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. 2019 Jul 31;28(153):190023.
doi: 10.1183/16000617.0023-2019. Print 2019 Sep 30.

Screening for pulmonary arterial hypertension in systemic sclerosis

Jason Weatherald  1   2 , David Montani  3   4   5 , Mitja Jevnikar  3   4   5 , Xavier Jaïs  3   4   5 , Laurent Savale  3   4   5 , Marc Humbert  6   4   5
Affiliations

Screening for pulmonary arterial hypertension in systemic sclerosis

Jason Weatherald et al. Eur Respir Rev. .

Abstract

Pulmonary arterial hypertension (PAH) is a dreaded complication of systemic sclerosis (SSc) that occurs in ∼10% of patients. Most individuals present with severe symptoms, significant functional impairment and severe haemodynamics at diagnosis, and survival after PAH diagnosis is poor. Therefore, early diagnosis through systematic screening of asymptomatic patients has the potential to identify PAH at an early stage. Current evidence suggests that early diagnosis and treatment of PAH in patients with SSc may lead to better clinical outcomes. Annual screening may include echocardiography, but this can miss some patients due to suboptimal visualisation or insufficient tricuspid regurgitation. Other options for screening include the DETECT algorithm or the use of a combination of pulmonary function testing (forced vital capacity/diffusing capacity of the lung for carbon monoxide ratio) and N-terminal-pro-brain natriuretic peptide levels. Symptomatic patients, those with an elevated tricuspid regurgitation velocity on echocardiogram with or without secondary echocardiographic features of PAH, and those who screen positive on the DETECT or other pulmonary function test algorithms should undergo right heart catheterisation. Exercise echocardiography or cardiopulmonary exercise testing, nailfold capillaroscopy and molecular biomarkers are promising but, as yet, unproven potential options. Future screening studies should employ systematic catheterisation to define the true predictive values for PAH.

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Conflict of interest statement

Conflict of interest: J. Weatherald reports grants, personal fees and non-financial support from Actelion, personal fees and non-financial support from Bayer, personal fees from Novartis, and grants from the European Respiratory Society and Canadian Vascular Network, outside the submitted work. Conflict of interest: D. Montani reports grants and personal fees from Actelion and Bayer, and personal fees from GSK, MSD and Pfizer, outside the submitted work. Conflict of interest: M. Jevnikar has nothing to disclose. Conflict of interest: X. Jais reports grants, personal fees and non-financial support from Actelion and Bayer, personal fees and non-financial support from MSD, and grants and non-financial support from GSK, outside the submitted work. Conflict of interest: L. Savale reports grants, personal fees and non-financial support from Actelion, Bayer, MSD and GSK, outside the submitted work. Conflict of interest: M. Humbert reports personal fees and non-financial support from Actelion, grants, personal fees and non-financial support from Bayer, grants and personal fees from GSK, and personal fees from Merck and United Therapeutics, outside the submitted work.

Figures

FIGURE 1
FIGURE 1
Epidemiologic concepts for pulmonary arterial hypertension (PAH) screening programmes. a) Lead-time bias. b) Overdiagnosis.
FIGURE 2
FIGURE 2
Comparison of screening algorithms for pulmonary arterial hypertension (PAH) in systemic sclerosis. a) The DETECT algorithm and b) the Australian Scleroderma Interest Group (ASIG) algorithm. FVC: forced vital capacity; DLCO: diffusing capacity of the lung for carbon monoxide; NT-proBNP: N-terminal pro-brain natriuretic peptide; TRV: tricuspid regurgitation velocity; RHC: right heart catheterisation; PFT: pulmonary function test; HRCT: high-resolution computed tomography; V/Q: ventilation perfusion scan.
See this image and copyright information in PMC

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