Pulmonary function tests as outcomes for systemic sclerosis interstitial lung disease.

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 ( D _LCO ) 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 D _LCO Only five studies specifically aimed to validate the PFTs: two concluded that D _LCO 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 D _LCO and TLC should not yet be discounted as potential surrogate markers for SSc-ILD progression.
Competing Interests: Conflict of interest: M. Caron reports grants from the Fonds de Recherche du Québec (Santé PhD Studentship) and from the Canadian Institutes of Health Research (GSD–146268) during the conduct of the study. S. Hoa reports grants from the Université de Montréal Rheumatology Clinical Fellowship Program (Abbvie educational grant) and from the Arthritis Society's Postdoctoral Fellowship Award, during the conduct of the study.
(Copyright ©ERS 2018.)