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Updated: Jun 24, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Pulmonary involvements in patients with collagen disease]
1Division of Rheumatology and Infectious Disease, Department of Internal Medicine, Kitasato University School of Medicine.
Nihon Rinsho. Japanese Journal of Clinical Medicine
|March 14, 2009
Summary
Pulmonary complications in collagen diseases, like scleroderma and lupus, can be severe and resistant to treatment. Early immunosuppressive therapy, including cyclophosphamide, shows promise for managing interstitial pneumonitis and alveolar hemorrhage.
Area of Science:
- Rheumatology and Pulmonology
- Immunology and Autoimmune Diseases
Context:
- Collagen diseases frequently manifest with pulmonary complications.
- Certain pulmonary involvements, including interstitial pneumonitis in scleroderma, acute diffuse alveolar damage (DAD) in polymyositis/dermatomyositis (PM/DM), alveolar hemorrhage, and pulmonary hypertension, are often resistant to current treatments and carry a poor prognosis.
Purpose:
- To review the challenges and potential therapeutic strategies for severe pulmonary manifestations in collagen vascular diseases.
- To highlight the efficacy of specific immunosuppressive agents in managing refractory pulmonary conditions.
Summary:
- Interstitial pneumonitis in scleroderma may respond to intensive immunosuppressive treatment, with oral cyclophosphamide demonstrating efficacy in a randomized trial.
- Acute diffuse alveolar damage (DAD) in polymyositis/dermatomyositis (PM/DM) is a steroid-resistant complication often requiring combination therapy with corticosteroids and immunosuppressants like cyclosporin A or cyclophosphamide.
- Diffuse alveolar hemorrhage, seen in conditions such as ANCA-associated vasculitis and systemic lupus erythematosus, necessitates intensive treatment to control bleeding.
Impact:
- Early and targeted immunosuppressive therapy can potentially improve outcomes for patients with severe, treatment-resistant pulmonary involvement in collagen diseases.
- This review underscores the need for aggressive management strategies for life-threatening pulmonary complications in autoimmune disorders.
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