Clinical phenotypes and survival of pre-capillary pulmonary hypertension in systemic sclerosis

PLoS One. 2018 May 15;13(5):e0197112. doi: 10.1371/journal.pone.0197112. eCollection 2018.

Abstract

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.

Publication types

  • Clinical Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Hemodynamics*
  • Humans
  • Hypertension, Pulmonary* / diagnostic imaging
  • Hypertension, Pulmonary* / etiology
  • Hypertension, Pulmonary* / mortality
  • Hypertension, Pulmonary* / physiopathology
  • Male
  • Middle Aged
  • Phenotype*
  • Retrospective Studies
  • Risk Factors
  • Scleroderma, Systemic* / complications
  • Scleroderma, Systemic* / diagnostic imaging
  • Scleroderma, Systemic* / mortality
  • Scleroderma, Systemic* / physiopathology
  • Survival Rate

Grants and funding

This work was funded in part by a restricted grant of Pfizer (WI176902 to Eric Hachulla). The funder had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.