Under detection of interstitial lung disease in juvenile systemic sclerosis (jSSc).
Ivan FoeldvariJens KlotscheBernd HinrichsNicola HelmusOzgur KasapcopurAmra AdrovicFlavio SztajnbokMaria Teresa TerreriJordi AntonVanessa SmithMaria KatsicasMikhail KostikNatalia Vasquez-CanizaresTadej AvcinBrian FeldmanMahesh JanarthananMaria Jose SantosSujata SawhneyDieneke Schonenberg-MeinemaWalter-Alberto Sifuentes-GiraldoEkaterina AlexeevaSimone AppenzellerCristina BattagliottiLillemor BerntsonBlanca BicaPatrícia Costa ReisDespina EleftheriouTilmann KallinichThomas LehmanEdoardo MarraniKirsten MindenSusan NielsenFarzana NuruzzamanAnjali PatwardhanRaju KhubchandaniValda StanevichaYosef UzielKathryn S TorokPublished in: Arthritis care & research (2020)
The performance of PFTs in jSSc to detect underlying ILD was quite limited. Specifically, the FVC, which is one of the main clinical parameters in adult SSc to detect and monitor ILD, would miss approximately 60% of children that had ILD changes on their accompanying HRCT. The DLCO was more sensitive in detecting potential abnormalities in HRCT, but with less specificity than the FVC. These results support the use of HRCT in tandem with PFTs for the screening of ILD in jSSc.