La malattia reumatica è un processo morboso infiammatorio multifocale, a patogenesi autoimmune, che si manifesta in seguito ad un’ infezione faringea da streptococco beta emolitico del gruppo A.
La malattia interessa principalmente le articolazioni, il cuore, il sistema nervoso centrale, la cute e il sottocutaneo. Il 50% circa dei pazienti colpito dalla malattia reumatica sviluppa negli anni un danno cardiaco permanente, responsabile delle varie forme di valvulopatia reumatica cronica.
Epidemiologia
L’incidenza della malattia reumatica è diminuita drasticamente nei paesi industrializzati grazie alle migliorate condizioni socio-economiche e alla disponibilità della penicillina per il trattamento della faringite streptococcica. La malattia è ancora presente in forma endemica nei paesi in via di sviluppo e tra le popolazioni in cui le condizioni ambientali e socio-sanitarie sono precarie (povertà, malnutrizione, eccessivo affollamento, insufficiente prevenzione di assistenza sanitaria). Sebbene possa interessare tutte le fasce di età, la malattia reumatica colpisce principalmente i bambini e gli adolescenti. La prevalenza della valvulopatia reumatica, al contrario, aumenta con l’età e raggiunge un picco tra i 25-34 anni.
Patogenesi
La faringo-tonsillite da streptococco beta emoliticodel gruppo A, non adeguatamente trattata con antibiotici, è l’evento che precipita la malattia reumatica. Sebbene l’esatto meccanismo che associa
l’ infezione streptococcica alla flogosi reumatica sia ancora incerto, la malattia reumatica è comunemente considerata il risultato di una esagerata risposta immunitaria alle componenti antigeniche dello streptococco. Le similitudini molecolari e immunologiche tra gli antigeni batterici e i tessuti dell’organismo sarebbero poi responsabili della successiva risposta crociata di tipo autoimmune che scatena l’ attacco acuto di malattia reumatica.
Nel quadro clinico della malattia emergono i cinque elementi fondamentali per la diagnosi:
cardite
poliartrite
corea di Sydenham
eritema marginato
noduli sottocutanei.
Questi elementi possono presentarsi singolarmente o in combinazione tra loro e costituiscono nel loro insieme i cosiddetti criteri maggiori di Jones.
Altri reperti come.
febbre
artralgie
positività dei test ematochimici di flogosi acuta
allungamento del intervallo P-R alll’ECG
sono considerati invece manifestazioni minori della malattia.
Sono necessari almeno 2 criteri maggiori o 1 maggiore e 2 minori insieme alla dimostrazione di infezione pregressa da SBEA per fare diagnosi di malattia reumatica.
Criteri di Jones
Manifestazioni cliniche maggiori
Cardite
Poliartrite
Corea
Eritema marginato
Noduli sottocutanei
Manifestazioni cliniche minori
Algia
Febbre
Aumento della VES 0 PCR
Allungamento intervallo della P-R all’elettrocardiogramma
Secondo lo schema di Jones, la presenza di 2 manifestazioni maggiori oppure di una manifestazione maggiore e 2 minori in un paziente con evidenza di infezione streptococcica recente (positività del tampone faringeo) indica un’alta probabilità di malattia reumatica acuta. Il periodo di latenza tra una faringite streptococcica e l’inizio dei sintomi varia da 1 a 5 settimane. Nel 75% dei casi, la febbre e la poliartrite, rappresentano i segni clinici iniziali dell’attacco di malattia reumatica. L’artrite interessa prevalentemente le grandi articolazioni degli arti (ginocchia, gomiti, polsi). A differenza dell’artrite reumatoide sono risparmiate le piccole articolazioni delle mani e dei piedi. Al quadro clinico della poliartrite si sovrappone spesso quella della cardite e in generale la gravità dei sintomi articolari è inversamente proporzionale all’ interessamento cardiaco: nei pazienti con forme gravi di artrite, le manifestazioni cliniche della cardite tendono ad essere attenuate e viceversa. La cardite, presente nel 50% dei pazienti con malattia reumatica acuta, è associata quasi sempre a un soffio cardiaco secondario alla valvulite. Il reperto ascultatorio più frequente è un soffio olosistolico apicale, ad alta frequenza, irradiato all’ascella, indicativo di un’ insufficienza della valvola mitralica. Quest’ultima rappresenta l’elemento clinico più caratteristico della cardite reumatica.
