Quantitative HRCT as a surrogate outcome measure for nintedanib treatment in systemic sclerosis-interstitial lung disease and idiopathic pulmonary fibrosis

Abstract

Objective

We assessed the effect of nintedanib (NIN) in terms of quantitative HRCT changes in both idiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated progressive interstitial lung disease (SSc-ILD), evaluating the relationships between imaging variations and clinical-functional outcomes.

Methods

We prospectively enrolled SSc-ILD and IPF patients treated with NIN and retrospectively selected the same number of subjects from a historical untreated cohort comparable for disease, age, gender and follow-up period. HRCT scans were processed with CALIPER software, obtaining the percentage of normal parenchyma, ILD and vascular-related structures (VRS).

Results

Quantitative HRCT changes of 36 NIN treated patients (12 SSc-ILD and 24 IPF) were compared with 36 untreated subjects with pulmonary fibrosis. After a mean follow-up period of 22 months, NIN therapy was associated with a percentage stabilization of normal parenchyma (from 81.3 ± 11.8% to 78.6 ± 15.6%; P = not significant) and ILD (from 14.5 ± 10.4% to 16.7 ± 14.2%; P = not significant) both in SSc-ILD and IPF, avoiding the loss of normal parenchyma (from 87.4 ± 7.3% to 78.8 ± 16.7%; P < 0.001) and ILD worsening (from 9.0 ± 5.9% to 16.5 ± 14.8%; P < 0.001) observed in the untreated cohort. VRS was significantly increased regardless of antifibrotic therapy (P < 0.001). NIN treated patients who experienced a clinically meaningful worsening at pulmonary function tests or at the reported dyspnoea, presented a significant loss of normal parenchyma in parallel with a greater increase in ILD (P < 0.05 for all).

Conclusion

NIN appears effective in reducing the radiological decline of pulmonary fibrosis. Quantitative HRCT is proposed as a surrogate outcome measure for clinical practice and future trials.