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Updated: May 5, 2026

Second Harmonic Generation Signals in Rabbit Sclera As a Tool for Evaluation of Therapeutic Tissue Cross-linking TXL for Myopia
Published on: January 6, 2018
Novel investigational agents for the treatment of scleroderma
Zsuzsanna Hortobagyi McMahan1, Fredrick M Wigley
1Johns Hopkins University, Medicine/Rheumatology , 55200 Eastern Avenue, MFL Center Tower, Suite 5300, Baltimore, MD 21224 , USA.
Introduction:
The purpose of this article is to highlight novel therapies that are being used in scleroderma (SSc). Therapeutic interventions in SSc generally target at least one of three ongoing biological processes characteristic of the disease: vasculopathy, autoimmunity and tissue fibrosis. Treatment decisions in SSc are determined by the level of disease activity and the degree of specific organ involvement. Traditional therapy has primarily focused on organ-specific management without clear evidence of overall disease modification.
Areas Covered:
The authors provide a review of a variety of agents, which are already used for other autoimmune diseases, that are now being used to treat active SSc skin or lung disease, including rituximab, tocilizumab and IVIG. Several agents studied in vitro and in animal models of fibrosis have shown promise, including bortezomib, LPA-1 antagonists, anti-CCN2 therapy, anti-IL-13 and thrombin antagonists. The authors also provide details on targeting intracellular molecular pathways and matricellular proteins, which is another novel area of investigation.
Expert Opinion:
Combination therapy may be necessary to control the complex biological network active in SSc. Most of the current evidence that suggest benefit of these agents is based on small population studies. Ultimately well-designed clinical trials are required to define the role of these agents in treating SSc.
Insights
Novel therapies for systemic sclerosis (SSc) target vasculopathy, autoimmunity, and fibrosis. Combination treatments show promise, but further clinical trials are needed to confirm efficacy for this complex autoimmune disease.
Area of Science:
- Rheumatology
- Immunology
- Fibrosis Research
Background:
- Systemic sclerosis (SSc) involves complex biological processes including vasculopathy, autoimmunity, and tissue fibrosis.
- Current treatment decisions are based on disease activity and organ involvement, often focusing on organ-specific management.
- Traditional SSc therapies lack clear evidence of overall disease modification.
Purpose of the Study:
- To review novel therapeutic agents for systemic sclerosis (SSc).
- To highlight treatments targeting SSc's core pathological mechanisms.
- To discuss emerging strategies for SSc management.
Main Methods:
- Review of agents used in other autoimmune diseases for SSc treatment (e.g., rituximab, tocilizumab, IVIG).
- Examination of agents studied in vitro and in animal models for fibrotic diseases (e.g., bortezomib, LPA-1 antagonists, anti-CCN2, anti-IL-13, thrombin antagonists).
- Exploration of novel approaches targeting intracellular pathways and matricellular proteins.
Main Results:
- Agents like rituximab, tocilizumab, and IVIG are being investigated for active SSc skin and lung disease.
- Preclinical studies show promise for agents targeting fibrosis, including bortezomib and anti-fibrotic therapies.
- Intracellular pathways and matricellular proteins represent new avenues for SSc therapeutic development.
Conclusions:
- Combination therapy may be essential for managing SSc's complex biological network.
- Current evidence supporting novel SSc agents often comes from small studies.
- Well-designed clinical trials are crucial to establish the definitive role of these agents in SSc treatment.
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