Esami di laboratorio
La diagnosi di malattia reumatica è spesso non facile, non solo per la variabilità del quadro clinico, ma anche per la mancanza di un test diagnostico sicuro e definitivo. Gli indici di flogosi appaiono costantemente alterati nella fase acuta della malattia. La velocità di eritrosedimentazione (VES) e la proteina-C reattiva (PCR) sono marcatori affidabili, ma aspecifici, della risposta autoimmune e dell’infiammazione associate alla cardite e alla poliartrite. In tutti i casi di sospetta malattia reumatica è indispensabile documentare, ai fini diagnostici, una recente infezione streptococcica. I test più utilizzati sono la ricerca di anticorpi diretti contro alcune componenti dello streptococco (streptolisina O, desossoribonucleasi B) e l’esame colturale faringeo (tampone faringeo). La positività del tampone faringeo deve essere interpretata con cautela perché molti individui normali possono ospitare streptococchi del gruppo A nelle vie aeree superiori. D’altra parte la negatività dell’esame colturale non permette di escludere in modo assoluto un episodio antecedente di infezione streptococcica.
Prognosi
La malattia si risolve spontaneamente entro tre mesi dall’esordio acuto. Sebbene siano stati descritti casi isolati di edema polmonare acuto fulminante, la mortalità della fase acuta è bassa e la prognosi dipende fondamentalmente dalla gravità delle lesioni valvolari che fanno seguito al primo episodio della malattia reumatica e/o alle recidive. La malattia reumatica tende a recidivare. I pazienti che hanno sofferto di un precedente attacco di malattie reumatiche che sviluppano successivamente nuovi episodi di faringite streptococcica sono ad alto rischio di una recidiva della malattia. L’infezione streptococcica ricorrente, specie se sostenuta da ceppi virulenti, riattiva la risposta autoimmune dell’organismo, favorendo così l’instaurarsi o il peggioramento del danno anatomico valvolare.
Non esiste un trattamento specifico della malattia reumatica. Gli agenti antinfiammatori sopprimono rapidamente il dolore articolare e altri segni e sintomi della flogosi acuta, ma non curano la malattia né prevengono la sua successiva evoluzione. Anche laterapia antibiotica con penicillina, obbligatoria nella fase acuta per sradicare l’ infezione streptococcica, non modifica il decorso dell’attacco acuto della malattia reumatica né impedisce lo svilupparsi della cardite. L’aspirina ad alte dosi è indicata nella poliartrite acuta, mentre l’impiego dei corticosteroidi è riservata ai casi con cardite grave complicata da insufficienza cardiaca.
Prevenzione
La prevenzione primaria della malattia reumatica acuta si identifica nella diagnosi precoce, nel trattamento antibiotico della faringo-tonsillite streptococcica. Il trattamento antibiotico se tempestivo e mirato (penicillina) elimina quasi completamente il rischio di malattia reumatica. La prevenzione secondaria è rivolta agli individui che hanno già avuto un attacco documentato di malattia reumatica acuta o che soffrono di recidive dopo un’infezione streptococcica. Il caposaldo è rappresentato dalla profilassi antibiotica continua delle recidive di infezione streptococcica, potenzialmente capace di innescare nuovi attacchi di malattia reumatica. La profilassi antimicrobica continua è necessaria perché il trattamento antibiotico di una nuova infezione streptococcica, anche se ottimale, non protegge il paziente con precedenti anamnestici di malattia reumatica dal rischio di una recidiva reumatica. Lo schema più efficace è costituito dalla benzilpenicillina somministrata in dose singola per via intramuscolare ogni 4 settimane. La durata della profilassi antibiotica deve essere adattata al singolo paziente a seconda del rischio di recidiva. Il rischio di ricorrenze reumatiche diminuisce con l’aumentare dell’età e con l’aumentare del tempo trascorso dall’ultimo attacco. I pazienti che non sviluppano la cardite durante il primo attacco sono meno esposti al rischio di recidive reumaiche, e quando queste si verificano hanno minori probabilità di manifestare una cardite. I pazienti che hanno sviluppato una cardite nel corso dell’ attacco acuto sono invece ad alto rischio di recidiva di cardite, con possibilità di ulteriore danno valvolare in occasione di ogni ricorrenza.
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