Significance of pulmonary arterial pressure as a prognostic indicator in lung-dominant connective tissue disease

PLoS One. 2014 Sep 30;9(9):e108339. doi: 10.1371/journal.pone.0108339. eCollection 2014.

Abstract

Background: Lung-dominant connective tissue disease (LD-CTD) is a new concept for classifying the subset of patients with interstitial pneumonia who have clinical features suggesting an associated CTD, but whose features fall short of a clear diagnosis of CTD under the current rheumatologic classification systems. The impact of mean pulmonary arterial pressure (MPAP) in LD-CTD has not been sufficiently elucidated.

Objectives: To evaluate the survival impact of MPAP measured during the initial evaluation in patients with LD-CTD.

Methods: We retrospectively analyzed the initial evaluation data of 100 LD-CTD patients undergoing pulmonary function test, 6-min walk test (6MWT), and right heart catheterization (RHC).

Results: The mean MPAP was 16.2±4.4 mm Hg, and 18 patients had MPAP≥20 mm Hg. A univariate Cox proportional hazard model showed that MPAP and several variables have a statistically significant impact on survival. With stepwise, multivariate Cox proportional analysis, MPAP (HR = 1.293; 95% CI 1.130-1.480; p<0.001) and mean forced vital capacity (FVC) % predicted (HR = 0.958; 95% CI 0.930-0.986; p = 0.004) were shown to be independent determinants of survival.

Conclusions: Higher MPAP and lower %FVC at the initial evaluation were significant independent prognostic factors of LD-CTD. MPAP evaluation provides additional information of disease status and will help physicians to predict mortality in LD-CTD.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Arterial Pressure
  • Cardiac Catheterization
  • Connective Tissue Diseases / diagnosis*
  • Connective Tissue Diseases / mortality
  • Connective Tissue Diseases / physiopathology
  • Female
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / mortality
  • Hypertension, Pulmonary / physiopathology
  • Lung / physiopathology*
  • Lung Diseases, Interstitial / diagnosis*
  • Lung Diseases, Interstitial / mortality
  • Lung Diseases, Interstitial / physiopathology
  • Male
  • Middle Aged
  • Prognosis
  • Proportional Hazards Models
  • Pulmonary Artery / physiopathology*
  • Retrospective Studies
  • Survival Analysis
  • Vital Capacity
  • Walking

Grants and funding

This work was supported in part by a grant-in-aid for the respiratory failure research group from the Japanese Ministry of Health, Labor and Welfare and NPO Respiratory Disease Conference. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.