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Management of ILD with and without progressive pulmonary fibrosis in ARDs
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PROGRESSIVE ILD IN IDIOPATHIC INFLAMMATORY MYOPATHIES: A MULTICENTRE STUDY
Abstract
Background and Aims
Interstitial lung disease (ILD) is a frequent organ involvement in idiopathic inflammatory myopathies (IIMs). Some patients with ILD may develop a progressive fibrosing phenotype despite treatment. The occurence of progressive IIMs-ILD and the associated phenotype remain to be determined. We aimed to investigate the prevalence, clinical/serological, and functional/radiological characteristics of patients with progressive IIMs-ILD.
Methods
We collected clinical, serological and functional/radiological data of 125 IIMs-ILD patients at diagnosis and follow-up. The prevalent radiological pattern at diagnostic high-resolution CT scans (ground-glass opacities, GGO; fibrotic changes, FC; consolidation, C) was evaluated by expert radiologists. Progression was defined in presence of at least two of these characteristics at one-year follow-up: decline in forced vital capacity (FVC)% pred.≥ 5%, worsening of HRCT or worsening of clinical symptoms.
Results
In 78/125 (62%, 55 females) IIMs-ILD patients, functional and radiological data were available after one-year follow-up. The predominant HRCT pattern was GGO (n=39; 50%), followed by FC (n=22, 28%) and C (n=17, 22%). At one-year follow-up, 14 (18%) patients had developed progressive ILD, whereas 65 (82%) were stable. Demographics, functional data and radiological pattern at ILD diagnosis did not differ between progressors vs. stable IIMs-ILD patients, as well as treatment with glucocorticoids and immunosuppressants. Anti-MDA5 autoantibodies [OR 6.10, 95%CI(1.30–28.4)], heliotropic rash [8.00(1.55–41.23)], and xerostomia [8.19 (1.84 – 36.36)] were progression-associated factors at univariate but not at multivariate analysis.
Conclusions
Progressive ILD occurred in one fifth of our IIMs-ILD cohort. Our results suggest that all patients with IIMs-ILD should be monitored to detect progression, independently of IIMs- and ILD-specific features.