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. 2020 Mar;79(3):370-378.
doi: 10.1136/annrheumdis-2019-216476. Epub 2019 Dec 9.

Haemodynamic phenotypes and survival in patients with systemic sclerosis: the impact of the new definition of pulmonary arterial hypertension

Panagiota Xanthouli  1   2 , Suzana Jordan  3 , Nicklas Milde  1   2 , Alberto Marra  4 , Norbert Blank  5 , Benjamin Egenlauf  1   2 , Matthias Gorenflo  6 , Satenik Harutyunova  1   2 , Hanns-Martin Lorenz  5 , Christian Nagel  1   2   7 , Vivienne Theobald  1   2 , Mona Lichtblau  8 , Charlotte Berlier  8 , Silvia Ulrich  8 , Ekkehard Grünig  1   2 , Nicola Benjamin  9   2 , Oliver Distler  3
Affiliations

Haemodynamic phenotypes and survival in patients with systemic sclerosis: the impact of the new definition of pulmonary arterial hypertension

Panagiota Xanthouli et al. Ann Rheum Dis. 2020 Mar.

Abstract

Background: In this study, we investigated the impact of the new haemodynamic definition of pulmonary arterial hypertension (PAH) as proposed by the 6th PH World Symposium on phenotypes and survival in patients with systemic sclerosis (SSc).

Methods: In SSc patients who were prospectively and consecutively screened for PAH including right heart catheterisation in Heidelberg or Zurich, haemodynamic and clinical variables have been reassessed according to the new PAH definition. Patients have been followed for 3.7±3.7 (median 3.4) years; Kaplan-Meier survival analysis was performed. Patients with significant lung or left heart disease were excluded from comparative analyses.

Results: The final dataset included 284 SSc patients, 146 patients (49.2%) had mean pulmonary arterial pressure (mPAP) ≤20 mm Hg, 19.3% had mPAP 21-24 mm Hg and 29.4% had mPAP ≥25 mm Hg. In the group of mildly elevated mPAP, only four patients (1.4% of the whole SSc cohort) had pulmonary vascular resistance (PVR) values ≥3 Wood Units (WU) and could be reclassified as manifest SSc-APAH. Twenty-eight (9.8%) patients with mPAP of 21-24 mm Hg and PVR ≥2 WU already presented with early pulmonary vascular disease with decreased 6 min walking distance (6MWD) (p<0.001), TAPSE (p=0.004) and pulmonary arterial compliance (p<0.001). A PVR ≥2 WU was associated with reduced long-term survival (p=0.002). PVR and 6MWD were independent prognostic predictors in multivariate analysis.

Conclusion: The data of this study show that a PVR threshold ≥3 WU is too high to enable an early diagnosis of PAH. A PVR threshold ≥2 WU was already associated with pulmonary vascular disease, significantly reduced survival and would be more appropriate in SSc patients with mild PAH.

Keywords: outcomes research; systemic sclerosis; treatment.

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Conflict of interest statement

Competing interests: PX has received personal fees from MSD and OMT. AM has received personal fees from Bayer, outside the submitted work. NBlank has received consulting fees, speaker fees and/or honoraria from MSD, GSK, Actelion and Bayer Vital. BE received travel fees, consulting fees, speaking fees and/or honoraria from Actelion, MSD, Bayer and OMT (less than $10 000 each). MG serves on an advisory Board for Bayer AG and receives lecture fees from Pfizer, MSD and Actelion Pharmaceuticals. SH has received personal fees from Bayer, MSD, Actelion and GSK, outside the submitted work. H-ML has received consulting fees, speaking fees and/or honoraria from AbbVie, BMS, Pfizer, Cellgene, Medac, GSK, Roche, Chugai, Novartis, UCB, Janssen-Cilag, AstraZeneca and Lilly (less than $10 000 each) and research support from AbbVie, MSD, BMS, Cellgene, Medac, GSK, Roche, Chugai, Novartis, UCB, Janssen-Cilag, AstraZeneca, Lilly, Baxter, SOBI, Biogen, Actelion, Bayer Vital, Shire, Octapharm, Sanofi, Hexal, Mundipharm and Thermo Fisher. CN has received consulting fees, speaking fees and/or honoraria from Actelion, MSD, Boehringer, Novartis, Bayer and AstraZeneca (less than $10 000 each). CB received travel support from Actelion SA and Orpha Swiss and speakers’ fee from MSD. SU received grants from the Swiss National Science foundation and the Zurich Lung League, travel support and speakers’ fees from Actelion SA, Switzerland, Bayer SA, Germany, MSD, Switzerland and Orpha Swiss. EG has received grants and personal fees from Actelion, Bayer AG and MSD; grants from GSK, Novartis, and United Therapeutics; and personal fees from SCOPE, OrPha Swiss GmbH, and Zurich Heart House (less than $10 000 each). NBenjamin received speaker fees from Actelion pharmaceuticals, Bayer HealthCare and MSD. OD has received grants and personal fees from research consultancies from AM, Acceleron Pharma, Amgen, AnaMar, Bayer, Beacon Discovery, Boehringer Ingelheim, Catenion, CSL Behring, ChemomAb, Ergonex, GSK, Inventiva, Italfarmaco, iQone, iQvia, Lilly, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Sanofi, Blade Therapeutics, Glenmark Pharmaceuticals, Target Bio Science and UCB, outside the submitted work, to investigate potential treatments of scleroderma and its complications. In addition, OD has a patent mir-29 for the treatment of systemic sclerosis issued (US8247389, EP2331143).

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