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Updated: Jul 13, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Therapeutic options for systemic sclerosis
1Dept. of Rheumatology, Seth GS Medical College and KEM Hospital, Mumbai.
Abstract:
Systemic sclerosis is a uncommon connective tissue disorder characterized by vascular damage, immune cell activation and fibrogenesis. Each of these components may respond to different therapies. Therefore, a combination strategy treating all three processes is more likely to control the disease than single agent therapy. Clinical trials have gone a long way towards defining the therapy of scleroderma and many drugs previously used for scleroderma have been critically assessed. Angiotensin blockade is effective in treating as well as preventing scleroderma renal crisis. The 9-year cumulative survival has improved from 38% to 68% after the introduction of angiotensin blockade. There is definitive evidence supporting the use of cyclophosphamide in systemic sclerosis associated alveolitis. Newer molecules aimed at various cytokines are being tried. The therapy for systemic sclerosis is far from perfect at present. But, individualization of the treatment with respect to stage and subset of disease as well as organ involvement can eventually result in rational, effective management.
Insights
Systemic sclerosis treatment requires a combined approach targeting vascular damage, immune activation, and fibrogenesis. Angiotensin blockade and cyclophosphamide show promise, improving survival and managing alveolitis in systemic sclerosis patients.
Area of Science:
- Rheumatology
- Immunology
- Vascular Biology
Background:
- Systemic sclerosis is a rare connective tissue disease with complex pathophysiology.
- Key features include vascular damage, immune cell activation, and excessive fibrogenesis.
- These distinct pathological processes may necessitate multifaceted therapeutic strategies.
Purpose of the Study:
- To review current therapeutic strategies for systemic sclerosis.
- To assess the efficacy of various treatments, including combination therapies.
- To highlight the importance of individualized treatment approaches.
Main Methods:
- Critical assessment of clinical trial data for systemic sclerosis therapies.
- Review of evidence for specific drug classes, such as angiotensin receptor blockers and cyclophosphamide.
- Exploration of emerging therapeutic targets, including cytokine modulators.
Main Results:
- Angiotensin blockade significantly improves survival and prevents renal crisis in systemic sclerosis.
- Cyclophosphamide is definitively effective for treating associated alveolitis.
- Ongoing research explores novel agents targeting cytokines.
Conclusions:
- Current systemic sclerosis therapy is evolving but not yet perfect.
- Combination therapy offers a more promising approach than single-agent treatment.
- Individualized treatment based on disease stage, subset, and organ involvement is crucial for effective management.
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