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Idiopatisk lungfibros

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Sakkunnig:
Definition:
Idiopatisk lungfibros (IPF) är ett tillstånd med gradvis ökande fibros i lungorna. Etiologin är okänd.
Förekomst:
Relativt sällsynt. Prevalensen (USA) är beräknad till 14–43 per 100 000 per år och ökar med åldern. Diagnosen ställs oftast i 60-årsåldern.
Symtom:
Huvudsymtomen är kroniska besvär med gradvist ökande andnöd och hosta. 
Kliniska fynd:
Kliniska fynd kan vara vilodyspné, takypné, trumpinnefingrar och cyanos. Typiska torra basala inspiratoriska rassel vid lungauslultation.
Diagnostik:
Viktiga diagnostiska undersökningar är DT av torax, spirometri, bronkoskopi med kryobiopsi och eventuellt torakoskopisk lungbiopsi.
Behandling:
Nya läkemedel (pirfenidon och nintedanib) har visat sig kunna bromsa sjukdomsutvecklingen något. Lungtransplantation kan vara aktuellt.
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  • Gerdt Riise, docent och överläkare, Avdelningen för lungmedicin, Sahlgrenska universitetssjukhuset, Göteborg
  • Anna Nager, med dr och specialist i allmänmedicin, Karolinska institutet