Southern Brazil and to verify factors related to treatment response. METHODS: this was a retrospective cohort study, which analyzed demographic, epidemiological, clinical, laboratory, and histologic data. Patients with AIH diagnosed according to the criteria of the International Autoimmune Hepatitis Group (IAIHG) were included. In liver biopsies, the degree of fibrosis, histological activity, presence of hepatocyte rosettes, plasma cell infiltrates, and confluent necrosis were evaluated. In the statistical analysis, the significance level was 5%. RESULTS: Forty adults patients diagnosed with AIH were included. The evaluated population predominantly consisted of women (75.0%) and the average age at diagnosis was 44.2 years. The association with extrahepatic autoimmune diseases occurred in 20.0% of cases. Clinically, 35.0% of patients presented with acute onset hepatitis, 37.5% with cirrhosis, and 27.5% with other forms of presentation. The most common clinical manifestation was jaundice (47.5%). Thirty-five patients were treated, and of these, 97.1% used prednisone combined with azathioprine. The average treatment time was 2.7 years. Response to treatment was complete or partial in 30 (85.7%) and absent in 5 (14.3%) patients. There was no statistically significant difference when evaluating response to treatment in relation to forms of presentation, histological findings, and the presence of autoantibodies. Regarding fibrosis, regression was observed in 18.75% of the cases. CONCLUSION: Most patients with AIH were young at presentation and of female sex. The association with extrahepatic autoimmune diseases and cirrhosis at presentation was seen in a considerable proportion of patients. Treatment was effective, but there were no clinical, histological or serological parameters capable of predicting treatment response.

Clinical Characteristics of Autoimmune Hepatitis in a Middle Eastern Population: A Tertiary Care Center Experience

“…Previous observations of female predominance in AIH can be attributed to immunogenetic factors and sex hormones, despite the enigmatic nature of such a pattern of sex difference in autoimmunity in general [ 14 , 38 ]. The pattern of female predominance was also seen among AIH patients in previous and recent studies from the US and UK (77%), Italy (88%), Japan (79%), Saudi Arabia (76%), Brazil (75%), and Egypt (60% in a small sample of children) [ 29 , 35 , 39 , 40 , 41 , 42 , 43 ]. Additionally, and consistent with the previous literature, a large variability in terms of age at time of diagnosis was observed in this cohort, and age ranged from as young as 16 years up to 82 years.…”

Hepatite autoimune pediátrica: necessidade do diagnóstico precoce e tratamento oportuno

“…A HAI mostra ter uma significativa associação a outras doenças autoimunes extra-hepáticas, sendo mais recorrente em pacientes do sexo femenino (FEDRIZZI RS, et al, 2020). As doenças autoimunes extrahepáticas mais comuns relatadas incluem: doenças da tireóide; doenças autoimunes da pele; doenças celíacas; doenças inflamatórias intestinais; vitiligo, diabetes mellitus tipo 1, doença mista do tecido conjuntivo, artrite, síndrome de Sjögren, trombocitopenia inmune primária, anemia hemolítica, esclerose múltipla, síndrome poliglandular autoimune (AFAA TJ, et al, 2020; VÉLEZ RP, 2017; MIRANDA EA, 2017).…”

Lactobacillus improves the effects of prednisone on autoimmune hepatitis via gut microbiota-mediated follicular helper T cells

“…It is characterized by increased hypergammaglobulinaemia, damaged hepatocytes, and positive circulating autoantibodies [ 2 , 3 ]. Prednisone (CAS: 53-03-2), a synthetic glucocorticoid with anti-inflammatory and immunomodulating properties, is widely used for treatment of AIH [ 4 – 6 ]. Autoantibodies are responsible for the pathogenesis of AIH, which decorate hepatocytes and induce complement-mediated cell lysis [ 7 ].…”