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Orientador(es)
Resumo(s)
Deficiency of adenosine deaminase 2 (DADA2), first reported in 2014, is a disease with great phenotypic variability, which has been increasingly reported. Therapeutic response depends on the phenotype. We present a case of an adolescent with recurrent fever, oral aphthous ulcers, and lymphadenopathy from 8 to 12 years of age and subsequently presented with symptomatic neutropenia. After the diagnosis of DADA2, therapy with infliximab was started, but after the second dose, she developed leukocytoclastic vasculitis and showed symptoms of myopericarditis. Infliximab was switched to etanercept, with no relapses. Despite the safety of tumor necrosis factor alpha inhibitors (TNFi), paradoxical adverse effects have been increasingly reported. The differential diagnosis between disease new-onset manifestations of DADA2 and side effects of TNFi can be challenging and warrants further clarification.
Descrição
Palavras-chave
DADA2 pericarditis leukocytoclastic vasculitis infliximab anti-TNF-α
Contexto Educativo
Citação
Simão Raimundo D, Cordeiro AI, Parente Freixo J, Valente Pinto M, Neves C and Farela Neves J (2023) Case Report: Patient with deficiency of ADA2 presenting leukocytoclastic vasculitis and pericarditis during infliximab treatment. Front. Pediatr. 11:1200401. doi: 10.3389/fped.2023.1200401
Editora
Frontiers Media
