Impact of quantitative radiological features of interstitial lung disease on immunomodulatory treatment response in three autoimmune interstitial lung disease cohorts

Affiliations

¹ Division of Pulmonary, Critical Care and Sleep Medicine, The University of Kansas School of Medicine, Kansas City, Kansas, USA smatson@kumc.edu.
² Department of Radiological Sciences, David Geffen School of Medicine at UCLA, Los Angeles, California, USA.
³ Department of Radiology, National Jewish Health, Denver, Colorado, USA.
⁴ Department of Medicine and Health Sciences Research, University of California, Los Angeles, Los Angeles, California, USA.
⁵ Department of Radiologic Sciences, University of California, Los Angeles, Los Angeles, California, USA.
⁶ UCLA Health, Los Angeles, California, USA.
⁷ Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California, Los Angeles, Los Angeles, California, USA.
⁸ Department of Medicine, Division of Internal Medicine and Health Sciences Research, Los Angeles, California, USA.
⁹ Medicine, Division of Rheumatology & Immunology, University of Nebraska Medical Center, Omaha, NE USA and Veterans Affairs Nebraska-Western Iowa Health Care System, Omaha, NE, USA, Omaha, Nebraska, USA.
¹⁰ Department of Medicine, Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Anschutz Campus, Aurora, Colorado, USA.

PMID: 40210443
PMCID: PMC12263334 (available on 2026-07-15)
DOI: 10.1136/thorax-2024-222367

Impact of quantitative radiological features of interstitial lung disease on immunomodulatory treatment response in three autoimmune interstitial lung disease cohorts

Scott M Matson et al. Thorax. 2025.

. 2025 Jul 15;80(8):540-546.

doi: 10.1136/thorax-2024-222367.

Authors

Affiliations

¹ Division of Pulmonary, Critical Care and Sleep Medicine, The University of Kansas School of Medicine, Kansas City, Kansas, USA smatson@kumc.edu.
² Department of Radiological Sciences, David Geffen School of Medicine at UCLA, Los Angeles, California, USA.
³ Department of Radiology, National Jewish Health, Denver, Colorado, USA.
⁴ Department of Medicine and Health Sciences Research, University of California, Los Angeles, Los Angeles, California, USA.
⁵ Department of Radiologic Sciences, University of California, Los Angeles, Los Angeles, California, USA.
⁶ UCLA Health, Los Angeles, California, USA.
⁷ Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California, Los Angeles, Los Angeles, California, USA.
⁸ Department of Medicine, Division of Internal Medicine and Health Sciences Research, Los Angeles, California, USA.
⁹ Medicine, Division of Rheumatology & Immunology, University of Nebraska Medical Center, Omaha, NE USA and Veterans Affairs Nebraska-Western Iowa Health Care System, Omaha, NE, USA, Omaha, Nebraska, USA.
¹⁰ Department of Medicine, Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Anschutz Campus, Aurora, Colorado, USA.

PMID: 40210443
PMCID: PMC12263334 (available on 2026-07-15)
DOI: 10.1136/thorax-2024-222367

Abstract

Background: The defining radiological features of autoimmune interstitial lung disease (ILD) are ground glass opacification (GGO) and fibrosis. The associations between these features and physiological response to immunomodulation remain unclear.

Methods: This study leveraged three autoimmune ILD cohorts: two with systemic sclerosis (SSc) and one with rheumatoid arthritis (RA) which were selected for inherent differences in fibrotic extents/patterns. Linear regression models examined associations between baseline quantitative GGO, fibrosis, their ratio and forced vital capacity (FVC)%-predicted changes after 12 months of immunomodulatory therapy.

Results: Patients with SSc-ILD (N=262) exhibited a higher GGO-to-fibrosis ratio compared with patients with RA-ILD (N=130) (mean ratio 3.0 vs 0.25). Increased GGO-to-fibrosis was not associated with improved FVC%-predicted in any cohort. Conversely, in patients with SSc-ILD treated with cyclophosphamide (CYC), increased fibrosis (estimate 0.17 (95% CI 0.003, 0.33); p=0.04) and increased GGO (estimate 0.15 (95% CI 0.004, 0.30); p=0.044) were both significantly associated with FVC% improvement. Given the negative direction of the estimate for GGO-to-fibrosis ratio (estimate -0.33 (95% CI -0.61, -0.06); p=0.016), CYC was associated with greater FVC% improvement in patients with a higher degree of fibrosis relative to GGO. No significant correlation was seen in patients with SSc-ILD treated with mycophenolate (N=56) or in patients with RA-ILD treated with immunomodulation (N=130).

Discussion: Increased quantitative GGO relative to fibrosis was not significantly associated with improved response to immunomodulation in patients with RA-ILD and SSc-ILD. However, increased quantitative fibrosis and GGO extent were associated with improved response to CYC in SSc-ILD. More research is needed to understand how to use radiological features to guide treatment selection in ILD.

Keywords: Interstitial Fibrosis; Rheumatoid lung disease.

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Conflict of interest statement

Competing interests: None declared.

References

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Impact of quantitative radiological features of interstitial lung disease on immunomodulatory treatment response in three autoimmune interstitial lung disease cohorts

Affiliations

Impact of quantitative radiological features of interstitial lung disease on immunomodulatory treatment response in three autoimmune interstitial lung disease cohorts

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Abstract

Conflict of interest statement

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