Hepatite auto-imune em crianças e adolescentes: estudo clínico, diagnóstico e resposta terapêutica

Abstract

ResumoObjetivo: 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 autoanticorpos, 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 indesejá-vel do corticóide.J Pediatr (Rio J) 2002; 78 (4): 309-14: hepatite auto-imune, criança, adolescente, diagnóstico, tratamento. AbstractObjective: 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 treatm...

Effect of Treatment of Hepatic Histopathology in Children and Adolescents With Autoimmune Hepatitis

“…The immunosuppressant treatment used was an association of prednisone and azathioprine, according to the protocol adopted in our service (5):…”

“…Although AIH does not display pathognomonic histological features, it is characterized by the presence of an inflammatory infiltrate, with a predominance of mononuclear cells in the portal tracts, variable degrees of piecemeal necrosis (interface hepatitis), and frequent rosette formation. Cirrhosis has been the most frequent histopathological finding in the diagnosis of AIH, varying from 56.5% to 100% in the pediatric literature (1)(2)(3)(4)(5).…”

Desvendando a resposta autoimune contra o fígado: Hepatite Autoimune

“…Embora a etiologia exata da HAI ainda não esteja completamente elucidada, evidências sugerem uma interação complexa entre fatores genéticos, ambientais e imunológicos 1,2,3,4 .…”

Hepatite auto-imune tipo 1 em crianças e adolescentes: avaliação da suspensão do tratamento imunossupressor

“…8,12 By evaluating children and adolescents with AIH in our setting, we noted full therapeutic response in 77.8% of patients, but with complications arising from immunosuppressive therapy in 31.3% of the cases, and also height deficit indicated by the height/age z score. 19 Due to the deleterious effects of immunosuppressants and to the paucity of literature on the discontinuation of treatment in children and adolescents with AIH, 8,12 the present study aims to assess the possibility of children and adolescents with AIH showing remission of the disease after the withdrawal of immunosuppression. Thus, the aim of this study is to assess the discontinuation of immunosuppressive therapy in children and adolescents with AIH, who had clinical and laboratory remission of the disease within at least 24 months of treatment, determining the relapse rate after treatment withdrawal and the percentage of untreated patients that maintain remission.…”