UCLA

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.