Welcome to ReachMD. This medical industry feature, titled “Exploring a Screening Tool to Assess PAH Risk in SSc Patients” is an educational activity brought to you by the Janssen Pharmaceutical Companies of Johnson & Johnson. This program is intended for US healthcare professionals only and is not certified for continuing medical education.
Pulmonary arterial hypertension, or PAH, is a rare and progressive disease where the walls of the pulmonary arteries become thick and stiff, leading to elevated pressure in the lungs.1,2
PAH associated with connective tissue disease, or CTD, is the second most prevalent type of PAH, after idiopathic PAH, in the US and Europe.3 Of the CTDs, PAH occurs most commonly in patients with systemic sclerosis, or SSc.4,5 Patients with SSc can develop PAH at any time and may experience a median delay in diagnosis of four years, so it’s important to screen patients regularly and diagnose as early as possible.6,7
The DETECT Screening Tool app can help healthcare professionals assess the risk of PAH in patients with SSc, as well as optimize the use of right heart catheterization, or RHC.8
The DETECT Screening Tool for PAH-SSc was funded and developed by Actelion Pharmaceuticals Ltd, a Janssen Pharmaceutical Company of Johnson & Johnson. It is limited for use by healthcare specialists who treat patients with SSc, and who are familiar with PAH as a complication of SSc. It is not intended to replace the medical or professional judgment of healthcare providers. It does not provide professional advice and should not be considered a diagnosis. Healthcare professionals using the tool should exercise clinical judgment as to the information they provide to patients based upon the tool.
The noninvasive DETECT Screening Tool uses a two-step process. Step 1 helps calculate an SSc patient’s risk of developing PAH and assesses if they should be screened for PAH with an echocardiogram. Step 2 helps physicians determine the need for right heart catheterization to confirm the patient’s diagnosis.9
In order to determine a patient’s risk score using the DETECT Screening Tool, a physician needs to enter results for specified parameters following their evaluation of the patient. These include results from pulmonary function tests, the presence of telangiectasias, specified lab values, and results from an electrocardiogram.9
Step 2 uses the total risk score from Step 1, as well as the results from an echocardiogram, to determine whether the patient should be referred for a right heart catheterization. Right atrium area, an echocardiographic parameter, is routinely measured in patients with PAH. Tricuspid regurgitation velocity may also be reported with echocardiogram.9 If the tricuspid regurgitation velocity is immeasurable, the DETECT Screening Tool will automatically impute it based on the data pool from the DETECT study.
The DETECT Screening Tool app can help healthcare professionals identify patients with systemic sclerosis who should be evaluated for PAH using echocardiography and who may need an RHC for confirmation of PAH-SSc.8
To learn more and download the app, visit www.suspectpahctd.com/DETECT.
This program was brought to you by the Janssen Pharmaceutical Companies of Johnson & Johnson.
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- Lai YC, Potoka KC, Champion HC, et al. Pulmonary arterial hypertension: the clinical syndrome. Circ Res. 2014;115(1):115-130.
- Sun W, Chan SY. Pulmonary arterial stiffness: an early and pervasive driver of pulmonary arterial hypertension. Front Med. 2018;5:204.
- Galiè N, Humbert M, Vachiery J, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016; 37:67-119.
- Zanatta E, Polito P, Famoso G, et al. Pulmonary arterial hypertension in connective tissue disorders: Pathophysiology and treatment. Exp Biol Med. 2019;244(2):120-131.
- Mathai SC, Hassoun PM. Pulmonary arterial hypertension in connective tissue diseases. Heart Fail Clin. 2012;8(3):413-425.
- Hachulla E, Launay D, Mouthon L, et al. Is pulmonary arterial hypertension really a late complication of systemic sclerosis?. CHEST. 2009;136(5):1211-1219.
- Vachiéry J, Gaine S. Challenges in the diagnosis and treatment of pulmonary arterial Hypertension. Eur Respir Rev. 2012; 21:313-20.
- Mandras SA, Ventura HO, Corris PA. Breaking Down the Barriers: Why the Delay In Referral For Pulmonary Arterial Hypertension? Ochsner J. 2016;16(3):257-262.
- Coghlan JG, Denton CP, Grünig E, et al. DETECT study group. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis. 2014;73(7):1340-1349.