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Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients

Yan-Jie Hao1, Xin Jiang2, Wei Zhou1

  • 1Dept of Rheumatology and Clinical Immunology, Peking University First Hospital, Beijing, China.

The European Respiratory Journal
|May 6, 2014
PubMed

Insights

Connective tissue disease-associated pulmonary arterial hypertension (CTD-APAH) in China shows similar survival rates to Western countries. Higher pulmonary vascular resistance (PVR) and alkaline phosphatase (ALP) levels are key risk factors for mortality in these patients.

Area of Science:

  • Cardiology
  • Rheumatology
  • Pulmonology

Background:

  • Connective tissue disease-associated pulmonary arterial hypertension (CTD-APAH) presents unique challenges in different populations.
  • Understanding contemporary survival and mortality predictors is crucial for effective management.

Purpose of the Study:

  • To investigate the characteristics, survival rates, and mortality risk factors in Chinese patients with CTD-APAH.
  • To compare findings with Western cohorts in the modern therapy era.

Main Methods:

  • A cohort of 129 adult patients with confirmed CTD-APAH from three Chinese referral centers were enrolled.
  • Right heart catheterization confirmed diagnoses; survival data were collected over 5 years, with all-cause death or censoring as endpoints.

Main Results:

  • Systemic lupus erythematosus (49%) was the most common underlying CTD, unlike in Western cohorts where systemic sclerosis is more prevalent.
  • 1- and 3-year survival rates were 92% and 80%, respectively.
  • Pericardial effusion, reduced 6-minute walk distance, lower mixed venous oxygen saturation, higher pulmonary vascular resistance (PVR), higher alkaline phosphatase (ALP), and lower total cholesterol were associated with increased mortality risk.

Conclusions:

  • Systemic lupus erythematosus is the predominant CTD in Chinese patients with CTD-APAH.
  • Survival rates for Chinese CTD-APAH patients in the modern era are comparable to those in Western countries.
  • Elevated PVR and ALP are independent predictors of mortality, highlighting them as critical therapeutic targets.

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