New England Journal of Medicine MAY 30, 2019

Efficacy and Safety of Nintedanib in Systemic Sclerosis-Associated Interstitial Lung Disease (SENSCIS)

Distler O, Highland KB, Gahlemann M, et al.

Bottom Line

The SENSCIS trial demonstrated that nintedanib significantly reduces the annual rate of decline in forced vital capacity (FVC) in patients with systemic sclerosis-associated interstitial lung disease compared to placebo.

Key Findings

1. The primary endpoint was met: the adjusted annual rate of decline in FVC was -52.4 mL/year in the nintedanib group compared to -93.3 mL/year in the placebo group.
2. This resulted in an absolute difference of 41.0 mL/year (95% CI 2.9 to 79.0; P=0.04), representing a 44% reduction in the rate of lung function decline.
3. The clinical benefit of nintedanib in slowing FVC decline was consistent regardless of the use of background mycophenolate therapy at baseline.
4. Gastrointestinal adverse events, particularly diarrhea, were significantly more common in the nintedanib group, occurring in 75.7% of patients versus 31.6% in the placebo group.
5. Nintedanib did not demonstrate a significant effect on secondary outcomes including the modified Rodnan skin score or the St. George’s Respiratory Questionnaire (SGRQ) total score.

Study Design

Design
RCT
Double-Blind
Sample
576
Patients
Duration
52 wk
Median
Setting
Multicenter, Global
Population Adults with systemic sclerosis-associated interstitial lung disease with symptom onset within 7 years, fibrotic ILD >10% on HRCT, and FVC >=40% predicted.
Intervention Nintedanib 150 mg twice daily.
Comparator Matched placebo twice daily.
Outcome Annual rate of decline in forced vital capacity (FVC) (mL/year) measured over 52 weeks.

Study Limitations

The 52-week duration is relatively short for a chronic, slowly progressive disease, although an open-label extension was conducted.
Gastrointestinal side effects were frequent and often necessitated dose reductions or treatment interruptions, which may affect long-term adherence in real-world clinical practice.
The study was not powered to detect differences in mortality or hospitalization rates, and no significant benefit was observed in skin fibrosis or patient-reported quality of life measures.

Clinical Significance

The SENSCIS trial established nintedanib as the first tyrosine kinase inhibitor approved by the FDA for the treatment of systemic sclerosis-associated interstitial lung disease, providing a critical therapeutic option for slowing the irreversible progression of pulmonary fibrosis in these patients.

Historical Context

Prior to the SENSCIS trial, there were no approved pharmacological treatments specifically indicated for slowing lung function decline in patients with systemic sclerosis-associated interstitial lung disease, leaving clinical management largely reliant on off-label immunosuppressive agents with limited evidence of efficacy.

Guided Discussion

High-yield insights from every perspective

Med Student
Medical Student

How does the pharmacological mechanism of nintedanib differ from traditional immunosuppressive therapies like mycophenolate mofetil in the context of systemic sclerosis-associated interstitial lung disease (SSc-ILD)?

Key Response

Nintedanib is a multi-tyrosine kinase inhibitor targeting platelet-derived growth factor receptors (PDGFR), fibroblast growth factor receptors (FGFR), and vascular endothelial growth factor receptors (VEGFR). Unlike immunosuppressants that target the inflammatory cascade and lymphocyte proliferation, nintedanib directly interferes with signaling pathways involved in the proliferation and differentiation of fibroblasts, the primary drivers of fibrosis in SSc-ILD.

Resident
Resident

Given the high incidence of gastrointestinal adverse events reported in the SENSCIS trial, what is the recommended management strategy for a patient who develops grade 2 diarrhea while on nintedanib for SSc-ILD?

Key Response

In the trial, 75.7% of patients on nintedanib experienced diarrhea. Management includes early intervention with anti-diarrheals (e.g., loperamide), dose reduction from 150 mg twice daily to 100 mg twice daily, or temporary treatment interruption until the symptom resolves. This is particularly relevant in SSc patients who may have pre-existing GI involvement or dysmotility.

Fellow
Fellow

The SENSCIS trial allowed background mycophenolate mofetil (MMF) use in nearly half of the study population. How should the subgroup analysis regarding background MMF use influence our clinical decision-making regarding sequential vs. upfront combination therapy?

