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Updated: Aug 9, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Assessing prognostic factors correlating with response to nintedanib for connective tissue disease-associated
Hiraku Kokubu1,2, Saki Takeuchi1, Takahisa Tozawa1
1Department of Dermatology, Japan Community Healthcare Organization Chukyo Hospital, Nagoya, Japan.
Insights
Early diagnosis and treatment with nintedanib are crucial for connective tissue disease-associated interstitial lung disease (CTD-ILD). Starting nintedanib promptly, particularly for high-risk patients, helps preserve lung function.
Area of Science:
- Pulmonology
- Rheumatology
- Pharmacology
Background:
- Connective tissue disease-associated interstitial lung disease (CTD-ILD) necessitates early intervention.
- Nintedanib is an antifibrotic medication used for ILD.
- Real-world data on nintedanib use in CTD-ILD is valuable.
Purpose of the Study:
- To evaluate the real-world effectiveness of nintedanib in patients with CTD-ILD.
- To identify patient subgroups that benefit most from early nintedanib initiation.
Main Methods:
- Retrospective analysis of CTD-ILD patients treated with nintedanib from January 2020 to July 2022.
- Review of medical records and stratified analysis based on patient demographics and disease characteristics.
Main Results:
- Nintedanib use was associated with preserved forced vital capacity (%FVC) in younger patients (<55 years), those treated within 10 months of ILD diagnosis, and those with less extensive pulmonary fibrosis (<35%).
- Conversely, a reduction in %FVC was observed in elderly patients (>70 years), males, those treated later (>80 months post-diagnosis), and those with severe diffusing capacity for carbon monoxide (<40% DLco) or extensive fibrosis (>35%).
Conclusions:
- Early diagnosis of ILD and timely initiation of antifibrotic therapy are critical for managing CTD-ILD.
- Nintedanib should be considered early for high-risk CTD-ILD patients, including the elderly, males, and those with significant pulmonary fibrosis or impaired DLco.
Objective:
For patients with connective tissue disease-associated interstitial lung disease (CTD-ILD), early medical intervention would be desirable. This study analyzed the real-world, single-center use of nintedanib for CTD-ILD patients.
Methods:
Patients with CTD who received nintedanib from January 2020 to July 2022 were enrolled. Medical records review and stratified analyses of the collected data were conducted.
Results:
Reduction in the percentage of predicted forced vital capacity (%FVC) was seen in the elderly group (>70 years; P = .210), males (P = .027), the late group who started nintedanib >80 months after confirmation of an ILD disease activity (P = .03), the severe %DLco (diffusing capacity for carbon monoxide as a percentage of predicted) group (<40%; P = .20), the group who had extensive pulmonary fibrosis at the beginning of nintedanib (pulmonary fibrosis score >35%), and the low-dose group (nintedanib 50-100 mg/d; P = .40). %FVC did not decrease by >5% in the young group (<55 years), the early group who started nintedanib within 10 months after confirmation of an ILD disease activity, and the group whose pulmonary fibrosis score at the beginning of nintedanib was <35%.
Conclusion:
It is important to diagnose ILD early and start antifibrotic drugs with proper timing for cases in need. It is better to start nintedanib early, especially for patients at risk (>70 years old, male, <40% DLco, and >35% areas of pulmonary fibrosis).
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