Síndrome Inflamatória Multissistémica Pediátrica - Temporalmente Associada ao SARS-CoV-2: características clínicas e epidemiológicas

Abstract

Background: Paediatric Inflammatory Multisystem Syndrome (PIMS) is a multisysteminflammatory syndrome temporally associated to severe acute respiratory syndromecoronavirus 2 (SARS-CoV-2) infection. Because this syndrome was recently identified, it isimportant to contribute to the description of its clinical and epidemiological characteristics,which was the main objective of this study.Methods: Descriptive and retrospective study of PIMS’ cases in the reference hospital forpaediatric COVID-19 in the central region, between April 2020 and July 2021. The WorldHealth Organization’s case definition for PIMS was used.Results: Fifteen cases were identified, with a median age of 8 years (IQR:1,25-12). Ninewere male. Three had underlying comorbidities: one had a congenital complex cardiopathypreviously operated and thrombophilia, another had an alteration of haemostasis underinvestigation, and the other had treated and well controlled asthma. Nine patients presentedwith a Kawasaki-like phenotype. Eleven had gastrointestinal symptoms, with the diagnosis ofappendicitis being considered in three of them. One was submitted to surgical intervention.Five patients had signs of meningitis. Bilateral palpebral oedema was identified in six. Cardiacinvolvement occurred in 14/15; the median NT-proBNP was 3953 pg/mL (IQR: 388-34229),and six presented ultrasonographic anomalies (two had hypokinesis of the interventricularseptum, two had myocardial disfunction and two had pericardial effusion). Inflammatoryparameters were elevated in all cases. There was evidence of coagulopathy in thirteen (Ddimers median was 1780 ng/mL, IQR: 283-8617). All patients were hospitalized for a mean of6,3 days (SD±3,34). Three developed hypotension and/or shock and were admitted to theintensive care unit for inotropic support, two needed high-flow oxygen therapy. None requiredinvasive ventilation. One patient presented pulmonary interstitial syndrome, pleural effusion,and a lung consolidation and another acute glomerulonephritis. Every patient receivedantiaggregant/anticoagulant therapy. Corticotherapy alone was administered in four patients,due to stock rupture of immunoglobulin (Ig), Ig alone was administered in another four and anassociation of steroids and Ig was given to seven. Two sequalae were identified (myopathyand hypertension), with resolution. No deaths were registered, and every patient had afavourable outcome.Discussion: We verified a low occurrence of PIMS, with clinical characteristics that weresimilar to the ones described in other case series, but with a less severe presentation. Everypatient had a favourable clinical course, with resolution of the identified sequalae and withoutother short-medium term problems.Key Words: paediatrics; children; MIS-C; PIMS-TS; SARS-CoV-2

Introdução: A Paediatric Inflammatory Multisystem Syndrome (PIMS) é a síndromeinflamatória multissistémica temporalmente associada à infeção pelo severe acute respiratorysyndrome coronavirus 2 (SARS-CoV-2). Como entidade recente, é importante contribuir paraa sua caracterização clínica e epidemiológica, que foi o principal objetivo deste estudo.Métodos: Estudo descritivo e retrospetivo dos casos de PIMS do hospital de referência paraa COVID-19 pediátrica na região centro, entre abril de 2020 e julho de 2021. Foi utilizada adefinição de caso da Organização Mundial de Saúde.Resultados: Ocorreram quinze casos, com idade mediana de 8 anos (AIQ: 1,25-12). Noveeram do género masculino. Três tinham doença crónica: um com cardiopatia congénitacomplexa operada e trombofilia, outro com alteração da hemóstase em estudo e outra comasma controlada com terapêutica de fundo. Apresentaram fenótipo Kawasaki-like novedoentes. Onze apresentaram sintomas gastrointestinais, considerando-se a hipótese deapendicite aguda em três, tendo um sido submetido a intervenção cirúrgica. Cinco doentestinham sinais meníngeos e foi identificado edema palpebral bilateral em seis. Houveenvolvimento cardíaco em 14/15; a mediana do NT-proBNP foi 3953 pg/mL (AIQ: 388-34229),e seis apresentaram alterações ecográficas (dois com hipocinésia do septo interventricular,dois com disfunção miocárdica e dois com derrame pericárdico). Todos tiveram elevação dosparâmetros inflamatórios. Houve evidência de coagulopatia em treze (mediana D-dímeros1780 ng/mL, AIQ: 283-8617). Todos foram internados, em média, durante 6,3 dias (DP±3,34).Desenvolveram hipotensão e/ou choque três, admitidos em cuidados intensivos sob suporteinotrópico, dois sob oxigenoterapia high-flow. Nenhum necessitou de ventilação invasiva.Num ocorreu síndrome intersticial pulmonar, derrame pleural e condensação pulmonar enoutro glomerulonefrite aguda. Todos fizeram terapêutica antiagregante/anticoagulante. Foiutilizada corticoterapia isolada em quatro, em período de falha de stock de imunoglobulina(Ig), Ig isolada em quatro e associação de Ig e corticoide em sete. Foram registadas duassequelas (miopatia e hipertensão arterial), com resolução. Não foram registados óbitos etodos evoluíram bem.Discussão: Verificou-se ocorrência de poucos casos de PIMS, com características clínicassobreponíveis às descritas noutras séries, mas com quadros clínicos menos graves. Todosos doentes evoluíram favoravelmente, com resolução das sequelas registadas e sem outrasconsequências a curto-médio prazo.Palavras-chave: pediatria; crianças; MIS-C; PIMS-TS; SARS-CoV-2