Mean pulmonary arterial pressure as a prognostic indicator in connective tissue disease associated with interstitial lung disease: a retrospective cohort study

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.

Mild Pulmonary Hypertension Is Associated With Increased Mortality: A Systematic Review and Meta‐Analysis

“…Forty full‐text articles were assessed for eligibility (Figure 1). Fifteen (8 RHC and 7 echocardiography) studies were included in the meta‐analysis 2, 3, 4, 5, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20. The characteristics of the included studies are shown in Table 2.…”

“…Studies included in this analysis are Valerio et al,10 Heresi et al,11 Kovacs et al,5 Suzuki et al,12 Maron et al,3 Takahashi et al,13 Douschan et al,4 Assad et al,2 Abramson et al,14 Kjaergaard et al,15 Shalaby et al,16 Lam et al,17 Damy et al,18 Cabrita et al,19 and Choudhary et al 20. CI indicates confidence interval; M‐H, Mantel‐Haenszel; RHC, right heart catheterization.…”

“…Studies included in this analysis are Heresi et al,11 Suzuki et al,12 Maron et al,3 Takahashi et al,13 Assad et al,2 Shalaby et al,16 Cabrita et al,19 and Choudhary et al 20. CI indicates confidence interval; PH, pulmonary hypertension; IV, Inverse Variance; RHC, right heart catheterization.…”

CT‐determined pulmonary artery to aorta ratio as a predictor of elevated pulmonary artery pressure and survival in idiopathic pulmonary fibrosis

“…Elevated mean pulmonary artery pressure (mPAP) is associated with a worse survival in a range of respiratory diseases including idiopathic pulmonary fibrosis (IPF) . The presence of pulmonary hypertension (PH), defined by mPAP ≥ 25 mm Hg at rest, is associated with a worse prognosis in IPF …”

Pulmonary Hypertension in Diffuse Parenchymal Lung Diseases—Is There Any Benefit of PAH-Specific Therapy?

“…Baughman et al [8] observed the worst survival in cases of PH due to lung disease, and much better prognosis in subjects with PH due to left heart failure, in the group of sarcoidosis patients. Takahashi et al [25] found mPAP to be a significant independent survival predictor in DPLD associated with CTD. Data obtained from PH registries indicate that prognosis of the patients with severe PH-DPLD is extremely poor, with only 20-30% of patients surviving 3 years [26,27].…”