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Updated: Jul 12, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
The spectrum of scleroderma lung disease
Kristin B Highland1, Margaret C Garin, Kevin K Brown
1Division of Pulmonary Critical Care, Allergy, and Sleep Medicine, Medical University of South Carolina, Charleston, South Carolina 29425, USA. highlakb@musc.edu
Seminars in Respiratory and Critical Care Medicine
|September 4, 2007
Summary
Systemic sclerosis frequently causes death through pulmonary disease, affecting lungs via hypertension, interstitial disease, and cancer. This review details lung complications, treatments, and transplantation options.
Area of Science:
- Rheumatology
- Pulmonology
- Internal Medicine
Background:
- Pulmonary disease is the leading cause of mortality in patients with systemic sclerosis.
- Systemic sclerosis impacts the lungs through diverse pathologies.
Purpose of the Study:
- To comprehensively review the spectrum of pulmonary manifestations in systemic sclerosis.
- To outline current knowledge on etiology, risk factors, diagnosis, treatment, and prognosis for each lung condition.
- To discuss lung transplantation in this patient population.
Main Methods:
- Literature review of pulmonary manifestations in systemic sclerosis.
- Synthesis of current evidence on lung disease in systemic sclerosis patients.
Main Results:
- Systemic sclerosis encompasses pulmonary hypertension, interstitial lung disease, restrictive and obstructive disorders, pleural disease, lung cancer, and aspiration.
- Each manifestation presents unique etiological factors, risk profiles, diagnostic challenges, and therapeutic strategies.
Conclusions:
- Understanding the multifaceted pulmonary involvement is crucial for managing systemic sclerosis.
- Prompt diagnosis and tailored treatment are essential for improving outcomes and prognosis in patients with systemic sclerosis-related lung disease.
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