Fibrosi polmonare, cause, sintomi, diagnosi e cure

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La fibrosi polmonare è una grave condizione medica caratterizzata dalla cicatrizzazione del tessuto polmonare. Questo si verifica quando gli alveoli, o sacche d’aria, si infiammano e sviluppano cicatrici sul tessuto polmonare nel tentativo di riparare se stessi. Non esiste attualmente una cura o un modo per invertire le cicatrici sui polmoni una volta che si sono sviluppate, ma solo ( ed esclusivamente di recente) alcuni farmaci in grado di rallentare la progressione della malattia laddove la diagnosi avvenga per tempo. Nei casi più gravi e possibili c’è il trapianto di polmoni. Scopriamo insieme quali sono i sintomi, le cause e come si fa la diagnosi.

fibrosi polmonare

 

Sintomi di fibrosi polmonare

Quando il tessuto polmonare, fondamentale per la respirazione, in qualche modo viene lesionato a causa di un’infezione, infiammazione o comunque una qualunque aggressione, si ripara da solo, ma sviluppandone uno fibroso, più spesso e rigido che rende più difficile agli alveoli di incanalare ossigeno e rilasciare anidride carbonica. Quando la quantità di questi tessuti comincia ad essere tanta si sviluppa la sensazione di mancanza di respiro, tosse secca cronica, senso di fastidio al petto, affaticamento. Una infiammazione ai polmoni (polmonite) è spesso il primo segnale della malattia.
La cicatrizzazione dei tessuti polmonari sviluppa una riduzione permanente e progressiva della capacità di fornire ossigeno al corpo. Più avanza la cicatrizzazione e meno il paziente sarà in grado di respirare correttamente.

Cause

Ci sono molte possibili cause di fibrosi polmonare. L’inalazione di amianto è tra le prime, ma anche di terra o polvere di pietre e metalli. Anche la sarcoidosi (malattia infiammatoria) è tra le possibili cause di tale patologia grave, come pure l’assunzione di alcuni farmaci (chemioterapici per lo più), ma nella maggioranza dei casi purtroppo la causa è sconosciuta: si parla quindi di fibrosi polmonare idiopatica. Di recente si sta valutando tale patologia anche come una malattia autoimmune.

 

Fattori di rischio

Uno dei fattori di rischio principali per la fibrosi polmonare è il fumo, che aumenta notevolmente le probabilità di una persona di incappare nella malattia oltre che di avere una problematica respiratoria di base al momento in cui questa si dovesse sviluppare. Allo stesso modo lavorare in settori in cui l’inquinamento atmosferico è comune, come l’edilizia, l’agricoltura o l’industria mineraria, può mettere una persona a rischio di contrarre tale malattia. Di sicuro sembra esserci una componente genetica (quindi la familiarità). In più, colpisce maggiormente le persone, uomini, al di sopra dei 60 anni.

Diagnosi

Per diagnosticare correttamente una fibrosi polmonare è necessario analizzare bene la storia clinica e professionale del paziente, oltre che personale (hobby o consumo di sostanze che possono esporre al rischio). Esami del sangue, radiografia del torace, una broncoscopia per vedere all’interno dei polmoni e una biopsia polmonare andranno a complemento del quadro complessivo, anche se la biopsia sembra essere considerato il test più efficace per la fibrosi polmonare, e coinvolge un campione di tessuto polmonare che viene rimosso per ulteriori esami e prove.

Cura per fibrosi polmonare

Fino a poco tempo fa non vi era alcuna cura conosciuta per la fibrosi polmonare, ma oggi delle speranze ci sono, benché dipendano molto dal grado di avanzamento della malattia al momento della diagnosi. Di recente infatti è stato commercializzato (anche in Italia) il pirfenidone di InterMune, indicato nei casi medio-lievi della malattia, si atende l’autorizzazione per l’immissione in commercio del nintedanib di Boheringer Ingelheim ed è allo studio una nuova molecola il Simtuzumab di Gilead Science, per la quale si è a buon punto nella sperimentazione.  Tra le cure basilari e di supporto c’è l’ossigenoterapia supplementare e laddove possibile il trapianto di polmone.

Prognosi

Purtroppo attualmente si rivela fatale in media entro 3 – 5 anni dalla diagnosi.

 

Leggi anche:

Inquinamento e polmoniti, fenomeni strettamente legati

Tosse è un campanello d’allarme per altre patologie

 

Foto: Thinkstock

Fonte: Wisegeek.org

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