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Acute rheumatic fever (ARF) results from the body’s autoimmune response to a throat infection caused by Streptococcus pyogenes, also known as the group A Streptococcus bacteria. Rheumatic heart disease (RHD) refers to the long-term cardiac damage caused by either a single severe episode or multiple recurrent episodes of ARF. It is RHD that remains a significant worldwide cause of morbidity and mortality, particularly in resource-poor settings. While ARF and RHD were once common across all populations, improved living conditions and widespread treatment of superficial S. pyogenes infections have caused these diseases to become comparatively rare in wealthy areas (Carapetis, 2007). Currently, these diseases mainly affect those in low- and middle-income nations, as well as in indigenous populations in wealthy nations where initial S. pyogenes infections may not be treated, which allows for the development of harmful post-infectious sequelae (Carapetis, 2007).
The development of ARF occurs approximately two weeks after S. pyogenes infection (Gewitz, et al., 2015). The clinical manifestations and symptoms of ARF can be severe and are described in the Revised Jones Criteria (Gewitz, et al., 2015). Symptoms of ARF can include polyarthritis, carditis, chorea, the appearance of subcutaneous nodules, and erythema marginatum or a rash associated with ARF (Gewitz, et al., 2015; Martin, et al., 2015). These symptoms usually require patients to be hospitalized for two to three weeks, during which time the outward symptoms resolve, but the resultant cardiac damage may persist. With repeated S. pyogenes pharyngitis infections, ARF can recur and cause cumulative damage to the heart valves (Martin, et al., 2015).
This chapter will briefly cover the epidemiology and pathophysiology of ARF and RHD, and will also outline the clinical manifestations, diagnostic considerations, and recommended treatment and management options for both conditions. Finally this chapter will also highlight prevention strategies for ARF and RHD and will discuss current vaccination efforts against S. pyogenes.
Epidemiology of ARF and RHD
Burden of Disease
The global burden of ARF and RHD is significant, and is predominantly found in populations living in low-resource settings (Carapetis, Steer, Mulholland, & Weber, 2005). Incidence rates of ARF are poorly documented in most low- and middle-income countries, including in populations with a high prevalence of RHD, where it is presumed that a high incidence of ARF also occurs. This relates both to the lack of infrastructure for disease surveillance in those settings, but also to a paucity of ARF cases that are presented for clinical care. It is not known if the latter issue is a result of health-seeking behavior (people with ARF who choose not to seek health care), or due to inadequate diagnosis of ARF by health staff. The latter may in turn be due to true misdiagnosis as a result of problems with training, a lack of access to diagnostic facilities (such as electrocardiography, streptococcal serology, acute phase reactant testing, and echocardiography); or possibly because many cases of ARF may be milder, or even sub-clinical, in highly endemic settings (Bishop, Currie, Carapetis, & Kilburn, 1996).
ARF incidence rates have been reported to be as high as 155 per 100,000 children aged 5 to 14 years in indigenous populations in North Queensland, Australia (Gray, Brown, & Thomson, 2012) with rates in the Northern Territory reported at 380.1 per 100,000 children in 2002 (Parnaby & Carapetis, 2010). In New Zealand, ARF affects mainly children and teens aged 4–19 years who are predominantly of Māori and Pasifika descent and are living in low socioeconomic regions of the North Island (Jack, et al., 2015). Between 1993 and 2009, the average incidence rates for ARF based on hospitalization data for children 5–14 years were 81.2 per 100,000 for Pasifika children, as compared with 40.2 per 100,000 for Māori children and 2.1 per 100,000 for non-Māori, non-Pasifika children (Milne, Lennon, Stewart, Vander Hoorn, & Scuffham, 2012a).