Table 1.
Baseline assessments for all potential participants
Domain | Assessments |
History | Symptoms, date of onset, family history, comorbid diseases, medications, smoking history, exposures (environmental/occupational). |
Examination | Vital 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). |
Serology | Full 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 function | Spirometry, lung volumes, DLCO. |
6MWT | Distance, start SpO2, nadir SpO2. |
HRCT scan | Images 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;