Amber Young1, Vivek Nagaraja1, Mark Basilious2, Mirette Habib2, Whitney Townsend3, Heather Gladue4, David Badesch5, J Simon R Gibbs6, Deepa Gopalan7, Alessandra Manes8, Ronald Oudiz9, Toru Satoh10, Adam Torbicki11, Fernando Torres12, Vallerie McLaughlin13, Dinesh Khanna14. 1. Division of Rheumatology, Scleroderma Program, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. 2. University of Michigan Undergraduate Program, Ann Arbor, MI, USA. 3. Taubman Health Sciences Library, University of Michigan Library, Ann Arbor, MI, USA. 4. Arthritis & Osteoporosis Consultants of the Carolinas, Charlotte, NC, USA. 5. Division of Pulmonary Sciences & Critical Care Medicine, University of Colorado, Denver, CO, USA. 6. National Heart and Lung Institute, Imperial College London, London, UK. 7. Department of Radiology, Imperial College London, London, UK. 8. Department of Investigational, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy. 9. LA Biomedical Research Institute at Harbor-University of California Los Angeles, Torrance, CA, USA. 10. Division of Cardiology, Department of Medicine, Kyorin University School of Medicine, Tokyo, Japan. 11. Medical Centre for Postgraduate Education, CMKP European Health Centre, Otwock, Poland. 12. University of Texas Southwestern Medical Center, Dallas, TX, USA. 13. Division of Cardiology, University of Michigan, Ann Arbor, MI, USA. 14. Division of Rheumatology, Scleroderma Program, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. Electronic address: khannad@med.umich.edu.
Abstract
OBJECTIVE: Pulmonary arterial hypertension (PAH) has high morbidity and mortality in connective tissue diseases (CTDs), especially systemic sclerosis (SSc). In this systematic review, we provide an update on screening measures for early detection of PAH in CTD. METHODS: Manuscripts published between July 2012 and October 2017, which incorporated screening measures to identify patients with PAH by right heart catheterization, were identified. Risk of bias was assessed using the QUADAS-2 tool. RESULTS: The systematic review resulted in 1514 unique citations and 22 manuscripts were included for final review; the majority of manuscripts had a lower risk of bias based on the QUADAS-2 tool. There were 16 SSc cohort studies and 6 case-control studies (SSc 4, SLE 2). Four SSc cohort studies evaluated transthoracic echocardiography (TTE) only. Eight SSc cohort studies evaluated composite measures including ASIG, DETECT, and a combination of tricuspid regurgitation velocity (TRV) and PFT variables. DETECT and ASIG had greater sensitivity and negative predictive value (NPV) compared to the 2009 ESC/ERS guidelines in different cohorts. The addition of PFT variables, such as DLCO or FVC/ DLCO ratio, to TRV, resulted in greater sensitivity and NPV compared to TRV alone. CONCLUSION: Current screening for PAH in CTDs is centered on SSc. Data continues to support the use of TTE and provides additional evidence for use of composite measures.
OBJECTIVE:Pulmonary arterial hypertension (PAH) has high morbidity and mortality in connective tissue diseases (CTDs), especially systemic sclerosis (SSc). In this systematic review, we provide an update on screening measures for early detection of PAH in CTD. METHODS: Manuscripts published between July 2012 and October 2017, which incorporated screening measures to identify patients with PAH by right heart catheterization, were identified. Risk of bias was assessed using the QUADAS-2 tool. RESULTS: The systematic review resulted in 1514 unique citations and 22 manuscripts were included for final review; the majority of manuscripts had a lower risk of bias based on the QUADAS-2 tool. There were 16 SSc cohort studies and 6 case-control studies (SSc 4, SLE 2). Four SSc cohort studies evaluated transthoracic echocardiography (TTE) only. Eight SSc cohort studies evaluated composite measures including ASIG, DETECT, and a combination of tricuspid regurgitation velocity (TRV) and PFT variables. DETECT and ASIG had greater sensitivity and negative predictive value (NPV) compared to the 2009 ESC/ERS guidelines in different cohorts. The addition of PFT variables, such as DLCO or FVC/ DLCO ratio, to TRV, resulted in greater sensitivity and NPV compared to TRV alone. CONCLUSION: Current screening for PAH in CTDs is centered on SSc. Data continues to support the use of TTE and provides additional evidence for use of composite measures.
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