Objetivo: avaliar o quadro clínico, laboratorial, histopatológico e a resposta ao tratamento imunossupressor em crianças e adolescentes com hepatite auto-imune. Métodos: estudo descritivo de trinta e nove crianças e adolescentes, atendidos no setor de gastroenterologia pediátrica do Hospital das Clínicas da UFMG, no período de 1986 a 1998. Resultados: a idade variou de 1,6 a 17 anos (média 8,7±3,49), com predomínio do sexo feminino (87,2%). Ocorreram três formas de apresentação clínica: crônica (53,9%), aguda (41%) e falência hepática grave (5,1%). A alteração laboratorial mais relevante foi a elevação das aminotransferases e gama-globulina. O fator antinuclear foi positivo em 66,7% dos pacientes, antimúsculo liso em 52,8%, e o antimicrossomal de fígado e rim em 3%. Na histopatologia, destacaram-se a necrose em saca-bocados (93,7%), inflamação portal moderada a acentuada (78,1%), cirrose definitiva e/ou incompleta (76,9%), ausência de lesões dos ductos biliares (93,7%) e presença de rosetas (90,6%). No tratamento, 77,8% obtiveram resposta completa, com efeitos colaterais em 27,8% dos pacientes. Ocorreram sete (17,9%) óbitos. No decurso do tratamento, ocorreu redução com significância estatítica do escore z para estatura/idade. Conclusões: a hepatite auto-imune predomina no sexo feminino, com formas de apresentação clínica variada, presença de auto-anticorpos, elevação das aminotransferases e hipergamaglobulinemia. Predomínio histopatológico de cirrose incipiente e/ou definitiva, com inflamação portal de moderada a acentuada e necrose em saca-bocados. O tratamento com corticosteróide e azatioprina em doses diárias revelou-se efetivo, todavia, a diminuição do escore z para estatura/idade representa, provavelmente, um efeito indesejável do corticóide.
Objective: the aim of this study was to evaluate the clinical, laboratory and histopathological characteristics and the response to immunosuppression in children and adolescents with autoimmune hepatitis (AIH). Methods: the present research is a descriptive study consisting of 39 children and adolescents with AIH who receive care at the Department of Pediatric Gastroenterology of Hospital das Clínicas (UFMG) from 1986 to 1998. Results: children's age ranged from 1.6 to 17 years (mean 8.7 ± 3.49), most of them were females (87.2%). There were three types of clinical presentations: chronic (53.9%), acute (41%), and serious hepatic failure (5.1%). The most relevant laboratory parameters were the aminotransferases and g-globulin increase. Antinuclear antibodies were positive in 66.7% of the patients, while smooth muscle antibodies were positive in 52.8% and anti-LKM1 in 3% of the patients. In the histopathology the most important findings were the piecemeal necrosis (93.7%), moderate to severe portal inflammation (78.1%), definitive or incomplete cirrhosis (76.9%), absence of lesion of biliary ducts (93.7%) and presence of rosettes (90.6%). During the treatment, 77.8% obtained complete resolution, associated to side effects in 27.8% of them. Seven patients died (17.9%). During the treatment there was significant z score reduction (p< 0.05) for height/age. Conclusions: after carrying out this study, we observed that the typical characteristics of AIH were: female sex, several clinical presentations, increased aminotransferase, and hypergammaglobulinemia. Histopathology showed a predominance of incipient and/or definitive cirrhosis associated with moderate to severe portal inflammation and piecemeal necrosis. Treatment using corticosteroids and azathioprine, turned out to be effective. However, the reduction in the height/age z score probably represents an adverse effect of corticoid treatment.