DETECT - Early, simple and reliable DETECTion of PAH in Systemic Sclerosis
Systemic sclerosis (SSc) is a chronic autoimmune connective tissue disease characterized by excessive collagen deposition in the skin and internal organs, such as the gastrointestinal tract, kidney, heart, and also the lungs. Symptoms result from vascular dysfunction, inflammation, and progressive fibrosis, which, lead to occlusion of the microvasculature resulting in pulmonary vascular injury.
A substantial proportion (12–27%) of SSc patients develop PAH (PAH-SSc), which is the leading cause of mortality in this condition. Early detection of PAH-SSc is considered essential to allow for optimal patient management. However, PAH-SSc is typically diagnosed late. Current screening recommendations are consensus-based rather than evidenced-based and rely on echocardiography and symptoms only. Furthermore, the rate of missed PAH diagnoses has never been determined.
The prospective DETECT study aimed to develop the first comprehensive evidence-based tool for the detection of PAH-SSc that would limit the number of missed PAH diagnoses. The study was designed by an independent international panel of rheumatology and cardiology experts, in collaboration with epidemiologists and statisticians.
Consenting patients with confirmed SSc and an increased risk of having undiagnosed PAH (on the basis of duration of SSc and pulmonary function tests) were enrolled in 62 participating centers from 18 countries. The accuracy of multiple variables with the potential to reveal PAH-SSc (clinical, serum markers, ECG, and echocardiography variables) was analyzed versus right heart catheterization (RHC), the confirmatory gold-standard diagnostic test for PAH. Univariable and multivariable analyses selected the best discriminatory variables for detecting PAH. These variables were assessed for clinical plausibility and feasibility and incorporated into a detection algorithm. Electronic and paper-based tools for practical application of the detection algorithm are being developed.
Key study results have been presented at the American College of Chest Physicians (CHEST) and the American College of Rheumatology (ACR) meetings 2012. Full data were published in 2013: Coglan JG, Denton CP Grünig E, et al. (2013). Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. AnnRheumdisdoi:10.1136/annrheumdis-2013-203301