Update of screening and diagnostic modalities for connective tissue disease-associated pulmonary arterial hypertension

Amber Young; Vivek Nagaraja; Mark Basilious; Mirette Habib; Whitney Townsend; Heather Gladue; David Badesch; J Simon R Gibbs; Deepa Gopalan; Alessandra Manes; Ronald Oudiz; Toru Satoh; Adam Torbicki; Fernando Torres; Vallerie McLaughlin; Dinesh Khanna

doi:10.1016/j.semarthrit.2018.10.010

Update of screening and diagnostic modalities for connective tissue disease-associated pulmonary arterial hypertension

Semin Arthritis Rheum. 2019 Jun;48(6):1059-1067. doi: 10.1016/j.semarthrit.2018.10.010. Epub 2018 Oct 14.

Authors

Affiliations

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.

Keywords: Connective tissue disease; Pulmonary arterial hypertension; Screening.

Publication types

Research Support, N.I.H., Extramural
Systematic Review

MeSH terms

Case-Control Studies
Connective Tissue Diseases / complications*
Echocardiography
Exercise Test
Humans
Mass Screening
Pulmonary Arterial Hypertension / diagnosis*
Pulmonary Arterial Hypertension / etiology
Respiratory Function Tests

Abstract

Publication types

MeSH terms

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