An urgent need for early diagnosis and treatment of acute rheumatic fever
Authors: James Kayima, Mark Kaddumukasa
Journal: The Lancet Global Health (2024)

In this issue of The Lancet Global Health, Scott Wirth and colleagues1Wirth SH Pulle J Seo J et al.Outcomes of rheumatic fever in Uganda: a prospective cohort study.Lancet Glob Health. 2024; 12: e500-e508Google Scholar report that rheumatic heart disease remains the largest contributor of cardiovascular-related mortality in children worldwide. Rheumatic heart disease affects 40 million people globally, with almost 300 000 deaths annually in low-income and middle-income countries (LMICs).2Ghamari SH Abbasi-Kangevari M Saeedi Moghaddam S et al.Rheumatic heart disease is a neglected disease relative to its burden worldwide: findings from Global Burden of Disease 2019.J Am Heart Assoc. 2022; 11e025284 Crossref PubMed Scopus (14) Google Scholar High-income countries (HICs) have almost eliminated rheumatic heart disease over the last half of the 20th century. Timely use of affordable and widely available antibiotics for the treatment of streptococcal throat and skin infection and management and prevention of acute rheumatic fever have been central in reducing the burden of acute rheumatic fever in HICs.3Ordunez P Martinez R Soliz P Giraldo G Mujica OJ Nordet P Rheumatic heart disease burden, trends, and inequalities in the Americas, 1990–2017: a population-based study.Lancet Glob Health. 2019; 7: e1388-e1397Summary Full Text Full Text PDF PubMed Scopus (36) Google Scholar However, the adoption of these approaches in high-burden LMICs has been suboptimal. The main challenges for rheumatic heart disease prevention have been pervasive poverty, widening disparity in health-care provision, sparse data on the true burden of disease, late diagnosis and presentation with advanced valvular disease, and ineffective delivery of antibiotics for treatment and prophylaxis. Wirth and colleagues contribute to our understanding of the burden of acute rheumatic fever and report a high incidence of acute rheumatic fever among patients recruited from the community into a rheumatic heart disease registry in Uganda. The authors also report a high mortality rate among Ugandan patients diagnosed with definitive rheumatic heart disease followed up after 3–5 years, especially during the first year of diagnosis. The authors also describe a reduction in severity of carditis with monthly benzathine penicillin injections, these results are similar to other studies.4Torres RPA Torres RFA de Crombrugghe G et al.Improvement of rheumatic valvular heart disease in patients undergoing prolonged antibiotic prophylaxis.Front Cardiovasc Med. 2021; 8676098 Crossref Scopus (0) Google Scholar, 5Beaton A Okello E Rwebembera J et al.Secondary antibiotic prophylaxis for latent rheumatic heart disease.N Engl J Med. 2022; 386: 230-240Crossref PubMed Scopus (57) Google Scholar This finding is expected and is consistent with a study that showed high mortality among Indigenous Australians compared with other Australians.6Colquhoun SM Condon JR Steer AC Li SQ Guthridge S Carapetis JR Disparity in mortality from rheumatic heart disease in Indigenous Australians.J Am Heart Assoc. 2015; 4e001282 Crossref Scopus (20) Google Scholar In LMICs, where access to valvular surgery remains largely out of reach, emphasis should be placed on prevention of acute rheumatic fever, early diagnosis and management of streptococcal sore throat infection, and incident cases of acute rheumatic fever. Studies show that prompt treatment of suspected streptococcal pharyngitis with oral antibiotics like amoxicillin or intramuscular benzathine penicillin reduces attack rate of acute rheumatic fever by up to 70–80.7Gerber MA Baltimore RS Eaton CB et al.Prevention of rheumatic fever and diagnosis and treatment of acute streptococcal pharyngitis: a scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research: endorsed by the American Academy of Pediatrics.Circulation. 2009; 119: 1541-1551Crossref PubMed Scopus (499) Google Scholar The use of clinical prediction rules rather than the expensive microbiological confirmation of group A streptococcal infection has supported this endeavor especially in low-resource settings with a paucity of laboratories.8Engel ME Cohen K Gounden R et al.The Cape Town clinical decision rule for streptococcal pharyngitis in children.Pediatr Infect Dis J. 2017; 36: 250-255Crossref PubMed Scopus (14) Google Scholar Additionally, addressing issues related to acute rheumatic fever knowledge, understanding the links between sore throat and heart disease, and improving health-seeking behaviours in those with acute rheumatic fever and rheumatic heart disease could provide another avenue to reduce the burden of rheumatic heart disease. The majority of patients with group A streptococcal pharyngitis are either asymptomatic or have mild forms that do not prompt seeking medical care.9Oliver JR Pierse N Stefanogiannis N Jackson C Baker MG Acute rheumatic fever and exposure to poor housing conditions in New Zealand: a descriptive study.J Paediatr Child Health. 2017; 53: 358-364Crossref PubMed Scopus (29) Google Scholar Moreover, most of episodes of acute rheumatic fever go unrecognised, especially in deprived environments, with most of the patients presenting with advanced carditis. Developing strategies for diagnosis and management of acute rheumatic fever through identification and early diagnosis of strep sore throat and acute rheumatic fever is urgently needed in communities. The success of antibiotic prophylaxis in preventing the worsening of valvular lesions underscores the importance of screening for rheumatic heart disease with subsequent initiation of antibiotic prophylaxis. Antibiotic prophylaxis is probably one of the key strategies to reduce the acute rheumatic fever and rheumatic heart disease burden in LMICs. Any successful acute rheumatic fever and rheumatic heart disease control programme depends heavily on adherence to antibiotic prophylaxis, which remains suboptimal. A need for implementation research to develop better ways of organising the health-care system to optimse the delivery of antibiotic prophylaxis is mandated. Evidence from more successful control programmes in Australia and New Zealand, that focused on early acute rheumatic fever and rheumatic heart disease diagnosis and improving the secondary prophylaxis coverage over time has shown improved adherence to penicillin prophylaxis, reduced acute rheumatic fever recurrence rates, and most importantly no progression of valvular lesions in those who adhered to prophylaxis correctly.10Stacey I RA de Dassel J Nedkoff L et al.The evidence that rheumatic heart disease control programs in Australia are making an impact.Aust N Z J Public Health. 2023; 47100071 Crossref Scopus (1) Google Scholar It is likely that adapting the approach of organising successful acute rheumatic fever and rheumatic heart disease control efforts shown elsewhere into programmes in LMICs would improve the outcomes of patients with the disease. Therefore, improving and innovating strategies for group A streptococcal diagnosis and treatment cannot be neglected. Scalable and sustainable models for delivery of primary and secondary prevention are needed, as well as the conduct of economic evaluation of these prevention strategies so that a suitable solution is found for LMICs. Community and government engagements in the implementation and dissemination of research of various primary prevention strategies cannot be overstated. These opportunities are availed as a reference point for all stakeholders who aim to contribute to the increasing momentum towards the global control and prevention of rheumatic heart disease. We declare no competing interests. Outcomes of rheumatic fever in Uganda: a prospective cohort studyThese are the first contemporary data from sub-Saharan Africa on medium-term acute rheumatic fever outcomes. Mortality rates exceeded those reported elsewhere. Most decedents already had chronic carditis at initial acute rheumatic fever diagnosis, suggesting previous undiagnosed episodes that had already compounded into rheumatic heart disease. Our data highlight the large burden of undetected acute rheumatic fever in these settings and the need for improved awareness of and diagnostics for acute rheumatic fever to allow earlier detection. Full-Text PDF Open Access