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

Author:

Morrisroe Kathleen12,Hansen Dylan2,Stevens Wendy2,Ross Laura12ORCID,Sahhar Joanne3,Ngian Gene-Siew3,Hill Catherine L456,Host Lauren7,Walker Jennifer8,Proudman Susanna46,Nikpour Mandana12

Affiliation:

1. Department of Medicine, The University of Melbourne at St Vincent’s Hospital , Melbourne, VIC, Australia

2. Department of Rheumatology, St Vincent’s Hospital , Melbourne, VIC, Australia

3. Department of Medicine, Monash University, Clayton and Monash Health , Clayton, VIC, Australia

4. Rheumatology Unit, Royal Adelaide Hospital , Adelaide, SA, Australia

5. Rheumatology Unit, The Queen Elizabeth Hospital , Woodville, SA, Australia

6. Discipline of Medicine, University of Adelaide , Adelaide, SA, Australia

7. Department of Rheumatology, Fiona Stanley Hospital , Perth, WA, Australia

8. Rheumatology Unit, Flinders Medical Centre , Adelaide, SA, Australia

Abstract

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 1.00–1.03, P = 0.011), dcSSc (OR 1.54, 95% CI 1.06–2.25, P = 0.024) and SSc-specific antibodies (anticentomere antibody OR 0.51, 95%CI 0.29–0.91, P = 0.021 and anti-Scl-70 antibody OR 1.46, 95%CI 1.01–2.09, P = 0.043). Raised CRP was numerically associated with PPF but did not reach statistical significance (OR 1.29, 95%CI 0.99–1.68, P = 0.064) nor did GORD or dysphagia (OR 1.18, 95%CI 0.57–2.42, P = 0.658 and OR 1.17, 95%CI 0.57–2.40, P = 0.664, respectively). The presence of PPF significantly impacted survival in SSc-ILD (hazard ratio 2.66, 95%CI 1.59–4.41, P < 0.001). Conclusions PPF occurred in a third of our incident SSc-ILD cohort; however, its occurrence was significantly associated with mortality indicating an at-risk group who may be suitable for earlier introduction of immunosuppressive and/or antifibrotic therapy.

Funder

Scleroderma Australia

Arthritis Australia

Actelion Australia

Bayer

CSL Biotherapies

GlaxoSmithKline Australia

Pfizer

National Health and Medical Research Council of Australia

Publisher

Oxford University Press (OUP)

Subject

Pharmacology (medical),Rheumatology

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