Conference: 2010 International PHA Conference and Scientific Sessions
Release Date: 06.24.2010
Presentation Type: Abstracts
McLaughlin V1, Khanna D2, Bonderman D3, Coghlan G4, Denton CP4, Distler O5, Grünig E6, Müller-Ladner U7, Pope J8, Vonk M9, Seibold JR10
1. University of Michigan, Ann Arbor, MI, USA
2. UCLA, Los Angeles, CA, USA
3. Medizinische Universitätsklinik, Wien, Germany
4. Royal Free Hospital, London, UK
5. Department of Rheumatology, University Hospital, Zurich, Switzerland
6. Universitätsklinikum Heidelberg, Germany
7. Kerckhoff-Klinik, Bad Nauheim, Germany
8. St. Joseph's Hospital, London, Ontario, Canada
9. Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
10. University of Connecticut Health Center, Farmington, CT, USA
BACKGROUND: PAH and PH are leading causes of mortality and late stage disease morbidity in systemic sclerosis (SSc) but current screening algorithms lack sensitivity and specificity. The DETECT study was developed to identify and validate an accessible, cost effective, reliable and accurate screening tool (or combination of tools) for the early detection of PAH/PH in SSc patients. The DETECT study aims to determine the discriminatory and predictive ability of single or multiple screening tests (e.g. NTproBNP, ECG, echocardiography, others) for PAH (and more broadly, PH) in SSc by means of comparison with right heart catheterization, the current gold standard diagnostic test; and to estimate the incidence of PAH and PH in the cohort of SSc patients during a 3-year longitudinal follow-up period.
METHODS: This is a two stage prospective observational cohort study of patients with SSc. Screening tests for PAH/PH are evaluated in the cross sectional stage (primary objective) and the incidence of PAH/PH is evaluated in the 3-year longitudinal phase of the study. Right heart catheterization is performed at baseline and the final visit. Main inclusion criteria are: 1) Age ≥ 18 years, 2) SSc as diagnosed by the American College of Rheumatology (ACR) criteria, 3) SSc disease duration > 3 years dated from onset of first non-Raynauds feature, 4) Diffusing capacity of the lung for carbon monoxide (DLCO) < 60% of predicted, 5) Forced Vital Capacity (FVC) ≥ 40%, and 6) No previous PH or PAH diagnosis.
CONCLUSIONS: A reliable and accessible non-invasive screening tool for PAH/PH in SSc is highly desirable. Hence, it is expected that the results of this study will influence future PAH/PH screening guidelines and clinical practice in SSc patients. DETECT is ongoing and has recruited 317 out of 500 patients. Recruitment is expected in up to 20 countries over a 2-year period.