Table 1

Studies including patients that would meet the INBUILD criteria for progression

ILD subtypeStudy sizeProportion of patients with a progressive phenotype
SSc-ILDn=695~33% of patients with DLco pred <50% within 3 years of the onset of Raynaud’s phenomenon121
Limited cutaneous SScn=326Worsening of ILD (>10% decline in FVC from baseline to second visit) observed in 19.9% of patients at 24 months follow-up122
RA-ILDn=167*14% of patients with FVC <50% pred at diagnosis, increasing to 22% after 5 years; 29% of patients with DLco <40% pred at diagnosis, increasing to 40% after 5 years8
Inflammatory myopathy-associated ILDn=107Worsening of pulmonary symptoms, deterioration on HRCT, and decline in lung function (≥10% in FVC or ≥15% in DLco) observed in 15.9% of patients (despite therapy), after a median 34 months of follow-up (range 4–372 months)89
Sjögren’s syndrome-associated ILDn=18†5 patients (28%) had a decline in FVC pred of ≥10% or a decline in DLco pred of ≥15%, despite immunosuppression (median follow-up: 38 months)123
  • *167 patients encountered in clinical practice and referred for multi-specialty evaluation in a tertiary care centre (potential centre bias: severe cases are more often encountered at a specialised centre).

  • †18 patients selected over a 13-year period.

  • DLco, diffusing capacity of the lung for carbon monoxide; FVC, forced vital capacity; HRCT, high-resolution CT; ILD, interstitial lung disease; pred, predicted; RA, rheumatoid arthritis; SSc, systemic sclerosis.