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Type: Journal article
Title: Defining primary systemic sclerosis heart involvement: a scoping literature review
Author: Ross, L.
Prior, D.
Proudman, S.
Vacca, A.
Baron, M.
Nikpour, M.
Citation: Seminars in Arthritis and Rheumatism, 2019; 48(5):874-887
Publisher: Elsevier
Issue Date: 2019
ISSN: 0049-0172
Statement of
Laura Ross, David Prior, Susanna Proudman, Alessandra Vacca, Murray Baron, Mandana Nikpour
Abstract: BACKGROUND:Clinically evident primary heart involvement due to systemic sclerosis (SHI) is considered a poor prognostic factor and is a leading cause of systemic sclerosis (SSc) related death. Yet, there remains no consensus definition of SHI and poor understanding of the natural history and risk factors for the development of SHI. METHODS:We performed a scoping literature review of published articles with a primary focus of SHI to capture previously used definitions of SHI and items used to measure SHI. Any factors reported to be associated with an increased risk of SHI were recorded. RESULTS:Of the 2436 records identified in a search of MEDLINE, EMBASE and PubMed databases, 295 were included in the final scoping review. Analysis of the literature revealed studies of variable quality, generally low patient numbers and highly heterogeneous definitions of SHI within studies. There is no clear consensus from the literature as to the scope of SHI and the prognostic significance of sub-clinical investigation abnormalities commonly detected. CONCLUSION:The lack of a standardised definition of SHI remains a significant unmet need in SSc. The results of this review will assist in the development of consensus classification criteria to enable more accurate quantification of the burden of SHI, identification of factors associated with increased risk of developing SHI, and evaluation of the efficacy of any novel therapeutic strategies.
Keywords: Cardiac disease; Definition; Myocardial disease; Scleroderma; Scoping review; Systemic sclerosis
Rights: © 2018 Elsevier Inc. All rights reserved.
RMID: 0030099617
DOI: 10.1016/j.semarthrit.2018.07.008
Grant ID:
Appears in Collections:Medicine publications

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