Related Experiment Video
Updated: Apr 27, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Low-dose oral imatinib in the treatment of systemic sclerosis interstitial lung disease unresponsive to
Introduction:
Pulmonary involvement represents a major cause of death of systemic sclerosis (SSc) patients. Recent data suggest that tyrosine kinase inhibitors, such as imatinib, may be a therapeutic option for SSc patients. However, preliminary published clinical trials were inconclusive about imatinib efficacy and showed side effects. The purpose of this study was to verify efficacy and tolerability of low-dose imatinib on interstitial lung disease in a cohort of SSc patients unresponsive to cyclophosphamide therapy.
Methods:
Thirty consecutive SSc patients with active pulmonary involvement, unresponsive to cyclophosphamide, were treated with imatinib 200 mg/day for 6 months followed by a 6-month follow-up. A "good response" was defined as an increase of forced vital capacity (FVC) by more of 15% and/or increase of diffusing capacity of carbon monoxide (DLCO) >15% and PaO2 > 90% of initial value and high-resolution computed tomography (HRCT)-scan pattern unchanged or improved.
Results:
Twenty-six patients completed the study. Three patients died and one patient was lost to follow-up. Four patients (15.32%) had a good response, 7 worsened and 15 had a stabilized lung disease. Overall, 19 (73.07%) patients had an improved or stabilized lung disease. After a 6-month follow-up, 12 (54.5%) of the 22 patients showed an improved or stabilized lung disease.
Conclusions:
Lung function was stabilized in a large proportion of patients unresponsive to cyclophosphamide therapy and a beneficial outcome emerged from the analysis of HRCT lung scans. There was no significant improvement of skin involvement, and the low dose was well tolerated. These data provide useful suggestions to design future randomized clinical trials for SSc therapeutics.
Trial Registration:
ClinicalTrials.gov NCT00573326. Registered 13 December 2007.
Insights
Low-dose imatinib stabilized lung disease in systemic sclerosis (SSc) patients unresponsive to cyclophosphamide. The treatment was well-tolerated, suggesting potential for future clinical trials in SSc interstitial lung disease.
Area of Science:
- Pulmonology
- Rheumatology
- Pharmacology
Background:
- Pulmonary involvement is a leading cause of mortality in systemic sclerosis (SSc).
- Tyrosine kinase inhibitors like imatinib show potential for SSc treatment, but prior trials yielded inconclusive results on efficacy and tolerability.
- This study investigated low-dose imatinib in SSc patients with interstitial lung disease refractory to cyclophosphamide.
Purpose of the Study:
- To evaluate the efficacy and tolerability of low-dose imatinib in SSc patients with pulmonary involvement.
- To assess imatinib's impact on lung function and high-resolution computed tomography (HRCT) findings in cyclophosphamide-refractory SSc patients.
Main Methods:
- Thirty SSc patients with active pulmonary involvement, unresponsive to cyclophosphamide, received imatinib 200 mg/day for 6 months.
- Response was defined by improvements in forced vital capacity (FVC), diffusing capacity of carbon monoxide (DLCO), PaO2, and HRCT scan results.
- A 6-month follow-up period was included to assess sustained effects.
Main Results:
- Of 26 completers, 15.32% (4 patients) showed a good response, while 7 worsened and 15 had stabilized lung disease.
- Overall, 73.07% (19 patients) demonstrated improved or stabilized lung disease after 6 months of treatment.
- Following a 6-month follow-up, 54.5% (12 patients) maintained improved or stabilized lung disease.
Conclusions:
- Low-dose imatinib stabilized lung function in a significant proportion of SSc patients unresponsive to cyclophosphamide.
- HRCT scans indicated beneficial outcomes, though skin involvement did not improve.
- The low-dose regimen was well-tolerated, providing valuable data for future SSc therapeutic trials.
More Related Videos
06:43Robotic Duodenal Sleeve Resection for Gastrointestinal Stromal Tumor with Rare Exon 8 KIT Mutation Following Neoadjuvant Imatinib
Published on: April 3, 2026
04:04Endobronchial Ultrasound-guided Intratumoral Injection of Cisplatin for the Treatment of Isolated Mediastinal Recurrence of Lung Cancer
Published on: February 12, 2017