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Updated: Jul 10, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Scleroderma lung disease: appropriate management
1Centre for Immunology, St Vincent's Hospital and University of New South Wales, Sydney, Australia.
Summary
Scleroderma lung disease, including pulmonary hypertension, has limited treatment options. New localized therapies show promise for managing scleroderma
Area of Science:
- Rheumatology and Pulmonology
- Rare autoimmune diseases
- Scleroderma and its complications
Background:
- Scleroderma frequently affects the lungs through interstitial lung disease, pulmonary vascular disease, and bronchiolitis.
- Pulmonary hypertension is the primary cause of mortality in scleroderma patients.
- Controlled treatment trials for scleroderma-associated lung diseases are scarce.
Purpose of the Study:
- To review current understanding and therapeutic approaches for lung involvement in scleroderma.
- To discuss the challenges and future directions in managing scleroderma-related pulmonary conditions.
Main Methods:
- Review of uncontrolled studies and pathological understanding of scleroderma.
- Analysis of current treatment strategies for interstitial and pulmonary vascular disease.
- Exploration of emerging therapeutic options, including locally administered therapies.
Main Results:
- Progressive interstitial lung disease is often managed with immunosuppressants like cyclophosphamide and prednisone.
- Pulmonary vascular disease in scleroderma carries a poor prognosis with limited current therapies.
- Locally administered therapies are emerging as a promising strategy to mitigate systemic drug side effects.
Conclusions:
- Treatment for scleroderma's lung manifestations relies on rational choices based on limited evidence.
- Pulmonary vascular disease management requires further research and novel therapeutic development.
- Locally administered treatments offer potential for improved safety and efficacy in scleroderma lung disease.
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