Background Pulmonary arterial hypertension (PAH) is one of the leading causes of mortality in systemic sclerosis (SSc) and has a 3 year survival rate of approximately 50%. Early screening for PAH in SSc may improve survival.

Objectives To compare the predictive accuracy of two SSc-PAH screening algorithms, DETECT and Australian Scleroderma Interest Group (ASIG), to the 2009 European Society of Cardiology/European Respiratory Society (ESC/ERS) screening recommendations in a well-defined North American cohort.

Methods Consecutive SSc patients with suspected PAH according to the 2013 recommendations for screening PAH (1) undergoing right heart catheterization (RHC) were evaluated. Those patients with no pulmonary hypertension or PAH on RHC who also had clinical variables available for applying the various screening algorithms had DETECT (2), ASIG (3) and 2009 ESC/ERS (4) screening algorithms applied. For each model, contingency table analysis was used to determine sensitivity, specificity, and positive and negative predictive values (PPV and NPV, respectively) for PAH [defined as mean pulmonary artery pressure (mPAP) > or =25, pulmonary capillary wedge pressure (PCWP) < or =15, and no/mild interstitial lung disease (ILD) on high-resolution CT scan of chest (HRCT), or FVC > or =70%].

Results A total of 108 patients were screened for PAH, 77 of those patients met the recommendations for RHC, and 65 of those patients underwent RHC. The prevalence of PAH was 17%. Those patients with RHC values indicating no PH (N=35) or PAH (N=11) who also had biochemical markers available for review were identified and DETECT, ASIG, and ESC/ERS algorithms were applied to all of those patients. Those patients who met inclusion/exclusion criteria for DETECT and ASIG were also separately evaluated. DETECT and ASIG both had similar performances with sensitivity of 100% for those who met the respective inclusion/exclusion criteria. ESC/ERS had a sensitivity of 80% (Table 1).

Conclusions In a well-defined North American cohort, DETECT and ASIG performed similarly and were superior to 2009 ESC/ERS screening recommendations in the detection of PAH in SSc patients. Many patients who are at-risk for PAH did not meet the inclusion/exclusion criteria for DETECT and ASIG in our cohort.

1. Khanna D et al. Arthritis Rheum. 2013 Dec; 65(12):3194–201.

2. Coghlan JG et al. Ann Rheum Dis. 2014 Jul;73(7):1340–9.

3. Thakkar V et al. Arthritis Res Ther. 2012 Jun 12;14(3):R143.

4. Galie N et al. Eur Heart J. 2009 Oct; 30 (20):2493–537

Acknowledgement Supported by NIH/MIAMS K24 AR063120

Disclosure of Interest A. Young: None declared, V. Nagaraja: None declared, S. Visovatti: None declared, V. McLaughlin: None declared, D. Khanna Grant/research support from: Bayer, BMS, Consultant for: Actelion, Gilead, Bayer