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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
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The contemporary management of systemic sclerosis
Maysoon Eldoma1,2,3, Janet Pope1,4
1a Division of Rheumatology, St. Joseph's Health Care, Department of Medicine , University of Western Ontario , Ontario , Canada.
Expert Review of Clinical Immunology
|June 7, 2018
Summary
Systemic sclerosis (SSc) treatments offer modest improvements by targeting affected organs. Future strategies may involve combination therapies and personalized approaches to improve efficacy and reduce mortality in SSc patients.
Area of Science:
- Rheumatology
- Immunology
- Internal Medicine
Background:
- Systemic sclerosis (SSc) is a rare autoimmune disease with complex pathophysiology, leading to organ damage despite current treatments.
- Vascular dysfunction, fibrosis, inflammation, and autoantibodies are key features of SSc.
- Existing therapies provide only modest improvements, highlighting the need for advanced treatment strategies.
Purpose of the Study:
- To review current treatment strategies for Systemic Sclerosis (SSc) across various affected organs.
- To outline an evidence-based approach to SSc treatment tailored to disease manifestations.
- To discuss future directions for improving SSc treatment efficacy and patient outcomes.
Main Methods:
- Literature review of treatment strategies for SSc.
- Evaluation of therapies based on organ involvement (skin, pulmonary, cardiac, renal, musculoskeletal, gastrointestinal).
- Analysis of current evidence and expert commentary on SSc management.
Main Results:
- Pulmonary hypertension and interstitial lung disease are the leading causes of mortality in SSc.
- Current treatments often adapt therapies from other conditions, such as idiopathic pulmonary arterial hypertension treatments for vascular manifestations.
- Treatment efficacy is modest, with significant unmet needs in managing SSc.
Conclusions:
- SSc management requires a multi-organ approach, with pulmonary and interstitial lung disease being critical concerns.
- Future SSc treatment may involve combination and maintenance therapies, similar to other autoimmune diseases.
- Personalizing treatment based on patient phenotype is crucial for enhancing efficacy and reducing SSc-related mortality.
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