There is likely a role for using anti-fibrotic medications in patients with myositis-associated interstitial lung disease (MA-ILD) to slow down disease progression, especially in patients who have fibrotic and progressive disease. These patients however are currently being excluded from clinical trials of anti-fibrotic agents in progressive ILD because of the concomitant use of immunosuppression. The benefit of anti-fibrotic agents is being assessed in other rheumatic diseases and should be assessed in MA-ILD as well. They are a unique group of patients with a heterogeneous disease, and are much more frequently on concomitant immune-modulating therapy. As such, they should be studied on their own in separate clinical trials, and the use of nintedanib should be studied as an addition to standard of care immunosuppression. The objective of this study is to assess safety and tolerability of nintedanib in patients with MA-ILD.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
11
All patients will be given nintedanib 150 milligrams orally twice daily
Research Institute McGill University Health Center
Montreal, Quebec, Canada
Tolerability - completed doses
Percentage of subjects who complete 24 weeks on nintedanib. Subjects will be considered to have completed the 24 weeks of the study if they took 90% of the study drug doses.
Time frame: 24 weeks
Safety and adverse events
numbers of patients with adverse events during course of the study
Time frame: 24 weeks
Change in forced vital capacity
Time frame: 24 weeks
Change in diffusion capacity of the lung for carbon monoxide
Time frame: 24 weeks
Change in 6 minute walking distance
Time frame: 24 weeks
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