The prognostic importance of worsening dyspnoea in systemic sclerosis related interstitial lung disease

To determine the prognostic importance of worsening dyspnoea in systemic sclerosis (SSc) related interstitial lung disease (ILD). SSc patients were recruited from the Australian Scleroderma Cohort Study. ILD was defined as present if characteristic fibrotic changes on high-resolution computer tomography (HRCT) scan lung were seen, performed based on clinical suspicion. Determinants of survival in SSc-ILD were analysed, specifically the prognostic impact of ILD progression, defined according to the ATS, INBUILD and spirometric definitions of progressive pulmonary fibrosis, and the presence of worsening dyspnoea using accelerated failure time survival analysis. In our cohort of 2198 SSc patients, 17.7% (n = 389) developed incident ILD over a median (IQR) follow-up period of 5.8 (2.8-9.9) years. A third of our incident ILD cohort (38.3%, n = 149) experienced progressive ILD. In those who experienced progressive ILD, mortality was significantly higher in those who reported recent worsening dyspnoea in the prior month irrespective of a decline in spirometric parameters. 38.8% of those with incident ILD experienced ILD progression over follow-up. Patient-reported worsening dyspnoea in the month prior, irrespective of changes in spirometry, was strongly associated with mortality, with a magnitude equivalent to that of the presence of progressive ILD as defined by the ATS, INBUILD and spirometric definitions of progressive ILD. This study has highlighted the prognostic importance of patient-reported symptoms in the evaluation of SSc-ILD.