Table 1

Baseline assessments for all potential participants

DomainAssessments
HistorySymptoms, date of onset, family history, comorbid diseases, medications, smoking history, exposures (environmental/occupational).
ExaminationVital signs, lung auscultation, signs of pulmonary hypertension or right ventricular failure, clubbing, Raynaud’s phenomenon, inflammatory arthritis, sclerodactyly, skin changes, muscle weakness, other connective tissue disease features (eg, Gottron’s papules).
SerologyFull blood count, biochemistry, coagulation studies, ANA, ENA, RF, anti-CCP, ds-DNA, ANCA (MPO/PR3), extended panel of myositis antibodies, farmers/pigeon/budgerigar immunoglobulins, ACE, creatine kinase, NT-pro-BNP.
Lung functionSpirometry, lung volumes, DLCO.
6MWTDistance, start SpO2, nadir SpO2.
HRCT scanImages must be obtained volumetrically on a multidetector CT with <1.25 mm slice collimation of axial images using a high-resolution reconstruction algorithm, or non-contiguously with 1–1.5 mm slices obtained at 10 mm intervals. Prone, supine, inspiratory and expiratory views will be acquired.
  • anti-CCP, anticyclic citrullinated peptide; ANA, antinuclear antibodies; ANCA, antineutrophil cytoplasmic antibody; ds-DNA, double-stranded DNA.DLCO, diffusing lung capacity for carbon monoxide; ENA, extractable nuclear antigen; HRCT, high-resolution CT; MPO, myeloperoxidase; 6MWT, 6 min walk test; NT-pro-BNP, N-terminal pro-brain natriuretic peptide; PR3, proteinase 3; RF, rheumatoid factor; SpO2, oxygen saturation on pulse oximetry;