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

Abstract

Suggested citation: Ferreira AR, Roquete ML, Penna FJ, Toppa NH, de Castro LP. Type 1 autoimmune hepatitis in children and adolescents: assessment of immunosuppressive treatment withdrawal. J Pediatr (Rio J). 2005;81:343-8. AbstractObjective: To assess treatment withdrawal in children and adolescents with autoimmune hepatitis, with clinical and laboratory remission for a minimum period of 24 months, determining the relapse rate after treatment withdrawal.Method: This is a descriptive, retrospective and partially prospective study of 21 children and adolescents with type 1 autoimmune hepatitis treated at the Outpatient Division of Pediatric Hepatology, Teaching Hospital of Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, Brazil, between January 1986 and December 2001. Results:We assessed 54 patients and selected 21, of whom 19 were female subjects (90.5%), aged between 5.7 and 17.6 years (median = 13.8 years), with a mean follow-up of 5.1±2.4 years (median = 4.4 years) and an average clinical and laboratory remission of 4.1±1.5 years (median = 4.1 years). Out of the 21 patients studied, 10 (47.6%) manifested some inflammatory activity that prevented the discontinuation of treatment, which was withdrawn in 11 patients (52.4%). Out of these, six patients (54.5%) presented reactivation of the disease and five maintained clinical and laboratory remission with a mean follow-up of 4±1 years (median = 3.9 years). The time interval between discontinuation of treatment and reactivation of the disease ranged from 29 days to 40.3 months (median = 2.2 months). Conclusions:We observed a high relapse rate (54.5%) in this group of patients with autoimmune hepatitis, which was more frequent within the first 12 months after treatment withdrawal, in addition to a high number of patients that presented some degree of inflammatory activity despite the long period of clinical and laboratory remission.

Autoimmune Hepatitis: Pathophysiology, Diagnosis and Pharmacological Therapy

“…However, the latter is a rare inflammatory disease [2], whose etiology is unknown albeit prevalent in 30-year-old females. In terms of laboratory diagnosis, the disease has been characterized by high serum transaminase and autoantibodies, which are used for disease classification [3][4][5].…”

“…This treatment results in greater resolution of symptoms and laboratory and histological abnormalities, and an increased life expectancy of patients [10]. The early suspension of treatment leads towards relapse and increased risk of progression towards cirrhosis [2].…”

Hepatite autoimune tipo 1 em crianças: fatores laboratoriais associados com a resposta histológica e evolução da doença

“…29 A suspensão do tratamento pode ser considerada nos pacientes que atingiram a remissão da doença clínica e histologicamente, pelo menos após dois anos de terapia com exames bioquímicos (AST, ALT) e IgG normais. 44 Todavia, a resolução da atividade inflamatória não garante a manutenção da remissão. 45,46 As recaídas e "retratamentos" estão associados a um pior prognóstico da HAI-1, aumentando o risco de progressão para cirrose hepática.…”

“…47 Em crianças as recaídas são frequentes, quer seja pela doença ou falta de adesividade adequada ao tratamento. 44 Seguimento clínico prolongado mostra que apenas poucas crianças conseguem parar completamente com o tratamento, e 70% mantém por toda a vida.…”

“…Existe uma dissociação entre laboratório e clínica em respeito à atividade histológica. A remissão histológica pode não ocorrer no mesmo momento, tendo sua resolução mais tardiamente ou até mesmo não ser possível 44. Para atingir este objetivo, podem-se aumentar as doses dos imunossupressores ou a duração, mas consequentemente aumentaríamos os efeitos deletérios, levando na infância a prejuízos no crescimento linear, desenvolvimento ósseo e aparência física.…”