Malattia di Behçet: cause, sintomi e cure
Table of Contents
Che cos’è la malattia di Behçet?
La malattia di Behçet è caratterizzata da fenomeni infiammatori che possono essere localizzati allemucose orali e genitali(afte),all’occhio(irite aftosa, uveite, ipopion),alla cute(eritema polimorfo) e da manifestazioniarticolari di tipo reumatico alle anche e alle ginocchia; possono compariredisturbi neurologici(gravi paralisi dei nervi cranici, convulsioni e obnubilamenti).
Quanto è diffusa la malattia di Behçet?
La malattia di Behçet colpisce soprattutto soggetti giovani nella seconda e la terza decade di vita. L’esordio di malattia prima dei 16 anni e dopo i 45 è un evento insolito. La malattiadi Behçet è più diffusa lungo la via della seta, dal Mediterraneo alla Cina; è rara negli Stati Uniti. Il sesso maschile tende ad essere più colpito di quello femminile, specie nei paesi a più alta prevalenza ove il rapporto maschi femmine è di circa 3:1, mentre nei paesi occidentali il rapporto maschi femmine risulta di 1:1. La malattia diBehçettende ad avere un decorso più aggressivo nei maschi, specie nei più giovani. Generalmente si presenta in forma sporadica anche se sono descritti casi familiari.
Eziopatogenesi
L’eziologia della malattia diBehçet è tuttora sconosciuta tuttavia negli ultimi decenni grazie ai progressi delle metodiche molecolari e delle ricerche di base le conoscenze sono migliorate. Numerosi agenti infettivi, quale ad esempioBorrelia burgdorferi,Helicobacter pylori,Citomegalovirus,Epstein-barr-virus,Parvovirus B19,Varicella zoster virus,Virus epaticisono stati a lungo proposti come possibili fattori scatenanti sebbene nessuno di essi sia stato con certezza associato allo sviluppo di malattia.
Quali sono i sintomi della mallattia di Behçet?
La malattia Behçet esordisce all’età di 20- 40 anni di solito, però nessuna età ne è esente. La malattia è caratterizzata da periodi di remissione seguiti da riacutizzazione.
I sintomi possono durare alcuni giorni o settimane, possono persistere per mesi o anni.
- Le afte oralisono la manifestazione cardine di questa malattia e sono presenti in quasi tutti i pazienti. Solitamente sono multiple, molto dolenti e ricorrenti. Sono rotonde o ovali, ben definite, con un aureola rossa che circonda una base necrotica gialla. Le ulcere possono interessare tutte le parti della bocca, lingua e gola. Le afte orali vengono distinte inafte maggiori(di diametro superiore a 10 mm),minori(di diametro inferiore a 10 mm), erpetiforme.
- Le afte genitalisono più specifiche delle afte orali per la malattia diBehçet. Sono simili come apparenza alle ulcere orali e compaiono nelle mucose genitali. Nei maschi si localizzano in particolare sullo scroto, nelle femmine sulle grandi labbra, vagina e /o parte cervicale dell’utero. Sono solitamente molto dolenti e guariscono spesso con una cicatrice.
- L’uveiteanteriore e posterioreè l’interessamento d’organo più frequente. L’uveite posteriore con interessamento retinico (vasculite) è la lesione oculare più grave, se non trattata adeguatamente, determina la formazione di cicatrici retiniche con calo e talora perdita del visus.
- Le lesioni cutanee del Behçetsono frequenti e si manifestano comeeritema nodoso, in genere agli arti inferiori, papulopustole o lesioni acneiformi.
- Le manifestazioni neurologichecomprendono segni piramidali, bilaterali in più di due terzi dei casi,emiparesi, alterazioni comportamentali, disturbi sfinerici, cefalea, segni di interessamento del tronco cerebrale, sindrome piramidocerebellare.
- L’artriteinteressa circa il 50% dei pazienti. L’artrite associata alla malattia di Behçet è tipicamente non erosiva, non deformante, in ordine di frequenza decrescente, le ginocchia, le caviglie, i gomiti e i polsi.
- Il coinvolgimento venoso è la manifestazione più comune e si presenta cometromboflebiti superficialie/o profonde in particolare degli arti inferiori. Altre manifestazioni vascolari sono latrombosi della vena cava, gli aneurismi dell’arteria polmonare, la sindrome di Budd-Chiari e la trombosi dei seni durali.
- La manifestazione cardiacapiù tipica è la trombosi intracardiaca, che di solito coinvolge il lato destro del cuore e spesso coesiste con una tromboembolia arteriosa polmonare, trombosi venosa e fibrosi endomiocardica.
- Il tratto intestinalepuò essere anche interessato; l’intestino è la parte del tratto gastrointestinale più frequentemente interessato. Le manifestazioni più frequenti sono addominalgia e melena mentre ladiarreaè più rara.
Come si fa la diagnosi della malattia di Behçet?
Non esiste un test di laboratorio specifico per lamalattia diBehçet, la cui diagnosi è clinica. Il test genetico HLA-B51 è positivo nel 60% di pazienti con malattia di Berchet italiani, ma anche positivo nel 18% della popolazione generale, per cui è di scarsa specificità. I reattanti della fase acuta (Ves e proteina C-reattiva) possono essere elevati, in particolare nei pazienti con vasculo-Behçet. Un test abbastanza specifico per la malattia è iltest cutaneo di patergia. Il test cutaneo di patergia è una puntura intradermica fatta dal medico con un ago sterile di calibro 20-22 ad una profondità di 5 mm. Nei pazienti con malattia diBehçet tale test è positivo in circa la metà dei casi; il testo è considerato positivo quando si hala formazione di una papula eritematosa di diametro superiore a 2 mm a 48 ore nel punto della puntura dell’ ago.
Come si cura la malattia di Behçet?
Poiché è la causa della malattia è ignota, il trattamento è diretto verso le manifestazioni che caratterizano il decorso della malattia in generale, i cortisonici hanno efficacia virtualmente su tutte le manifestazioni della malattia di Behçet; il dosaggio e la durata della terapia cortisonica dipendono dalla gravità delle manifestazioni cliniche. Il trattamento delle lesioni muco-cutanee si basa sull’uso di glucocorticoidi topici (per le afte) e sullacolchicina. Il trattamento delle manifestazioni oculari dipende dal tipo di interessamento. L’uveite anteriore può essere trattata solo con farmaci topici, viceversa, l’ interessamento del polo posteriore oculare richiede sempre un trattamento sistemico con glucocorticoidi. Il trattamento dell’artrite si basa sull’uso della colchicina.
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(Bibl. UNIREUMA, Reumatologia per stuenti e medici di medicina generale, IV ediz. a cura di C. Montecucco)
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