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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
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Pathogenesis of systemic sclerosis associated interstitial lung disease
Svetlana I Nihtyanova1, Christopher P Denton1
1Centre for Rheumatology and Connective Tissue Diseases, University College London, London, UK.
Journal of Scleroderma and Related Disorders
|April 6, 2022
Summary
Systemic sclerosis-interstitial lung disease involves genetic and environmental factors, inflammation, and epithelial injury. Understanding these elements is key to managing this autoimmune condition and its progression.
Area of Science:
- Immunology
- Pulmonology
- Rheumatology
Background:
- Systemic sclerosis is an autoimmune disease causing fibrosis in skin and organs.
- Interstitial lung disease (ILD) is a major cause of mortality in systemic sclerosis.
- The commonest ILD pattern in systemic sclerosis is non-specific interstitial pneumonia.
Purpose of the Study:
- To review factors involved in systemic sclerosis-interstitial lung disease (SSc-ILD) pathogenesis.
- To explore mechanisms leading to sustained fibrosis in SSc-ILD.
- To propose a model for SSc-ILD pathogenesis.
Main Methods:
- Literature review of genetic, environmental, cellular, and molecular factors.
- Analysis of inflammatory mediators and cellular players.
- Synthesis of information to propose a pathogenetic model.
Main Results:
- SSc-ILD pathogenesis involves susceptibility factors, inflammation (e.g., IL-6), epithelial injury (e.g., KL-6), and immune cells (monocytes, macrophages).
- Transforming growth factor beta is central to fibrosis.
- Various mediators (CCL2, CXCL4, CCL18) suggest immune involvement.
Conclusions:
- SSc-ILD pathogenesis is multifactorial, involving genetic, environmental, inflammatory, and cellular components.
- Understanding these factors is crucial for identifying progression risks and outcomes.
- A comprehensive model of SSc-ILD pathogenesis is proposed.
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