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Updated: Jun 22, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Overview of pathogenesis of systemic sclerosis
D J Abraham1, T Krieg, J Distler
1Centre for Rheumatology and Connective Tissue Disease, Royal Free and University College Medical School, University College London, Hampstead, London, UK. d.abraham@medsch.ucl.ac.uk
Abstract:
The aetiology of SSc is subject to ongoing research, as the precise events that underlie the development of this disease remain unclear. The pathogenesis is known to involve endothelium, epithelium, fibroblasts, innate and adaptive immune systems and their component immunological mediators. Endothelial cell damage may be the initiating factor, but the precise triggering event(s) remain elusive. Angiogenesis also appears to be dysregulated. Vasculopathy shows similarities in different organs (e.g. pulmonary arterial hypertension, renal disease, digital tip ulcers). Endothelin-1 is a potent mediator of vasculopathy, and hence represents a highly relevant target for intervention of vascular features in SSc.
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