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

Isolated pulmonary hypertension in scleroderma.

S R Cox1, J G Walker, M Coleman

  • 1Department of Immunology, Allergy and Arthritis, Flinders Medical Centre, Adelaide, South Australia, Australia.

Internal Medicine Journal
|January 26, 2005
PubMed
Summary

Pulmonary hypertension (PHT) is a frequent cause of death in limited scleroderma, affecting over 11% of patients. This complication significantly shortens survival, highlighting the need for early intervention.

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

The impact of gastroesophageal reflux disease and its treatment on interstitial lung disease outcomes.

Arthritis research & therapy·2024
Same author

Evidence of chromosomal damage in scleroderma.

Pathology·2021
Same author

Anti-Ro52/TRIM21 is independently associated with pulmonary arterial hypertension and mortality in a cohort of systemic sclerosis patients.

Scandinavian journal of rheumatology·2021
Same author

Is contact between men who have sex with men and non-governmental organizations providing harm reduction associated with improved HIV outcomes?

HIV medicine·2020
Same author

Cancer risk assessment tools in primary care: a systematic review of randomized controlled trials.

Annals of family medicine·2015
Same author

Cost savings with a new screening algorithm for pulmonary arterial hypertension in systemic sclerosis.

Internal medicine journal·2015

Area of Science:

  • Rheumatology
  • Cardiology
  • Pulmonology

Background:

  • Isolated pulmonary hypertension (PHT) is the leading cause of death in limited cutaneous scleroderma.
  • Current treatments offer symptomatic relief but do not improve survival rates.

Purpose of the Study:

  • To determine the incidence, characteristics, and survival outcomes of patients with isolated PHT in a scleroderma cohort.
  • To understand the clinical course and prognostic factors associated with PHT in scleroderma.

Main Methods:

  • Systematic review of patient data from the South Australian Scleroderma Register.
  • Inclusion of both living and deceased patients for comprehensive analysis.

Main Results:

  • Thirty-four patients with isolated PHT were identified, predominantly with limited scleroderma.

Related Experiment Videos

  • Over 11% of limited scleroderma patients develop PHT as a late-stage complication (approx. 20 years post-symptoms).
  • PHT patients exhibited telangiectasia, digital ulcers, reduced diffusing capacity, and elevated pulmonary artery pressure, with significantly shortened survival (2.5 years mean post-symptom onset).

    • Conclusions:

    • Isolated PHT is a late-stage complication in at least 11% of limited cutaneous scleroderma patients, leading to rapid death from right heart failure.
    • Early intervention with endothelin-1 receptor antagonists may alter the prognosis of this fatal condition.