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

Ferreira, Alexandre Rodrígues; Roquete, Mariza Leitão Valadares; Toppa, Nivaldo Hartung; Castro, Lúcia Porto Fonseca de; Fagundes, Eleonora Druve Tavares; Penna, Francisco José

doi:10.1097/01.mpg.0000304456.84552.13

Cited by 47 publications

(31 citation statements)

References 31 publications

(42 reference statements)

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“…18 Clinical and biochemical remission do not necessarily reflect histologic resolution, given that the histopathologic changes take longer. 19 It is stated that after 4 years of treatment, improvement in fibrosis and the intensity of portal inflammation is observed in more than 95% of the cases.…”

Section: Discussionmentioning

confidence: 99%

Autoimmune hepatitis in children: Progression of 20 cases in northern Mexico

Nares-Cisneros

Jaramillo-Rodríguez

2014

Revista de Gastroenterología de México (English Edition)

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Background: Autoimmune hepatitis (AIH) is a chronic inflammatory disease of the liver with nonspecific clinical manifestations that causes greater liver damage in children than in adults. Aims: To analyze the clinical progression, biochemical profiles, histopathologic changes, and treatment response in 20 children with AIH. Material and methods: A retrospective study was carried out on the variables associated with clinical progression, diagnosis, and treatment response in children seen at the the Unidad Médica de Alta Especialidad (UMAE) No. 71 IMSS in Torreón, Coahuila, Mexico, from 1992 to 2012. Results: Twenty patients were analyzed, 75% with type 1 AIH (AIH-1) and 25% with type 2 AIH (AIH-2). Girls predominated with a 3:1 ratio of girls to boys. The mean age was 10.07 ± 6.53 years for the AIH-1 cases and 6.75 ± 3.77 years for the AIH-2 cases. There was an association with immunologic diseases in 40% of the patients.The patients in the AIH-2 group had greater biochemical profile alterations and IgA deficiency. Anti-nuclear antibody and anti-smooth muscle antibody were positive in 100% of the patients with AIH-1, and anti-liver kidney microsomal type 1 antibody was positive in 100% of the AIH-2 patients. Liver biopsy revealed interface hepatitis in both groups. The AIH-2 group responded more quickly to treatment, but had a higher recurrence rate.Conclusions: Autoimmune hepatitis in the pediatric patient should be suspected in order to make an early diagnosis and thereby establish opportune treatment. Determining the type of AIH is necessary for making adequate diagnosis and for achieving a better outcome in relation to recurrence and complication rates. Hepatitis autoinmune en niños: evolución de 20 casos del norte de MéxicoResumen Antecedentes: La hepatitis autoinmune (HAI) es una enfermedad inflamatoria crónica del hígado con manifestaciones clínicas inespecíficas y mayor daño hepático en niños que en adultos. Objetivo: Analizar la evolución clínica, los perfiles bioquímicos, los cambios histopatológicos y la respuesta al tratamiento de 20 niños con HAI. Material y métodos: Estudio retrospectivo de las variables asociadas a evolución clínica, diagnóstico y respuesta al tratamiento en niños atendidos en la UMAE n. • 71 IMSS Torreón, Coahuila, México, en el período comprendido entre 1992 y 2012. Resultados: Se analizó a 20 pacientes, el 75% con HAI-1 y el 25% con HA1-2. Se observó predominio del sexo femenino 3:1. La edad promedio ± desviación estándar fue de 10.07 ± 6.53 años para HAI-1 y de 6.75 ± 3.77 para HAI-2. La asociación a enfermedades inmunológicas fue del 40%.Los pacientes del grupo HAI-2 mostraron mayores alteraciones en su perfil bioquímico y deficiencia de IgA. La positividad para anticuerpos antinucleares y anticuerpos antimúsculo liso fue del 100% en los pacientes con HAI-1, anticuerpos antimicrosomales para hígado y riñón tipo 1 en el 100% de HAI-2. Las biopsias hepáticas mostraron hepatitis de interfase en ambos grupos. Los pacientes del grupo HAI-2 respondieron más rápidamente al tratamiento...

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Section: Discussionmentioning

confidence: 99%

Autoimmune hepatitis in children: Progression of 20 cases in northern Mexico

Nares-Cisneros

Jaramillo-Rodríguez

2014

Revista de Gastroenterología de México (English Edition)

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“…The histological response lags behind the biochemical response [20] and clinical/biochemical remission does not necessarily reflect histological resolution. After a mean duration of 4 years of treatment, improvement of the intensity of portal inflammation is observed in up to 95% of AIH cases and is accompanied by an improvement of fibrosis scores [20]. Relapse is characterized by an increase of serum aminotransferase levels after remission has been achieved.…”

Section: Treatmentmentioning

confidence: 94%

Autoimmune hepatitis: Contrasts and comparisons in children and adults – A comprehensive review

Floreani

Liberal

Vergani

et al. 2013

Journal of Autoimmunity

105

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Section: Treatmentmentioning

confidence: 99%

“…Clinical and biochemical remission does not equate histological resolution, the histological response lagging behind the biochemical response 29. An improvement of portal inflammation is observed in up to 95% of AIH cases after ∼4 years of treatment and is accompanied by improved fibrosis scores 29. Relapse during treatment (ie, increased serum aminotransferase levels after remission has been achieved) is common, affecting about 40% of patients and requiring a temporary increase in immunosuppression 4.…”

Section: Treatmentmentioning

confidence: 99%

Paediatric autoimmune liver disease

Mieli‐Vergani

Vergani

2013

Arch Dis Child

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Autoimmune liver disorders in childhood include autoimmune hepatitis (AIH) and AIH/sclerosing cholangitis overlap syndrome (henceforth referred to as autoimmune sclerosing cholangitis, ASC). These inflammatory liver disorders are characterised histologically by interface hepatitis, biochemically by elevated transaminase levels, and serologically by autoantibodies and increased levels of immunoglobulin G. AIH is particularly aggressive in children and progresses rapidly unless immunosuppressive treatment is started promptly. With appropriate treatment, 80% of patients achieve remission and long-term survival. For non-responders and difficult-to-treat patients, novel and more effective therapeutic approaches are sought. ASC responds to the same treatment used for AIH in regards to parenchymal inflammation, but bile duct disease progresses in about 50% of cases, leading to a worse prognosis and a higher liver transplantation requirement; moreover, it has a high recurrence rate after transplant. Progression of liver disease and recurrence after transplant are more common in patients with associated poorly controlled inflammatory bowel disease. Though the mechanisms underlying the pathogenesis of liver autoimmunity are not fully understood, genetic and environmental factors are likely to be involved. A deeper understanding of the pathogenesis of these conditions will contribute to the development of novel treatments, aimed ultimately at the restoration of tolerance to liver-derived antigens.

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

Cited by 47 publications

References 31 publications

Autoimmune hepatitis in children: Progression of 20 cases in northern Mexico

Autoimmune hepatitis in children: Progression of 20 cases in northern Mexico

Autoimmune hepatitis: Contrasts and comparisons in children and adults – A comprehensive review

Paediatric autoimmune liver disease

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