RT Journal Article SR Electronic T1 Pulmonary function tests as outcomes for systemic sclerosis interstitial lung disease JF European Respiratory Review JO EUROPEAN RESPIRATORY REVIEW FD European Respiratory Society SP 170102 DO 10.1183/16000617.0102-2017 VO 27 IS 148 A1 Melissa Caron A1 Sabrina Hoa A1 Marie Hudson A1 Kevin Schwartzman A1 Russell Steele YR 2018 UL http://err.ersjournals.com/content/27/148/170102.abstract AB Interstitial lung disease (ILD) is the leading cause of morbidity and mortality in systemic sclerosis (SSc). We performed a systematic review to characterise the use and validation of pulmonary function tests (PFTs) as surrogate markers for systemic sclerosis-associated interstitial lung disease (SSc-ILD) progression.Five electronic databases were searched to identify all relevant studies. Included studies either used at least one PFT measure as a longitudinal outcome for SSc-ILD progression (i.e. outcome studies) and/or reported at least one classical measure of validity for the PFTs in SSc-ILD (i.e. validation studies).This systematic review included 169 outcome studies and 50 validation studies. Diffusing capacity of the lung for carbon monoxide (DLCO) was cumulatively the most commonly used outcome until 2010 when it was surpassed by forced vital capacity (FVC). FVC (% predicted) was the primary endpoint in 70.4% of studies, compared to 11.3% for % predicted DLCO. Only five studies specifically aimed to validate the PFTs: two concluded that DLCO was the best measure of SSc-ILD extent, while the others did not favour any PFT. These studies also showed respectable validity measures for total lung capacity (TLC).Despite the current preference for FVC, available evidence suggests that DLCO and TLC should not yet be discounted as potential surrogate markers for SSc-ILD progression.Despite the popularity of FVC, available evidence does not favour it as the best PFT surrogate marker for SSc-ILD http://ow.ly/l8vZ30j6kmj