Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

[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
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Complete response to mTOR inhibitor following JAKi failure in severe pulmonary sarcoidosis.

Pulmonology·2024
Same author

[Lung transplantation for sarcoidosis].

Revue des maladies respiratoires·2023
Same author

[Updated indications and contraindications in 2022 for lung transplantation in France].

Revue des maladies respiratoires·2022
Same author

[What do the recent recommendations for the diagnosis and treatment of sarcoidosis mean ?]

La Revue de medecine interne·2022
Same author

[Diagnostic difficulties of chronic pulmonary berylliosis in France].

Revue des maladies respiratoires·2020
Same author

[The effect of air pollution in diffuse interstitial lung disease].

Revue des maladies respiratoires·2020
Same journal

[French version of the guidelines for connective tissue disease-associated ILD].

Revue des maladies respiratoires·2026
Same journal

[Pulmonary expression of an asymptomatic lysosomal storage disorder].

Revue des maladies respiratoires·2026
Same journal

[Factors associated with FEV1 evolution in cystic fibrosis patients treated by CFTR modulator tritherapy].

Revue des maladies respiratoires·2026
Same journal

[Subacute pulmonary coccidioidomycosis: A differential diagnosis of lung cancer].

Revue des maladies respiratoires·2026
Same journal

[Relevance of the ACT score in severe asthma with obesity: A pilot study].

Revue des maladies respiratoires·2026
Same journal

[Automated analysis of mandibular movements for the screening of obstructive sleep apnea].

Revue des maladies respiratoires·2026
See all related articles

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.

Related Experiment Videos

  • 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.