Key Response

While nintedanib showed efficacy in reducing FVC decline regardless of background MMF use, the absolute rate of FVC decline was numerically lowest in the group receiving both medications. This suggests a potential synergistic effect of combining an anti-inflammatory (MMF) with an anti-fibrotic (nintedanib), though the trial was not formally powered to compare combination versus monotherapy directly.

Attending
Attending

The SENSCIS trial demonstrated a significant reduction in the rate of FVC decline but failed to show a significant difference in the Modified Rodnan Skin Score (mRSS) or the St. George's Respiratory Questionnaire (SGRQ). How should these discordant findings be framed when counseling a patient about the goals of therapy?

Key Response

The primary goal of nintedanib is the preservation of lung function (slowing the 'slope' of decline) rather than symptomatic improvement or reversal of skin disease. Clinicians must manage expectations by explaining that the drug is a long-term strategy to prevent future respiratory failure rather than a treatment for current breathlessness or cutaneous thickening.

Scholarly Review

Critical appraisal through the lens of expert reviewers and guideline development

PhD
PhD

SENSCIS utilized a random-coefficient regression model to analyze the primary endpoint. What are the specific advantages of using a slope-based analysis over a traditional change-from-baseline analysis in a chronic progressive disease like systemic sclerosis?

Key Response

A random-coefficient regression model (slope analysis) utilizes all available data points throughout the 52-week period rather than just the final measurement. This increases statistical power and provides a more robust estimate of the disease trajectory, effectively handling missing data which is common in trials involving patients with high morbidity and potential dropouts.

Journal Editor
Journal Editor

In the SENSCIS trial, the absolute difference in FVC decline between the nintedanib and placebo groups was 41 mL per year. Does this difference meet the threshold for a 'Minimal Clinically Important Difference' (MCID), and how might this affect the paper's perceived impact on clinical practice?

Key Response

While the result was statistically significant (p=0.04), a 41 mL difference is relatively small and falls below some established MCID thresholds for ILD. A critical reviewer would question whether this 'statistically significant' slowing of decline translates to 'clinically meaningful' outcomes like delayed time to death or supplemental oxygen use, which the 52-week trial duration was too short to capture.

Guideline Committee
Guideline Committee

How do the SENSCIS results compare to the evidence supporting mycophenolate mofetil in the SLS-II trial, and should current guidelines prioritize nintedanib as first-line therapy or an add-on for patients with progressive disease?

Key Response

The SLS-II trial established MMF as a standard of care due to its impact on FVC and better tolerability compared to cyclophosphamide. SENSCIS evidence supports nintedanib as the first FDA-approved therapy specifically for SSc-ILD. Most guideline committees (such as the ACR or ATS) now recommend nintedanib either as monotherapy or in combination with MMF, but often emphasize MMF first due to its broader effect on systemic features and the long-term safety data established in SSc.

Clinical Landscape

Noteworthy Related Trials

2016

Scleroderma Lung Study II

n = 142 · JAMA

Tested

Mycophenolate mofetil vs Cyclophosphamide

Population

Symptomatic systemic sclerosis-related interstitial lung disease

Comparator

Cyclophosphamide

Endpoint

Forced vital capacity (FVC) percent predicted

Key result: Mycophenolate mofetil was shown to be as effective as cyclophosphamide with a better side-effect profile.
2018

REACH Trial

n = 194 · Lancet Respir Med

Tested

Pirfenidone

Population

Progressive fibrosing interstitial lung disease

Comparator

Placebo

Endpoint

Forced vital capacity (FVC)

Key result: Pirfenidone slowed the decline of FVC in patients with progressive fibrosing ILDs, including SSc-ILD subtypes.
2021

FAST Trial

n = 226 · Lancet Rheumatol

Tested

Tocilizumab

Population

Systemic sclerosis-associated interstitial lung disease

Comparator

Placebo

Endpoint

Forced vital capacity (FVC) percentage predicted

Key result: Tocilizumab did not significantly improve FVC compared to placebo in patients with SSc-ILD, though it showed a trend toward benefit in skin outcomes.

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