Related Experiment Video
Updated: Nov 9, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Antifibrotics in systemic sclerosis
Maria Martin-Lopez1, Patricia E Carreira1
1Rheumatology Department, Hospital Universitario 12 de Octubre, Madrid, Spain; Instituto de Investigación Hospital 12 de Octubre (imas12), Madrid, Spain.
Abstract:
Systemic sclerosis (SSc) is a rare and complex disease, involving multiple organs, with high morbidity and mortality. Fibrosis is the hallmark of SSc, although vascular and inflammatory mechanisms are also implicated in its pathogenesis. Disease management is challenging, due to its heterogeneous presentation, and to the limited number of controlled clinical trials to guide treating clinicians. Immunosuppressive agents have been used to prevent progression, especially in the lung, before irreversible injury occurs, with some, although modest, benefit. Nintedanib, a tyrosine kinase inhibitor, has recently demonstrated safety and efficacy in interstitial lung disease (ILD) associated with SSc, and many other antifibrotics are being assessed as possible beneficial therapies, with promising results. An important unmet need remains, to clarify to which patients, when, and with which agent therapy should be initiated, to achieve optimal outcomes. This review summarizes available evidence for current and emerging antifibrotic therapies in SSc patients.
Insights
Systemic sclerosis (SSc) treatment is challenging. This review covers current and emerging antifibrotic therapies for SSc, focusing on nintedanib for SSc-associated interstitial lung disease (ILD).
Area of Science:
- Rheumatology and Pulmonology
- Fibrotic diseases
- Autoimmune disorders
Background:
- Systemic sclerosis (SSc) is a rare, multi-organ autoimmune disease characterized by fibrosis, vascular damage, and inflammation.
- High morbidity and mortality associated with SSc present significant management challenges due to disease heterogeneity and limited clinical trials.
- Current treatments, including immunosuppressants, offer modest benefits, highlighting the need for more effective antifibrotic strategies.
Purpose of the Study:
- To review current and emerging antifibrotic therapies for Systemic Sclerosis (SSc).
- To summarize evidence on the efficacy and safety of antifibrotic agents in SSc patients.
- To address the unmet need for personalized treatment strategies in SSc management.
Main Methods:
- Literature review of clinical trials and studies on antifibrotic therapies in Systemic Sclerosis.
- Analysis of data on nintedanib's efficacy in SSc-associated interstitial lung disease (ILD).
- Evaluation of emerging antifibrotic agents and their potential in SSc treatment.
Main Results:
- Nintedanib, a tyrosine kinase inhibitor, shows demonstrated safety and efficacy in treating ILD associated with SSc.
- Several other antifibrotic agents are under investigation with promising preliminary results.
- Evidence suggests a growing therapeutic landscape for antifibrotic interventions in SSc.
Conclusions:
- Antifibrotic therapies, including nintedanib, represent a significant advancement in managing Systemic Sclerosis, particularly for lung manifestations.
- Further research is needed to define optimal patient selection, timing, and choice of antifibrotic agents for SSc.
- Personalized therapeutic approaches are crucial for improving outcomes in SSc patients.
Related Concept Videos
Heart Failure Drugs: Inhibitors of Renin-Angiotensin System
Rheumatic Heart Disease III: Medical Management
Heart Failure V: Medical Management
Atherosclerosis III: Management
Rheumatic Heart Disease IV: Nursing Management
Myocarditis III: Medical Management

