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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
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Systemic Sclerosis-Associated Pulmonary Hypertension: Spectrum and Impact
Mario Naranjo1, Paul M Hassoun1
1Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University, Baltimore, MD 21287, USA.
Diagnostics (Basel, Switzerland)
|June 2, 2021
Summary
Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is a deadly complication of autoimmune disease. Despite advances, new therapies and phenotyping are crucial for better patient outcomes.
Area of Science:
- Rheumatology
- Pulmonology
- Cardiology
Background:
- Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is a severe complication of systemic sclerosis.
- SSc-PAH is the primary cause of mortality in systemic sclerosis patients.
- Early identification and expert referral are crucial for SSc-PAH diagnosis and management.
Purpose of the Study:
- To highlight the critical need for improved outcomes in SSc-PAH.
- To emphasize the necessity of rigorous phenotyping for SSc-PAH.
- To underscore the urgent search for novel therapeutic strategies for SSc-PAH.
Main Methods:
- Review of existing screening modalities and risk assessments for SSc-PAH.
- Analysis of data from multicenter clinical trials involving SSc-PAH patients.
- Assessment of current understanding and therapeutic advancements in SSc-PAH.
Main Results:
- SSc-PAH remains a leading cause of mortality despite current treatments.
- Clinical trials have provided insights but have not significantly improved outcomes.
- Existing screening tools aim for early detection and referral.
Conclusions:
- Outcomes for SSc-PAH patients remain suboptimal.
- Rigorous phenotyping is essential for advancing SSc-PAH research.
- Novel therapies are urgently required to improve the prognosis of SSc-PAH.
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