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

PloS One
David LaunayMarc Humbert

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 b...Continue Reading

References

Oct 2, 2004·The New England Journal of Medicine·Marc HumbertGérald Simonneau
Sep 2, 2006·Arthritis and Rheumatism·Micah R FisherPaul M Hassoun
Mar 3, 2007·Annals of the Rheumatic Diseases·Virginia D Steen, Thomas A Medsger
Mar 29, 2008·American Journal of Respiratory and Critical Care Medicine·Nicole S L GohAthol U Wells
Jan 12, 2010·Clinical Reviews in Allergy & Immunology·Jérôme Le PavecMarc Humbert
Feb 10, 2010·Clinical Reviews in Allergy & Immunology·Eric Hachulla, David Launay
Mar 3, 2010·American Journal of Respiratory and Critical Care Medicine·Jérôme Le PavecPaul M Hassoun
Oct 2, 2013·Chest·Neal F Chaisson, Paul M Hassoun
Oct 8, 2013·PloS One·Elisa DocampoXavier Estivill
Dec 21, 2013·Journal of the American College of Cardiology·Gerald SimonneauRogiero Souza
Sep 29, 2017·European Respiratory Review : an Official Journal of the European Respiratory Society·David LaunayMarc Humbert

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Citations

Sep 14, 2018·The European Respiratory Journal·Jason WeatheraldOlivier Sitbon
Dec 14, 2018·The European Respiratory Journal·Steven D NathanWerner Seeger
Nov 23, 2019·Der Internist·Susanne Schinke, Gabriela Riemekasten
May 16, 2019·Zeitschrift für Rheumatologie·M O BeckerB Maurer
Dec 10, 2019·Current Rheumatology Reports·George HungMonica Mukherjee
Aug 7, 2019·Der Hautarzt; Zeitschrift für Dermatologie, Venerologie, und verwandte Gebiete·M O BeckerB Maurer
Dec 18, 2020·Rheumatology·Christopher W WassonFrancesco Del Galdo
Nov 28, 2020·The Journal of Heart and Lung Transplantation : the Official Publication of the International Society for Heart Transplantation·Roberto BadagliaccaFranz Rischard
Aug 20, 2021·European Respiratory Review : an Official Journal of the European Respiratory Society·Ashraful HaqueRobin Condliffe

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