# The Clinical Efficacy and Safety of Nintedanib in the Treatment of Interstitial Lung Disease Among Patients With Systemic Sclerosis: Systematic Review

**Authors:** Khaled S. Al Oweidat, Ahmed A. Abdulelah, Ahmad A. Toubasi, Mohammad Abdulelah, Nour Z. Alatteili, Zaid A. Abdulelah

PMC · DOI: 10.1155/carj/1682546 · Canadian Respiratory Journal · 2025-07-01

## TL;DR

This paper reviews the effectiveness and safety of nintedanib for treating lung disease in systemic sclerosis patients.

## Contribution

The study provides a systematic review of nintedanib's clinical use in SSc–ILD, highlighting its favorable safety profile.

## Key findings

- Nintedanib shows adequate clinical efficacy in treating SSc–ILD.
- The drug has a more favorable safety profile compared to other treatments.
- It is the first fully licensed medication for SSc–ILD.

## Abstract

Systemic sclerosis (SSc) is predominantly characterized by an array of cutaneous manifestations including Raynaud's phenomenon, calcinosis, telangiectasias, and skin fibrosis contributing toward substantial morbidity and diminished quality of life. The monumental impact of the disease regarding mortality is due to its pulmonary involvement known as SSc-associated interstitial lung disease (SSc–ILD). Currently, treatment is chiefly directed toward impeding disease progression with the mainstay treatment approaches involving the utilization of cyclophosphamide, mycophenolate mofetil, rituximab, and tocilizumab. Recently, a tyrosine kinase inhibitor, nintedanib, has been approved for the treatment of SSc–ILD and thus became the first medication to be fully licensed for SSc–ILD. A systematic review based on the Preferred Reporting Items of Systematic Review with Meta-analysis (PRISMA) was conducted after successful registration in PROSPERO to evaluate the efficacy and safety of nintedanib in SSc–ILD. We searched PubMed, Scopus, and CENTRAL up to the first of September 2023 utilizing the following keywords: ((Diffuse Parenchymal Lung Disease) OR (Diffuse Parenchymal Lung Diseases) OR (Interstitial Lung Disease) OR (Interstitial Lung Diseases) OR (Interstitial Pneumonia) OR (Interstitial Pneumonitis) OR (Pulmonary Fibrosis)) AND ((Systemic Scleroderma) OR (Systemic Scleroderma)) AND ((BIBF 1120) OR (BIBF-1120) OR (BIBF1120) OR (Nintedanib esylate) OR (Ofev) OR (Vargatef)). The clinical safety profile of nintedanib was deemed more favorable than other therapeutic regimens currently utilized, in addition to adequate clinical efficacy toward SSc–ILD.

## Linked entities

- **Chemicals:** nintedanib (PubChem CID 135423438), cyclophosphamide (PubChem CID 2907), mycophenolate mofetil (PubChem CID 5281078)
- **Diseases:** Systemic Sclerosis (MONDO:0005100), Interstitial Lung Disease (MONDO:0015925)

## Full-text entities

- **Genes:** TXK (TXK tyrosine kinase) [NCBI Gene 7294] {aka BTKL, PSCTK5, PTK4, RLK, TKL}
- **Diseases:** calcinosis (MESH:D002114), telangiectasias (MESH:D013684), SSc (MESH:D012595), Diffuse Parenchymal Lung Disease (MESH:D017563), Pulmonary Fibrosis (MESH:D011658), Raynaud's phenomenon (MESH:D011928), skin fibrosis (MESH:D005355)
- **Chemicals:** tocilizumab (MESH:C502936), cyclophosphamide (MESH:D003520), rituximab (MESH:D000069283), mycophenolate mofetil (MESH:D009173), Nintedanib (MESH:C530716)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

1 figure with captions in the complete paper: https://tomesphere.com/paper/PMC12237565/full.md

## References

22 references — full list in the complete paper: https://tomesphere.com/paper/PMC12237565/full.md

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Source: https://tomesphere.com/paper/PMC12237565