Abstract
<b>Background:</b> Using prospective registries in Australia and Canada, this study evaluated trajectories and prognostic value of 6-minute walk test (6MWT) parameters in idiopathic pulmonary fibrosis (IPF) and non-IPF fibrotic ILD stratified by presence of criteria for progressive pulmonary fibrosis (PPF). <b>Methods:</b> Cumulative incidence of exertional and resting hypoxaemia and changes in walk distance (6MWD) and composite indices of 6MWD and oxygenation status were determined, with prognostic value evaluated when meeting criteria for PPF. New-onset hypoxaemia was evaluated as another potential criterion for PPF. <b>Results:</b> IPF/PPF and non-IPF/PPF had similar cumulative incidence of hypoxaemia and annualised decline in 6MWD and composite indices, which varied across each PPF criterion. Both IPF/non-PPF and non-IPF/non-PPF had lower cumulative incidence of hypoxaemia than IPF/PPF, with annualised increase in 6MWD and composite indices in non-IPF/non-PPF (Figure). Exertional or resting hypoxaemia at time of meeting criteria for PPF was independently associated with transplant-free survival. Adding new-onset exertional or resting hypoxaemia as an additional potential PPF criterion reduced the median time to develop PPF by 117 days (p<0.001). <b>Conclusion:</b> Patients with IPF/PPF and non-IPF/PPF have comparable deterioration in functional capacity. Oxygenation status provides prognostic information and may assist in defining disease progression in fibrotic ILD.
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