The chronic fibrosing idiopathic interstitial pneumonias (IIPs) are a group of heterogeneous pulmonary
parenchymal disorders described by radiologic and histological patterns termed usual interstitial pneumonia (UIP) and
non-specific interstitial pneumonia (NSIP). These include idiopathic pulmonary fibrosis (IPF) and those related to
connective tissue disease (CTD) and are associated with substantial morbidity and mortality. Beyond the importance of
establishing an appropriate diagnosis, designing optimal clinical trials for IIPs has been fraught with difficulties in
consistency of clinical endpoints making power analyses, and the establishment of efficacy and interpretation of results
across trials challenging.
Preliminary recommendations, developed by rigorous consensus methods, proposed a minimum set of outcome measures,
a ‘core set’, to be incorporated into future clinical trials (Saketkoo et al, THORAX. 2014.). This paper sets out to examine
the candidate instruments for each domain (Dyspnea, Cough, Health Related Quality of Life, Imaging, Lung Physiology
and Function, Mortality). Candidate measures that were not selected as well as measures that were not available for
examination at the time of the consensus process will also be discussed.