Malattia di Kawasaki: sintomi e trattamento
Table of Contents
Definizione
Lamalattia di Kawasaki, è una vasculite necrotizzante dei vasi di medio e piccolo calibro(arteriole, vene e capillari), che agisce con un pattern pressoché costante sulle arterie coronarie, producendo in forma tardiva, se non trattata aneurismi multipli (a “corona di rosario”).
Eziologia
Sconosciuta. Si suppone la partecipazione di unsuperantigenesimile alla tossina della sindrome da shock tossico stafilococcico, in grado di indurre un’ attivazione del sistema immunitario, con successiva danno endoteliale.
Epidemiologia
Lamalattia di Kawasakicolpisceiminori di 5 anni, con prevalenza nella popolazione maschile. Più frequente In Giappone. E’ la causa più frequente di cardiopatia acquisita nei bambini.
Segni clinici
Criteri diagnostici
- Febbrealta superiore a 5 giorni con scarsa risposta agli antipiretici;
- Congiuntivitebilaterale non purulenta
- Interessamento dellamucosa orofaringea, con labbra eritematose, secche e screpolate, lingua a fragola;
- Adenopatia cervicale>1,5 cm, unilaterale, non dolorosa né suppurativa;
- Esantema multiforme, non vescicolare, con esordio sul tronco;
- Edema o eritemasu mani e piedi in fase acuta desquamazione periungueale in fase tardiva;
- Può associarsi airidociclite, alterazione articolare irritabilità.
- Possono essere coinvolti altri organi (vasculite).
Esistono delle forme di malattia di Kawasaki che non si manifestano con i criteri classici.
In particolare
- Forma incompleta.È tipica dei bambini inferiori a 12 mesi confebbre, segni diinfiammazione sistemicasenza causa ben determinata e riscontro di aneurismi coronarici, in assenza degli altri criteri diagnostici.
- Forma atipica: nei bambini in cui si rilevano in associazione allafebbree aglianeurismi coronarici,segni clinici non inclusi nei criteri diagnostici (per esempio segni di coinvolgimento renale, pancreatite, polmonite a lenta risoluzione, paralisi del facciale ecc..)
Complicanze
Coinvolgimento cardiaco sotto forma divasculite coronarica con formazione di aneurismi. Si caratterizza per un esordio tardivo. A lungo termine può complicarsi con trombosi o stenosi arteriosa coronarica, la rottura di aneurisma, pericardite,miocarditeeendocardite,insufficienza cardiacaearitmie, che possono avere esiti fatali. Data la sua gravità ogni volta rivestono maggiore importanza e la diagnosi e il trattamento precoce della malattia di Kawasaki, pertanto, a fronte a un un sospetto anche se non si soddisfano i criteri diagnostici il trattamento è indicato.
Diagnosi
Clinica :febbre e almeno quattro degli altri criteri.
Laboratorio: leucocitosi, trombocitosi, lieve proteinuria, piuria, pleiocitosi livelli di bilirubina e transaminasi risulta nettamente aumentati. la presenza di dell’LRC. I livelli di birilubina e transaminasi risultano lievemente aumentati. L’albumina ridotta indica malattia più grave e prolungata.
Ecocardiografiabidimensionale:utile a conferma della diagnosi di malattia di Kawasaki per la determinazione dell’interessamento cardiaco. E’obbligatorio al momento della diagnosi e a 2-4-8 settimane di distanza, in quanto gli aneurismi possono comparire anche a distanza di tempo.
Biopsia:infiltrato infiammatorio nella tonaca media e intima delle arterie di medio calibro (coronarie) con occlusione da trombi piastrinici.
Prognosi
Favorevole in assenza di interessamento cardiaco. Mortalità correlata a coinvolgimento coronarico. Gli aneurismi scompaiono generalmente a 2 anni nel 50% dei casi.
Trattamento
Immunoglobulineumane ev: hanno un effetto antinfiammatorio e consentono di ridurre il rischio di formazione di aneurismi coronarici, se somministrate entro il 10° giorno di malattia.
Salicilatia dosi antinfiammatorie nella fase febbrile e a dosi antiaggreganti per 6-8 settimane (in assenza di lesioni coronariche) o fino alla scomparsa delle lesioni coronariche. In caso di pazienti non responder può essere indicato l’utilizzo di infliximab (anticorpo monoclonale anti-TNFalfa).
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