Abstract

BackgroundLung-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.ObjectivesTo evaluate the survival impact of MPAP measured during the initial evaluation in patients with LD-CTD.MethodsWe 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).ResultsThe 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.ConclusionsHigher 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.

Highlights

  • Pulmonary hypertension (PH) has many causes and is a source of significant mortality in affected individuals

  • Some studies suggest that connective tissue disease (CTD)-associated PH is a prognostic factor, seen especially in systemic scleroderma (SSc) and mixed connective tissue disease (MCTD) [6,7,8,9]

  • The aim of this study was to evaluate whether mean pulmonary arterial pressure (MPAP) predicts survival in LDCTD patients in whom background, pulmonary function test, 6min walk test (6MWT), and right heart catheterization (RHC) could be evaluated at the initial evaluation

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Summary

Introduction

Pulmonary hypertension (PH) has many causes and is a source of significant mortality in affected individuals. Mean pulmonary arterial pressure (MPAP) .20 mmHg) may be clinically relevant in both ILD and CTD [2,10]. Fischer et al [11] proposed ‘‘lung-dominant CTD’’ (LD-CTD) as 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. 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

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