1 - Unidade de Infecciologia Pediátrica, Hospital Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE, Lisboa
2 - Unidade de Reumatologia Pediátrica, Hospital Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE, Lisboa
3 - Serviço de Cardiologia Pediátrica, Hospital de Santa Marta, Centro Hospitalar de Lisboa Central, EPE, Lisboa
- European Society for Paediatrica Infectious Diseases Dublin, 2014 (Poster)
Resumo:
Introdução: Kawasaki disease (KD) is a self-limited systemic pediatric vasculitis. Its aetiology remains unknown. Current estimates indicate that 10 to 20% patients do not respond to single dose of intravenous immunoglobulin (IVIG). The optimal therapy of these refractory cases is still controversial. Relato do Caso Eight month-old boy presents with 3 days lasting fever, polymorphous rash, bilateral nonsuppurative conjunctivitis, cervical lymphadenopathy, dry fissured lips, strawberry tongue, BCGitis and periungueal desquamation. Coronary evaluation was initially normal but aneurysms were later detectable with left coronary artery (3,5mm), anterior descending artery (3,3mm) and right coronary artery (3,7mm) involvement. Aspirin and IVIG (2g/kg) were administered on day 4. On the7th day of illness fever and extremities changes recurred and warranted a new IVIG dose. On day 15th, due to reappearance of fever methylprednisolone (30mg/Kg) was administered for 5 days. Three days after corticosteroid weaning fever recurred and infliximab (5mg/kg) was given on day 32nd, with defervescence, reduction of inflammatory markers and coronary aneurisms.
Conclusões: The present case presented without several risk factors for severe disease and was refractory to standard treatment. Indeed, inflammatory response was only blocked by Infliximab. TNF-a blockade seems to be a good therapeutic option for refractory and/or high-risk patients, however a need for multicentre, randomized trials is imperative
Palavras Chave: Doença de Kawasaki, Infliximab.