Journal article

Progressive pulmonary fibrosis and its impact on survival in systemic sclerosis-related interstitial lung disease

K Morrisroe, D Hansen, W Stevens, L Ross, J Sahhar, GS Ngian, CL Hill, L Host, J Walker, S Proudman, M Nikpour

Rheumatology United Kingdom | OXFORD UNIV PRESS | Published : 2024

Abstract

Objective: To describe the frequency of progressive pulmonary fibrosis (PPF) in an incident cohort of systemic sclerosis (SSc)-related interstitial lung disease (ILD) and its impact on survival. Methods: Incident ILD was defined as the new development of characteristic fibrotic changes on chest HRCT scan. PPF was defined as per the 2022 American Thoracic Society. Determinants of PPF were identified using generalised estimating equations. Impact on survival was analysed using accelerated failure time regression modelling. Results: Of our incident SSc-ILD cases, 38.8% (n = 180) experienced PPF within a 12-month period after ILD diagnosis. Determinants of PPF included older age (OR 1.02, 95%CI ..

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Grants

Awarded by National Health and Medical Research Council of Australia Investigator Grant


Funding Acknowledgements

This work was supported by Scleroderma Australia, Arthritis Australia, Actelion Australia, Bayer, CSL Biotherapies, GlaxoSmithKline Australia, and Pfizer. K.M. holds a National Health and Medical Research Council of Australia Investigator Grant (GNT1197169). L.R. holds an Arthritis Australia/Australian Rheumatology Association-Victoria Fellowship. M.N. holds a National Health and Medical Research Council of Australia Investigator Grant(GNT1176538).