It is well known that screening for pulmonary arterial hypertension (PAH) in patients with connective tissue diseases (CTDs), in particular systemic sclerosis (SSc), is key to early diagnosis. As part of an initiative to update guidelines for the screening and diagnosis of CTD-associated PAH for the 2018 World Symposium on Pulmonary Hypertension, a systemic review was conducted to identify the best evidence available on the subject, with results published in Seminars in Arthritis & Rheumatism

The investigators selected manuscripts published between July 2012 and October 2017 that incorporated screening measures used to identify patients with PAH by right heart catheterization. Risk for bias in the studies was evaluated using the Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2) tool.

The systemic review generated 1514 unique citations, with a total of 22 manuscripts selected for final review. Of the manuscripts chosen for review, the majority had a lower risk for bias according to the QUADAS-2 tool. Overall, 16 SSc cohort studies and 6 case-control studies (4 involving patients with SSc and 2 involving patients with systemic lupus erythematosus) were selected.

Of the 16 SSc studies evaluated, 4 explored the use of transthoracic echocardiography (TTE) only. Moreover, 8 of the SSc cohort studies assessed composite measures, including the Australian Scleroderma Interest Group (ASIG) algorithm, the DETECT algorithm, and a combination of tricuspid regurgitation velocity (TRV) and pulmonary function test (PFT) variables.

DETECT and ASIG had greater sensitivity and negative predictive value compared with the 2009 European Society of Cardiology/European Respiratory Society guidelines in different cohorts. The addition of pulmonary function test variables, including diffusing capacity for carbon monoxide (DLCO) and forced vital capacity/DLCO ratio to TRV was associated with greater sensitivity and negative predictive value compared with the use of TRV alone.

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Data continue to support the use of TTE and provide further evidence for the use of composite measures in patients with CTDs. Future research is warranted to further assess these novel screening algorithms and cardiopulmonary changes during exercise in more longitudinal cohorts of patients. Additional longitudinal observational studies are needed to develop and validate screening algorithms for PAH in patients with non-SSc CTDs.

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Young A, Nagaraja V, Basilious M, et al. Update of screening and diagnostic modalities for connective tissue disease-associated pulmonary arterial hypertension [published online October 14, 2018].  Semin Arthritis Rheum.