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Eur Respir Rev. 2018 May 15;27(148). pii: 170102. doi: 10.1183/16000617.0102-2017. Print 2018 Jun 30.

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

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Dept of Epidemiology, Biostatistics and Occupational Health, McGill University, Montreal, QC, Canada.
Respiratory Epidemiology and Clinical Research Unit (RECRU), Montreal Chest Institute, McGill University Health Centre, Montreal, QC, Canada.
Division of Rheumatology, Jewish General Hospital, Montreal, QC, Canada.
Dept of Mathematics and Statistics, McGill University, Montreal, QC, Canada.


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.

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