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Updated: Dec 17, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Current and Potential New Targets in Systemic Sclerosis Therapy: a New Hope
Monique Hinchcliff1, Steven O'Reilly2
1Section of Rheumatology and allergy, Yale School of medicine, Yale University, New Haven, CT, USA.
Purpose Of Review:
Systemic sclerosis (SSc) is an autoimmune connective tissue disease in which there is an activation of fibroblast to a myofibroblast that secretes huge amounts of extracellular matrix. Currently, no treatment exists that modifies the fibrosis elements and new therapeutic targets are badly needed. This review examines the current state of treatments and emerging therapeutics.
Recent Findings:
Nintedanib was found to significantly reduce the rate of decline in SSc associated FVC, although it has no benefit on skin fibrosis. New cannabinoid receptor2 agonist has shown superb effects in phase II and results in phase III are anticipated. Other targets are currently being tested in clinical trials and new targets that are yet to be tested are increasing in the SSc literature. Nintedanib is now licenced for SSc interstitial lung disease but this does not modify the skin fibrosis. Current ongoing trials will determine the role of various targets. New targets are emerging as we gain a deeper understanding of disease pathogenesis.
Insights
Systemic sclerosis treatments are evolving, with Nintedanib approved for lung issues but not skin fibrosis. Emerging therapies show promise for this autoimmune disease, targeting fibrosis and offering new hope.
Area of Science:
- Rheumatology
- Immunology
- Pulmonology
Background:
- Systemic sclerosis (SSc) involves fibroblast activation and excessive extracellular matrix secretion.
- Current treatments lack efficacy in modifying SSc-related fibrosis.
- There is a critical need for novel therapeutic targets in SSc management.
Purpose of the Study:
- To review the current therapeutic landscape for Systemic Sclerosis.
- To examine emerging therapeutic targets and treatments for SSc.
- To assess the efficacy of existing and novel SSc interventions.
Main Methods:
- Literature review of current treatments for Systemic Sclerosis.
- Analysis of recent clinical trial findings for SSc therapeutics.
- Examination of emerging therapeutic targets in SSc pathogenesis.
Main Results:
- Nintedanib demonstrated efficacy in slowing FVC decline in SSc-associated interstitial lung disease but not skin fibrosis.
- Cannabinoid receptor 2 agonists show promising results in Phase II trials, with Phase III anticipated.
- Numerous other targets are under investigation in ongoing clinical trials for SSc.
Conclusions:
- Nintedanib is a licensed treatment for SSc-related lung disease, yet skin fibrosis remains unaddressed.
- Emerging therapeutics targeting novel pathways offer potential for improved SSc management.
- Continued research into SSc pathogenesis is crucial for identifying future treatment strategies.
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