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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying

Lorinda Chung1, Juliana Liu, Lori Parsons

  • 1VA Palo Alto Health Care System, Palo Alto, CA 94304, USA. shauwei@stanford.edu

Chest
|May 29, 2010
PubMed

Insights

Connective tissue disease-associated pulmonary arterial hypertension (CTD-APAH) patients have poorer survival and hospitalization outcomes compared to idiopathic PAH. Systemic sclerosis-associated PAH (SSc-APAH) shows the worst prognosis among CTD-APAH subgroups.

Area of Science:

  • Cardiology
  • Pulmonology
  • Rheumatology

Background:

  • Pulmonary arterial hypertension (PAH) is a severe condition affecting lung arteries.
  • Connective tissue diseases (CTDs) are a common cause of PAH.
  • The REVEAL registry provides extensive data on PAH patients in the US.

Purpose of the Study:

  • To analyze the clinical features and outcomes of patients with CTD-associated PAH (CTD-APAH).
  • To compare CTD-APAH patients with idiopathic PAH.
  • To differentiate outcomes among various CTD-APAH subgroups, particularly SSc-APAH.

Main Methods:

  • Utilized the REVEAL US registry data, including patients confirmed by right-sided heart catheterization (RHC).
  • Compared clinical characteristics, hemodynamics, and echocardiographic findings.
  • Analyzed 1-year mortality and hospitalization rates for CTD-APAH versus idiopathic PAH and compared CTD subgroups (SSc, SLE, MCTD, RA).

Main Results:

  • CTD-APAH patients (n=641) had lower 6-min walk distance, higher BNP, and lower Dlco compared to idiopathic PAH (n=1,251).
  • CTD-APAH group showed lower 1-year survival (86%) and freedom from hospitalization (67%) versus idiopathic PAH (93% and 73%, respectively).
  • Systemic sclerosis-associated PAH (SSc-APAH) patients had the highest BNP, lowest Dlco, and poorest 1-year survival (82%) among CTD-APAH subgroups.

Conclusions:

  • Patients with SSc-APAH exhibit a distinct and severe phenotype.
  • SSc-APAH is associated with the highest B-type natriuretic peptide levels, lowest diffusing capacity for carbon monoxide, and worst survival outcomes within the CTD-APAH population.
Abstract

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