Screening for PAH-CTD

Minimally Invasive Tests

A variety of tests are available to screen for possible pulmonary arterial hypertension associated with systemic sclerosis (PAH-SSc), to be followed by confirmatory right heart catheterization (RHC). They can be used at initial evaluation of SSc, regardless of PAH risk factors, and regularly to help identify the potential of associated PAH and trigger additional diagnostic tests before symptoms present.1-3

Pulmonary Function Tests (PFTs) FVC/Dlco ratio >1.6 and/or Dlco <60% of predicted may indicate a patient is at risk for PAH-SSc
Echocardiogram with focus on the right heart may be used in patients with suspected PAH-SSc
NT-proBNP Elevated biomarker may indicate myocardial dysfunction
Keep an eye out for symptoms, clinical findings, and risk factors that can aid in the diagnosis of PAH‑SSc. Learn How to Recognize Them >

PAH-SSc Screening Recommendations

Annual screening for PAH in patients with SSc is recommended:

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2015 ESC/ERS RECOMMENDATIONS2: Screening with resting echocardiography is recommended in asymptomatic patients, followed by annual screening with echocardiography, Dlco, and biomarkers

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CONSENSUS-BASED RECOMMENDATION FOR SCREENING AND DETECTION OF PAH-CTD1*: Every patient with SSc should be screened annually for PAH with noninvasive transthoracic echocardiogram (TTE) and PFTs due to the high prevalence of PAH in SSc

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6TH WORLD SYMPOSIUM ON PULMONARY HYPERTENSION RECOMMENDATIONS3: Annual screening for PAH using PFTs and echocardiography should be considered in patients with the SSc spectrum of diseases with uncorrected Dlco <80% of predicted

There may be racial differences in PAH-CTD: Among patients with PAH in the National Biological Sample and Data Repository for PAH (PAH Biobank), African Americans were 2.5 times more likely to have PAH-CTD than patients of other races.4 Watch for Differences in PAH-SSc Clinical Presentation and Severity >

Confirming PAH-CTD Diagnosis

RHC, also called pulmonary artery catheterization, is the gold standard test to diagnose PAH. It measures the blood pressure in the heart and pulmonary arteries, and is recommended in cases of suspected PAH-CTD to confirm diagnosis, determine severity, and rule out left-side heart disease.2,5

Improved Outcomes

Regular screening and early diagnosis may improve PAH-SSc patient outcomes: Retrospective analysis from the French PAH Registry and the ItinérAIR-Sclérodermie program showed higher survival rates in patients who were diagnosed with PAH-SSc with proactive screening in the detection cohort.6‡

Regular screening AND EARLY DIAGNOSIS may improve patient outcomes
Regular screening timeline chart for PAH-CTD Regular screening timeline chart for PAH-CTD
Reprinted from Arthritis & Rheumatology, Vol 63, Humbert M, et al, Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival, with permission from John Wiley and Sons.


Play the video below to understand the importance of regularly screening patients with SSc for PAH.

*RAND/UCLA methodology was used to develop case scenarios informed by a systematic literature review; consensus recommendations for the screening and detection of PAH-CTD were made after 2 rounds of anonymous voting and a face-to-face meeting by international experts from a variety of specialties. The task force panel recommendation for annual screenings using TTE and PFTs has a low quality of evidence on all patients with SSc and a very low quality of evidence on patients with SSc spectrum disorders.1
For patients with SSc and SSc spectrum disorders with uncorrected Dlco <80% of predicted, annual screening should be considered. The appropriate screening tools include DETECT, the 2015 ESC/ERS recommendations for TTE or FVC/Dlco ratio >1.6 (assuming none-to-mild interstitial lung disease), and >2-fold upper limit of normal of NT-proBNP. For those with uncorrected Dlco ≥80% of predicted, screening may be considered with TTE.3
Refers to survival rates at 1, 3, 5, and 8 years. Proactive screening cohort comprised consecutive patients with SSc who entered a systematic PAH detection program and were subsequently found to have PAH on RHC. Routine practice cohort comprised consecutive adult patients with symptomatic SSc diagnosed with PAH by RHC at the time of recruitment into the French PAH Registry.6
Dlco=diffusing capacity of the lungs for carbon monoxide; ERS=European Respiratory Society; ESC=European Society of Cardiology; FVC=forced vital capacity; HR=hazard ratio; NT-proBNP=N-terminal pro-brain natriuretic peptide; PAH-CTD=pulmonary arterial hypertension associated with connective tissue disease; SSc=systemic sclerosis; UCLA=University of California, Los Angeles.
References: 1. Khanna D, Gladue H, Channick R, et al. Recommendations for screening and detection of connective-tissue disease associated pulmonary arterial hypertension. Arthritis Rheum. 2013;65(12):3194-3201. 2. Galiè N, Humbert M, Vachiéry J-L, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2015;46(4):903-975. 3. Frost A, Badesch D, Gibbs JSR, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019;53(1):1801904. 4. Al-Naamani N, Paulus JK, Roberts KE, et al. Racial and ethnic differences in pulmonary arterial hypertension. Pulm Circ. 2017;7(4):793-796. 5. Fischer A, Bull TM, Steen VD. Practical approach to screening for scleroderma-associated pulmonary arterial hypertension. Arthritis Care Res (Hoboken). 2012;64(3):303-310. 6. Humbert M, Yaici A, de Groote P, et al. Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum. 2011;63(11):3522-3530.