Ricerca
Due anni di MIS-C: peculiarità cliniche e orizzonti terapeutici
Two years of MIS-C: clinical characteristics and therapeutic perspectives
Lorenzo Mambelli1, Andrea Uva1, Alessandra Iacono1, Lucia Del Vecchio2, Ilaria Bianchedi3, Annachiara Nuzzo4, Bruna Malta5, Federico Marchetti1
1UOC di Pediatria e Neonatologia, Ospedale di Ravenna, AUSL della Romagna
2Scuola di Specializzazione in Pediatria, Università di Bologna
3Scuola di Specializzazione in Pediatria, Università di Modena
4UOC di Cardiologia, 5UOC di Radiodiagnostica, Ospedale di Ravenna, AUSL della Romagna
Maggio 2022 - pagg. 299 -306 | DOI: 10.53126/MEB41299
Abstract
Introduction - MIS-C is the most relevant complication of SARS-CoV-2 infection and has an incidence of 1 case / 2,200 children and adolescents with previous infection. The clinical picture is characterized by a multiorgan involvement and an insufficiently known response to therapy.
Objectives - Retrospective analysis carried out in the Paediatric Department of the Ravenna Hospital from April 2020 to March 2022 of the cases admitted with MIS-C, with description of the presentation characteristics, the differences with respect to Kawasaki disease and the therapeutic strategies adopted also with regard to the clinical response.
Results - Thirteen cases diagnosed with MIS-C with an average age of 6 years and four months were hospitalized, 4 cases were of Italian origin, 6 of Balkan and 3 of African. All the cases presented with fever> 39 ° C with severe malaise / irritability, associated with skin rash in 9 cases, conjunctivitis in 7 and cheilitis in 5. The most prominent manifestations of organ involvement were abdominal pain / diarrhoea (N = 9), myocardial dysfunction in the absence of coronary involvement (N = 5), presence of pulmonary thickening (N = 5), painful laterocervical lymphadenitis with reduced neck motility (N = 5), subnephrotic proteinuria (N = 7) and hypertransaminasaemia (N = 8). Two cases with “encephalitic-like” psychomotor slowing, one with important ascites and one with oedema of the para/retropharyngeal tissues were clinically relevant for the severity of the onset. Twelve cases were treated with IVIG and methylprednisolone (2 mg /kg), 4 of them were found to be non-responders and required the use of steroid boluses (4 cases) and anakinra (4 cases), in three of the latter in association with steroid boluses, with rapid and decisive clinical response.
Conclusions - The serious clinical manifestations that characterize MIS-C do not concern only cardiac involvement. Therapy with an IL-1 inhibitor, anakinra, must also be envisaged as a possible first-line treatment in case of a particularly severe clinical picture at the onset.
Objectives - Retrospective analysis carried out in the Paediatric Department of the Ravenna Hospital from April 2020 to March 2022 of the cases admitted with MIS-C, with description of the presentation characteristics, the differences with respect to Kawasaki disease and the therapeutic strategies adopted also with regard to the clinical response.
Results - Thirteen cases diagnosed with MIS-C with an average age of 6 years and four months were hospitalized, 4 cases were of Italian origin, 6 of Balkan and 3 of African. All the cases presented with fever> 39 ° C with severe malaise / irritability, associated with skin rash in 9 cases, conjunctivitis in 7 and cheilitis in 5. The most prominent manifestations of organ involvement were abdominal pain / diarrhoea (N = 9), myocardial dysfunction in the absence of coronary involvement (N = 5), presence of pulmonary thickening (N = 5), painful laterocervical lymphadenitis with reduced neck motility (N = 5), subnephrotic proteinuria (N = 7) and hypertransaminasaemia (N = 8). Two cases with “encephalitic-like” psychomotor slowing, one with important ascites and one with oedema of the para/retropharyngeal tissues were clinically relevant for the severity of the onset. Twelve cases were treated with IVIG and methylprednisolone (2 mg /kg), 4 of them were found to be non-responders and required the use of steroid boluses (4 cases) and anakinra (4 cases), in three of the latter in association with steroid boluses, with rapid and decisive clinical response.
Conclusions - The serious clinical manifestations that characterize MIS-C do not concern only cardiac involvement. Therapy with an IL-1 inhibitor, anakinra, must also be envisaged as a possible first-line treatment in case of a particularly severe clinical picture at the onset.
