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 scleroderma.

Barbara White1

  • 1Baltimore Veterans Affairs Medical Center, Research Service, Baltimore, MD 21201, USA. bwhite@umaryland.edu

Rheumatic Diseases Clinics of North America
|July 5, 2003
PubMed
Summary

Pulmonary fibrosis affects many scleroderma patients, potentially causing severe lung disease and death. Early detection and intervention are crucial for high-risk individuals to prevent functional impairment.

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

Medications for adults with type 2 diabetes: a living systematic review and network meta-analysis.

BMJ (Clinical research ed.)·2025
Same author

Cannabinoid receptor 2 agonist, lenabasum, for the treatment of pulmonary exacerbations in cystic fibrosis.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society·2025
Same author

Comparative Efficacy of Immunosuppressive Therapies in the Treatment of Diffuse Cutaneous Systemic Sclerosis.

ACR open rheumatology·2025
Same author

Development and Evaluation of the Dermatomyositis Outcomes for Muscle and Skin as an Outcome Measure in Dermatomyositis Clinical Trials.

JID innovations : skin science from molecules to population health·2025
Same author

Long-Term Safety and Efficacy of Lenabasum, a Cannabinoid Receptor Type 2 Agonist, in Patients with Dermatomyositis with Refractory Skin Disease: Follow-Up Data from a 3-Year Open-Label Extension Study.

JID innovations : skin science from molecules to population health·2024
Same author

Bentracimab for Ticagrelor Reversal in Patients Undergoing Urgent Surgery.

NEJM evidence·2024

Area of Science:

  • Rheumatology
  • Pulmonology
  • Immunology

Background:

  • Scleroderma frequently involves the lungs, leading to pulmonary fibrosis in many patients.
  • Pulmonary fibrosis is a significant cause of mortality in scleroderma, progressing to severe restrictive lung disease in approximately 15% of cases.

Purpose of the Study:

  • To highlight the importance of early evaluation for pulmonary fibrosis in scleroderma patients.
  • To identify risk factors for developing and progressing pulmonary fibrosis in scleroderma.

Main Methods:

  • Assessment of risk factors including diffuse cutaneous scleroderma and anti-Scl-70 antibodies.
  • Evaluation of pulmonary function tests, high-resolution computed tomography (HRCT) of the lungs, Doppler echocardiogram, and bronchoalveolar lavage.
  • Consideration of cyclophosphamide therapy for patients not participating in clinical trials.

Main Results:

  • Diffuse cutaneous scleroderma and anti-Scl-70 antibodies are associated with developing pulmonary fibrosis.
  • Low pulmonary function test results and lung inflammation predict progressive disease and mortality.
  • Early identification of disease extent, severity, and inflammation guides therapy for at-risk patients.

Conclusions:

  • Early and comprehensive evaluation is essential for managing pulmonary fibrosis in scleroderma.
  • Identifying high-risk patients allows for timely therapeutic interventions to prevent severe functional decline.
  • Cyclophosphamide serves as a treatment option for eligible patients with scleroderma-related pulmonary fibrosis.

Related Experiment Videos