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Nintedanib for Systemic Sclerosis–Associated Interstitial Lung Disease
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BACKGROUND: Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis and a leading cause of systemic sclerosis-related death. Nintedanib, a tyrosine kinase inhibitor, has been shown to have antifibrotic and antiinflammatory effects in preclinical models of systemic sclerosis and ILD. METHODS: We conducted a randomized, double-blind, placebo-controlled trial to investigate the efficacy and safety of nintedanib in patients with ILD associated with systemic sclerosis. Patients who had systemic sclerosis with an onset of the first non-Raynaud's symptom within the past 7 years and a high-resolution computed tomographic scan that showed fibrosis affecting at least 10% of the lungs were randomly assigned, in a 1:1 ratio, to receive 150 mg of nintedanib, administered orally twice daily, or placebo. The primary end point was the annual rate of decline in forced vital capacity (FVC), assessed over a 52-week period. Key secondary end points were absolute changes from baseline in the modified Rodnan skin score and in the total score on the St. George's Respiratory Questionnaire (SGRQ) at week 52. RESULTS: A total of 576 patients received at least one dose of nintedanib or placebo; 51.9% had diffuse cutaneous systemic sclerosis, and 48.4% were receiving mycophenolate at baseline. In the primary end-point analysis, the adjusted annual rate of change in FVC was -52.4 ml per year in the nintedanib group and -93.3 ml per year in the placebo group (difference, 41.0 ml per year; 95% confidence interval [CI], 2.9 to 79.0; P = 0.04). Sensitivity analyses based on multiple imputation for missing data yielded P values for the primary end point ranging from 0.06 to 0.10. The change from baseline in the modified Rodnan skin score and the total score on the SGRQ at week 52 did not differ significantly between the trial groups, with differences of -0.21 (95% CI, -0.94 to 0.53; P = 0.58) and 1.69 (95% CI, -0.73 to 4.12 [not adjusted for multiple comparisons]), respectively. Diarrhea, the most common adverse event, was reported in 75.7% of the patients in the nintedanib group and in 31.6% of those in the placebo group. CONCLUSIONS: Among patients with ILD associated with systemic sclerosis, the annual rate of decline in FVC was lower with nintedanib than with placebo; no clinical benefit of nintedanib was observed for other manifestations of systemic sclerosis. The adverse-event profile of nintedanib observed in this trial was similar to that observed in patients with idiopathic pulmonary fibrosis; gastrointestinal adverse events, including diarrhea, were more common with nintedanib than with placebo. (Funded by Boehringer Ingelheim; SENSCIS ClinicalTrials.gov number, NCT02597933.).
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“This observation is therefore in accordance with recent clinical trials showing that antifibrotic therapies are effective in progressive fibrotic interstitial lung diseases other than IPF [20,26,27].”
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“nintedanib has also now been approved for systemic sclerosis (SSc)-associated ILD (SSc-ILD), based on the positive outcome of Safety and Efficacy of Nintedanib in Systemic Sclerosis (SENSCIS) trial (Distler et al., 2019).”
From Pulmonary fibrosis: Emerging diagnostic and therapeutic strategies (2023) · cites this paper as Distler et al., 2019
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Self-citation
Metadata
- Authors
- Distler, O., Highland, K. B., Gahlemann, M., Azuma, A., Fischer, A., Mayes, M. D., Raghu, G., Sauter, W., Girard, M., Alves, M., Clerisme-Beaty, E., Stowasser, S., Tetzlaff, K., Kuwana, M., & Maher, T. M.
- Affiliations
- Cleveland Clinic (US)
- ORCID
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 2019-06-27
- Volume
- 380
- Issue
- 26
- Pages
- 2518-2528
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2945521814
- Cited by
- 1,647
- References
- 36
- Citations by year
- 2026: 139 · 2025: 203 · 2024: 215 · 2023: 231 · 2022: 243 · 2021: 299 · 2020: 247 · 2019: 68
- FWCI
- 157.88
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- New England Journal of Medicine (publishedVersion)
- PubMed (publishedVersion)
- Zurich Open Repository and Archive (University of Zurich) (acceptedVersion, other-oa)
- Ghent University Academic Bibliography (Ghent University) (submittedVersion)
- Ghent University Academic Bibliography (Ghent University) (submittedVersion, other-oa)
- Digital Access to Libraries (Université catholique de Louvain (UCL), l'Université de Namur (UNamur) and the Université Saint-Louis (USL-B)) (submittedVersion, other-oa)
- Dépôt institutionnel de l'Université libre de Bruxelles (Université Libre de Bruxelles) (submittedVersion, other-oa)
- Archivio Istituzionale della Ricerca - Università degli Studi della Campania "Luigi Vanvitelli" (submittedVersion)
- CINECA IRIS Institutial Research Information System (University of Genoa) (submittedVersion, other-oa)
- IRIS Research product catalog (Sapienza University of Rome) (submittedVersion)
- Spiral (Imperial College London) (submittedVersion)
- Zurich Open Repository and Archive (University of Zurich) (submittedVersion, other-oa)
- Zurich Open Repository and Archive (University of Zurich)
- Funders
- Boehringer Ingelheim
- Topics
- Health Sciences › Medicine › Rheumatology › Systemic Sclerosis and Related Diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Keywords
- nintedanib, systemic sclerosis, systemic sclerosis-associated interstitial lung disease, tyrosine kinase inhibitors, antifibrotic therapy, forced vital capacity decline, pulmonary fibrosis, diffuse cutaneous systemic sclerosis, skin involvement, quality of life, diarrhea, adverse events, mycophenolate, modified Rodnan skin score, St George's Respiratory Questionnaire
- MeSH terms
- Administration, Oral, Adult, Diarrhea, Double-Blind Method, Enzyme Inhibitors, Female, Humans, Indoles, Male, Middle Aged, Protein-Tyrosine Kinases, Scleroderma, Systemic, Vital Capacity, Lung Diseases, Interstitial, Disease Progression
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