Please use this identifier to cite or link to this item: http://hdl.handle.net/2440/105353
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Type: Journal article
Title: Epidemiology and disease characteristics of systemic sclerosis-related pulmonary arterial hypertension: results from a real-life screening programme
Author: Morrisroe, K.
Stevens, W.
Sahhar, J.
Rabusa, C.
Nikpour, M.
Proudman, S.
Hill, C.
Lester, S.
Rischmueller, M.
Walker, J.
Nash, P.
Ngian, G.
Roddy, J.
Strickland, G.
Thakkar, V.
Zochling, J.
Citation: Arthritis Research and Therapy, 2017; 19(1):42-1-42-10
Publisher: BioMed Central
Issue Date: 2017
ISSN: 1478-6354
1478-6362
Statement of
Responsibility: 
Kathleen Morrisroe, Wendy Stevens, Joanne Sahhar, Candice Rabusa, Mandana Nikpour, Susanna Proudman and the Australian Scleroderma Interest Group (ASIG)
Abstract: Pulmonary arterial hypertension (PAH) is the leading cause of death in systemic sclerosis (SSc). Annual screening with echocardiogram (ECHO) is recommended. We present the methodological aspects of a PAH screening programme in a large Australian SSc cohort, the epidemiology of SSc-PAH in this cohort, and an evaluation of factors influencing physician adherence to PAH screening guidelines.Patient characteristics and results of PAH screening were determined in all patients enrolled in a SSc longitudinal cohort study. Adherence to PAH screening guidelines was assessed by a survey of Australian rheumatologists. Summary statistics, chi-square tests, univariate and multivariable logistic regression were used to determine the associations of risk factors with PAH.Among 1636 patients with SSc, 194 (11.9%) had PAH proven by right-heart catheter. Of these, 160 were detected by screening. The annual incidence of PAH was 1.4%. Patients with PAH diagnosed on subsequent screens, compared with patients in whom PAH was diagnosed on first screen, were more likely to have diffuse SSc (p = 0.03), be in a better World Health Organisation (WHO) Functional Class at PAH diagnosis (p = 0.01) and have less advanced PAH evidenced by higher mean six-minute walk distance (p = 0.03), lower mean pulmonary arterial pressure (p = 0.009), lower mean pulmonary vascular resistance (p = 0.006) and fewer non-trivial pericardial effusions (p = 0.03). Adherence to annual PAH screening using an ECHO-based algorithm was poor among Australian rheumatologists, with less than half screening their patients with SSc of more than ten years disease duration.PAH is a common complication of SSc. Physician adherence to PAH screening recommendations remains poor. Identifying modifiable barriers to screening may improve adherence and ultimately patient outcomes.
Keywords: Australian Scleroderma Interest Group (ASIG); Systemic sclerosis; scleroderma; pulmonary arterial hypertension; screening algorithm
Rights: © The Author(s). 2017 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
RMID: 0030066213
DOI: 10.1186/s13075-017-1250-z
Grant ID: http://purl.org/au-research/grants/nhmrc/1113954
http://purl.org/au-research/grants/nhmrc/1071735
Appears in Collections:Public Health publications

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