Acute Rheumatic Fever and Rheumatic Heart Disease

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.