7 research outputs found
Clinical phenotypes and survival of pre-capillary pulmonary hypertension in systemic sclerosis
<div><p>Pre-capillary pulmonary hypertension (PH) in systemic sclerosis (SSc) is a heterogeneous condition with an overall bad prognosis. The objective of this study was to identify and characterize homogeneous phenotypes by a cluster analysis in SSc patients with PH. Patients were identified from two prospective cohorts from the US and France. Clinical, pulmonary function, high-resolution chest tomography, hemodynamic and survival data were extracted. We performed cluster analysis using the k-means method and compared survival between clusters using Cox regression analysis. Cluster analysis of 200 patients identified four homogenous phenotypes. Cluster C1 included patients with mild to moderate risk pulmonary arterial hypertension (PAH) with limited or no interstitial lung disease (ILD) and low DLCO with a 3-year survival of 81.5% (95% CI: 71.4–88.2). C2 had pre-capillary PH due to extensive ILD and worse 3-year survival compared to C1 (adjusted hazard ratio [HR] 3.14; 95% CI 1.66–5.94; p = 0.0004). C3 had severe PAH and a trend towards worse survival (HR 2.53; 95% CI 0.99–6.49; p = 0.052). Cluster C4 and C1 were similar with no difference in survival (HR 0.65; 95% CI 0.19–2.27, p = 0.507) but with a higher DLCO in C4. PH in SSc can be characterized into distinct clusters that differ in prognosis.</p></div
Survival of the four clusters C1, C2, C3 and C4.
<p>The difference of survival between the 4 clusters was significant (p = 0.0002).</p
Distribution of patients with no, limited and extensive interstitial lung disease according to mean pulmonary artery pressure.
<p>Distribution of patients with no, limited and extensive interstitial lung disease according to mean pulmonary artery pressure.</p
Clinical phenotypes and survival of pre-capillary pulmonary hypertension in systemic sclerosis - Fig 3
<p>A. Radar plot of the four clusters according to clinical characteristics, presence and severity of interstitial lung disease and severity of hemodynamics. B. Algorithm of classification in the four clusters C1, C2, C3 and C4.</p
Baseline characteristics of the four clusters of systemic sclerosis patients with pre-capillary pulmonary hypertension.
<p>Baseline characteristics of the four clusters of systemic sclerosis patients with pre-capillary pulmonary hypertension.</p