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Updated: May 10, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Treating skin and lung fibrosis in systemic sclerosis: a future filled with promise?
Milos Antic1, Jörg H W Distler, Oliver Distler
1Department of Rheumatology and Center of Experimental Rheumatology, University Hospital Zurich, Switzerland.
Abstract:
Systemic sclerosis (SSc) is a rare autoimmune disorder characterized by immune activation, vascular damage and an excessive accumulation of extracellular matrix proteins in the skin and internal organs. Despite its high morbidity and increased mortality, currently available treatment options for fibrotic manifestations of SSc remain limited and their clinical antifibrotic effects are borderline. In this review, novel insights from recently published clinical trials in SSc on treatment concepts such as mycophenolate mofetil, oral type I collagen, recombinant human relaxin and autologous hematopoietic stem cell transplantation are discussed. In the past decade the most significant progress in this field has been made by the identification of a large number of cellular and molecular key players in the pathogenesis of fibrotic disease manifestations. This has led to the identification of novel candidates as molecular targets for treatment of fibrotic diseases. On the basis of their level of evidence from preclinical studies and based on the availability of first clinical results, the most promising targets are presented including inhibitors of B-cells, tyrosine kinases, 5-hydroxytryptamin receptors, interleukin-6 and Wnt signalling.
Insights
Systemic sclerosis (SSc) treatments are limited, but new research identifies key cellular and molecular targets. Future therapies may focus on novel pathways to combat fibrotic disease manifestations.
Area of Science:
- Immunology
- Rheumatology
- Fibrosis Research
Background:
- Systemic sclerosis (SSc) is a rare autoimmune disease causing immune activation, vascular damage, and fibrosis.
- Current treatments for SSc fibrosis are limited with borderline efficacy.
- High morbidity and mortality rates underscore the need for improved SSc therapies.
Purpose of the Study:
- To review novel insights from recent clinical trials in SSc.
- To discuss emerging treatment concepts for fibrotic manifestations.
- To identify promising molecular targets for SSc treatment.
Main Methods:
- Review of recently published clinical trials in Systemic Sclerosis.
- Analysis of cellular and molecular key players in fibrotic disease pathogenesis.
- Evaluation of preclinical and early clinical data for novel therapeutic targets.
Main Results:
- Recent trials explored mycophenolate mofetil, oral type I collagen, recombinant human relaxin, and stem cell transplantation.
- Identification of numerous cellular and molecular targets involved in SSc fibrosis.
- Promising targets include inhibitors of B-cells, tyrosine kinases, 5-hydroxytryptamin receptors, interleukin-6, and Wnt signaling.
Conclusions:
- Significant progress in understanding SSc pathogenesis has revealed novel therapeutic targets.
- Inhibitors targeting identified pathways show promise for treating fibrotic manifestations.
- Further research and clinical development are needed to translate these findings into effective SSc treatments.
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