Abstract
BackgroundPulmonary hypertension (PH) can develop in connective tissue disease associated interstitial lung disease (CTD-ILD), and contributes to increased morbidity and mortality. However, except for systemic sclerosis and mixed connective tissue disease, the impact of mean pulmonary arterial pressure (MPAP) on survival in CTD-ILD has not been sufficiently elucidated. We hypothesized that pulmonary arterial pressure may be a prognostic factor in CTD-ILDs regardless of the kind of CTD.MethodsWe evaluated the survival impact of MPAP, which is measured using right heart catheterization, on survival of patients with CTD-ILD with various CTD backgrounds. We retrospectively analyzed data of consecutive CTD-ILD patients undergoing a pulmonary function test and right-heart-catheterization at the initial evaluation.ResultsWe studied 74 patients (33 men and 41 women, mean age 62.8 ± 9.6, 24 with rheumatoid arthritis, 14 with systemic sclerosis, 14 with polymyositis/dermatomyositis, 11 with primary Sjögren’s syndrome, and 11 with other diagnoses). Six patients exhibited pulmonary hypertension (MPAP ≥ 25 mmHg), and 16 (21.6 %) had mild elevation of MPAP (≥20 mmHg). The mean MPAP was 17.2 ± 5.5 mmHg. We did not observe a significant difference in MPAP among various CTDs. A univariate Cox proportional hazard model showed that MPAP has a significant impact on survival, while the type of CTD did not contribute to survival in our cohort. A multivariate Cox proportional hazard model showed MPAP (HR = 1.087; 95 % CI 1.008–1.172; p = 0.030) to be the sole independent determinant of survival.ConclusionsMild elevation of MPAP is relatively common in CTD-ILD patients with various CTD backgrounds. A higher MPAP at the initial evaluation was a significant independent predictor of survival in CTD-ILD. MPAP evaluation provides additional information on disease status and will help physicians predict mortality in CTD-ILD.
Highlights
Pulmonary hypertension (PH) can develop in connective tissue disease associated interstitial lung disease (CTD-ILD), and contributes to increased morbidity and mortality
The aim of this study was to determine whether mean pulmonary arterial pressure (MPAP) predicts survival in CTD-ILD patients whose disease background, pulmonary function test, and right heart catheterization (RHC) data are available
Twenty-eight patients were excluded from the study for the following reasons: (1) they had been treated with PH targeted therapy, (2) evaluation was done using supplemental oxygen, (3) they were suffering from an unstable disease, such as acute exacerbation, infection, or heart failure, or (4) RHC was not performed within 3 months of initial evaluation or RHC data was missing
Summary
Pulmonary hypertension (PH) can develop in connective tissue disease associated interstitial lung disease (CTD-ILD), and contributes to increased morbidity and mortality. Except for systemic sclerosis and mixed connective tissue disease, the impact of mean pulmonary arterial pressure (MPAP) on survival in CTD-ILD has not been sufficiently elucidated. PH in patients with systemic sclerosis (SSc) and ILD (SSc-ILD) further worsens survival, but there are few data regarding outcomes in SSc-ILD patients with PH compared with those with the other connective tissue disease (CTD) associated ILD [4, 5]. The importance of mild elevation in pulmonary arterial pressure has been recognized in patients with several ILDs. It has been demonstrated that a mean pulmonary arterial pressure (MPAP) between 21 and 24 mmHg is clinically relevant and affects patient outcome in idiopathic pulmonary fibrosis (IPF) [3, 6]. We elucidated that MPAP ≥ 20 mmHg affects patient outcome in LDCTD [9]
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