Application of a Growth-Rate Model to Enhance Subgroup Identification in Heterogeneous Clinical Courses of the Idiopathic Inflammatory Myopathy-Associated Interstitial Lung Disease and Its Prognostic Implication

Int J Rheum Dis. 2025 Jun;28(6):e70320. doi: 10.1111/1756-185X.70320.

Abstract

Background: Analyzing longitudinal real-world data with nonuniform study-time intervals is challenging. This study aimed to identify subgroups in heterogeneous clinical courses of idiopathic inflammatory myopathies-associated interstitial lung disease (IIM-ILD) using a growth rate model and to assess their prognostic significance.

Methods: In this retrospective cohort study, 243 chest high-resolution computed tomography (HRCT) scans from 80 patients with IIM-ILD were analyzed using a computer-aided quantification system to estimate quantitative lung fibrosis (QLF) scores. Longitudinal patterns were identified through a growth-rate model, and a landmark survival analysis was performed using the last HRCT date as an anchor.

Results: Using the growth-rate model, we identified five different patterns in the serial QLF scores: progressive (n = 19), improving (n = 20), convex (n = 10), others (mostly concave, n = 22), and stable (n = 9). When the group with the progressive pattern was divided into the rapid progression and slow progression by the median progression rate (g = 1.029%/month), the rapid progressive group was significantly associated with mortality (Hazard ratio 15.926, 95% confidence interval 1.079-548.324, p = 0.043), compared to the reference group. However, the intensity of immunosuppression or QLF scores at landmark time were not associated with mortality.

Conclusion: Combined volumetric measurement of lung fibrosis and application of growth-rate model had the potential to identify subgroups in analyzing complex, dynamic real-world data of IIMs-ILD. This approach may help extrapolate the future course and provide useful information about prognosis in patients with ILD.

Keywords: automated quantification system; growth‐rate model; idiopathic inflammatory myopathy‐related interstitial lung disease; longitudinal change; mortality.

MeSH terms

  • Adult
  • Aged
  • Disease Progression
  • Female
  • Humans
  • Lung Diseases, Interstitial* / diagnostic imaging
  • Lung Diseases, Interstitial* / etiology
  • Lung Diseases, Interstitial* / mortality
  • Lung* / diagnostic imaging
  • Male
  • Middle Aged
  • Myositis* / complications
  • Myositis* / diagnosis
  • Myositis* / mortality
  • Predictive Value of Tests
  • Prognosis
  • Pulmonary Fibrosis* / diagnostic imaging
  • Pulmonary Fibrosis* / etiology
  • Pulmonary Fibrosis* / mortality
  • Radiographic Image Interpretation, Computer-Assisted
  • Retrospective Studies
  • Risk Factors
  • Time Factors
  • Tomography, X-Ray Computed