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Updated: Jul 9, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Interstitial lung disease in systemic sclerosis]
L Mouthon1, A Berezné, M Brauner
1Service de Médecine Interne, Hôpital Cochin, Centre de Référence pour la sclérodermie systémique, Assistance Publique-Hôpitaux de Paris et Université Paris-Descartes, Faculté de Médecine Paris-Descartes, Paris, France. luc.mouthon@cch.ap-hop-paris.fr
Revue Des Maladies Respiratoires
|November 23, 2007
Summary
Systemic sclerosis (SSc) interstitial lung disease (ILD) affects up to 75% of patients, often presenting as non-specific interstitial pneumonia. While cyclophosphamide shows modest benefits for respiratory function, its role in rapidly progressive ILD requires further confirmation.
Area of Science:
- Rheumatology
- Pulmonology
- Immunology
Context:
- Interstitial lung diseases (ILD) are a significant complication of systemic sclerosis (SSc), occurring in up to 75% of patients.
- ILD in SSc is often characterized by non-specific interstitial pneumonia, distinguishing it from idiopathic ILD and generally conferring a better prognosis.
- Despite a better prognosis than idiopathic ILD, SSc-associated ILD remains a leading cause of mortality in SSc patients.
Purpose:
- To summarize the current understanding of interstitial lung diseases (ILD) in systemic sclerosis (SSc).
- To review the diagnostic approaches and prognostic factors for SSc-ILD.
- To discuss the established and emerging treatment strategies for SSc-ILD.
Summary:
- Early detection of SSc-ILD is crucial, utilizing high-resolution computed tomography and pulmonary function tests, including DLCO.
- While anti-fibrotic therapies have shown limited efficacy, cyclophosphamide has demonstrated a modest, significant benefit on respiratory function in recent studies.
- A potential benefit of pulsed intravenous cyclophosphamide with prednisone exists for a subgroup of patients with rapidly progressive SSc-ILD, though further validation is needed.
Impact:
- Highlights the importance of early detection and monitoring of SSc-ILD.
- Provides an overview of current treatment options and their limitations.
- Suggests a potential therapeutic avenue for rapidly progressive SSc-ILD, emphasizing the need for continued research.
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