Riassunto
Introduzione - La MIS-C è la complicanza più rilevante della infezione da SARS-CoV-2 ed ha una incidenza di 1 caso/2200 bambini e adolescenti con pregressa infezione. Il quadro clinico si caratterizza per un interessamento multiorgano e una risposta alla terapia non sufficientemente nota.
Obiettivi - analisi retrospettiva effettuata nel Reparto di Pediatria dell’Ospedale di Ravenna da Aprile 2020 a Marzo 2022 dei casi ricoverati con MIS-C, con descrizione delle caratteristiche di presentazione, le differenze rispetto alla malattia di Kawasaki e le strategie terapeutiche adottate in merito anche alla risposta clinica.
Risultati - Sono stati ricoverati 13 casi con diagnosi di MIS-C con una età media di 6 anni 4/12°, di origine italiana in 4 casi, balcanica in 6 e africana in 3. Tutti i casi presentavano febbre >39°C con importante malessere/irritabilità associata a rash cutaneo in 9 casi presentavano, a congiuntivite in 7 e a cheilite in 5. Le manifestazioni di interessamento di organo più rilevanti sono state il dolore addominale/diarrea (N=9), disfunzione miocardica in assenza di coinvolgimento coronarico (N=5), presenza di addensamenti polmonari (N=5), linfadenite laterocervicale dolente con motilità ridotta del collo (N=5), proteinuria subnefrosica (N=7), ipertransaminasemia (N=8). Clinicamente rilevanti per la severità all'esordio due casi con rallentamento psicomotorio "simil encefalitico", e singoli casi con: importante ascite, edema dei tessuti para/retrofaringei. Dodici casi sono stati trattati con IVIG e metilprednisolone (2 mg/kg) e 1/3 dei casi è risultato essere non responder, con necessità di utilizzo dei boli di steroide (4 casi) e dell’anakinra (4 casi), in tre di questi ultimi in associazione con boli di steroide, con rapida e risolutiva risposta clinica.
Conclusioni - Le manifestazioni cliniche gravi che caratterizzano la MIS-C non riguardano solo l’interessamento cardiaco. La terapia con inibitore dell’IL-1, anakinra, deve essere prevista anche come possibile trattamento di prima linea in caso di quadro clinico particolarmente severo all'esordio.
Obiettivi - analisi retrospettiva effettuata nel Reparto di Pediatria dell’Ospedale di Ravenna da Aprile 2020 a Marzo 2022 dei casi ricoverati con MIS-C, con descrizione delle caratteristiche di presentazione, le differenze rispetto alla malattia di Kawasaki e le strategie terapeutiche adottate in merito anche alla risposta clinica.
Risultati - Sono stati ricoverati 13 casi con diagnosi di MIS-C con una età media di 6 anni 4/12°, di origine italiana in 4 casi, balcanica in 6 e africana in 3. Tutti i casi presentavano febbre >39°C con importante malessere/irritabilità associata a rash cutaneo in 9 casi presentavano, a congiuntivite in 7 e a cheilite in 5. Le manifestazioni di interessamento di organo più rilevanti sono state il dolore addominale/diarrea (N=9), disfunzione miocardica in assenza di coinvolgimento coronarico (N=5), presenza di addensamenti polmonari (N=5), linfadenite laterocervicale dolente con motilità ridotta del collo (N=5), proteinuria subnefrosica (N=7), ipertransaminasemia (N=8). Clinicamente rilevanti per la severità all'esordio due casi con rallentamento psicomotorio "simil encefalitico", e singoli casi con: importante ascite, edema dei tessuti para/retrofaringei. Dodici casi sono stati trattati con IVIG e metilprednisolone (2 mg/kg) e 1/3 dei casi è risultato essere non responder, con necessità di utilizzo dei boli di steroide (4 casi) e dell’anakinra (4 casi), in tre di questi ultimi in associazione con boli di steroide, con rapida e risolutiva risposta clinica.
Conclusioni - Le manifestazioni cliniche gravi che caratterizzano la MIS-C non riguardano solo l’interessamento cardiaco. La terapia con inibitore dell’IL-1, anakinra, deve essere prevista anche come possibile trattamento di prima linea in caso di quadro clinico particolarmente severo all'esordio.
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Corrispondenza: lorenzo.mambelli@auslromagna.it