Contemporary incidence and prevalence of rheumatic fever and rheumatic heart disease in Australia using linked data: The case for policy change

Judith M. Katzenellenbogen, Daniela Bond-Smith, Rebecca J. Seth, Karen Dempsey, Jeffrey Cannon, Ingrid Stacey, Vicki Wade, Nicholas de Klerk, Melanie Greenland, Frank M. Sanfilippo, Alex Brown, Jonathan R. Carapetis, Rosemary Wyber, Lee Nedkoff, Joe Hung, Dawn Bessarab, Anna P. Ralph

Research output: Contribution to journalReview articlepeer-review

69 Citations (Scopus)


BACKGROUND: In 2018, the World Health Organization prioritized control of acute rheumatic fever (ARF) and rheumatic heart disease (RHD), including disease surveillance. We developed strategies for estimating contemporary ARF/RHD incidence and prevalence in Australia (2015–2017) by age group, sex, and region for Indigenous and non-Indigenous Australians based on innovative, direct methods. 

METHODS AND RESULTS: This population-based study used linked administrative data from 5 Australian jurisdictions. A cohort of ARF (age <45 years) and RHD cases (<55 years) were sourced from jurisdictional ARF/RHD registers, surgical registries, and inpatient data. We developed robust methods for epidemiologic case ascertainment for ARF/RHD. We calculated age-specific and age-standardized incidence and prevalence. Age-standardized rate and prevalence ratios compared disease burden between demographic subgroups. Of 1425 ARF episodes, 72.1% were first-ever, 88.8% in Indigenous people and 78.6% were aged <25 years. The age-standardized ARF first-ever rates were 71.9 and 0.60/100 000 for Indigenous and non-Indigenous populations, respectively (age-standardized rate ratio=124.1; 95% CI, 105.2–146.3). The 2017 Global Burden of Disease RHD prevalent counts for Australia (<55 years) underestimate the burden (1518 versus 6156 Australia-wide extrapolated from our study). The Indigenous age-standardized RHD prevalence (666.3/100 000) was 61.4 times higher (95% CI, 59.3–63.5) than non-Indigenous (10.9/100 000). Female RHD prevalence was double that in males. Regions in northern Australia had the highest rates. 

CONCLUSIONS: This study provides the most accurate estimates to date of Australian ARF and RHD rates. The high Indigenous burden necessitates urgent government action. Findings suggest RHD may be underestimated in many high-resource settings. The linked data methods outlined here have potential for global applicability.

Original languageEnglish
Article numbere016851
Pages (from-to)1-26
Number of pages26
JournalJournal of the American Heart Association
Issue number19
Publication statusPublished - 6 Oct 2020

Bibliographical note

Funding Information:
This project received funding from the National Health and Medical Research Council through project grant (#114652) and seed funds from the End-RHD Centre for Research Excellence and HeartKids. Katzenellenbogen is supported by a Heart Foundation of Australia Future Leader Fellowship (#102043). Ralph (#1142011) and Nedkoff (#1110337) are supported by a National Health and Medical Research Council Fellowships. Wyber is supported by a National Health and Medical Research Postgraduate Scholarship (#1151165).

Publisher Copyright:
© 2020 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

Copyright 2021 Elsevier B.V., All rights reserved.


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