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Connective tissue disease-related pulmonary arterial hypertension

Vivek Thakkar1, Edmund M T Lau2

  • 1Department of Rheumatology, Liverpool Hospital, Sydney, New South Wales, Australia; School of Medicine, Western Sydney University, Campbelltown, New South Wales, Australia; South Western Sydney Clinical School, University of New South Wales, Liverpool, Australia; Ingham Institute, Liverpool, Australia.

Best Practice & Research. Clinical Rheumatology
|July 17, 2016
PubMed

Insights

Advances in assessing and managing connective tissue disease-related pulmonary arterial hypertension (CTD-PAH) have improved patient outcomes. This study focuses on these crucial developments in treating this severe condition.

Area of Science:

  • Cardiology
  • Rheumatology
  • Pulmonology

Background:

  • Connective tissue disease-related pulmonary arterial hypertension (CTD-PAH) is a lethal condition with significant impact.
  • Systemic sclerosis is the primary cause of CTD-PAH, followed by other autoimmune diseases.
  • Clinical registries provide essential data on disease burden, risk factors, and treatment trends in CTD-PAH.

Purpose of the Study:

  • To review recent advances in the assessment and management of CTD-PAH.
  • To highlight improvements in treatment outcomes for this severe disease.
  • To focus on the evolution of CTD-PAH care over the past two decades.

Main Methods:

  • Review of clinical registries and published literature.
  • Analysis of trends in disease classification and diagnostic criteria.
  • Evaluation of evidence-based therapeutic strategies and specialized care centers.

Main Results:

  • Significant progress in disease classification and diagnostic accuracy.
  • Emergence of effective, evidence-based therapies, including combination treatments.
  • Improved patient outcomes attributed to enhanced management strategies and specialized PAH centers.

Conclusions:

  • Recent advances have substantially improved the outlook for patients with CTD-PAH.
  • Integrated care approaches, including early diagnosis and targeted therapies, are key to better outcomes.
  • Continued research and specialized centers are vital for advancing CTD-PAH treatment.

Keywords:
Connective tissue diseaseDiagnosisEarly detectionPulmonary arterial hypertensionPulmonary hypertensionReviewScreeningSurvivalSystemic sclerosisTreatment

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