findings In his treatise Traité clinique des maladies du coeur he introduced the term endocarditis and clarified the clinical picture, giving an accurate account of the cardiac involvement and other manifestations in patients with RF The eponym maladie de Bouillaud for RF recognized his great contribution, distinguished by its exceptional accuracy and clinical significance In the same year, James Hope, as had already been pointed out by Wells and Dundas, detailed and emphasized the association of acute pericarditis with RF In the Harveian lecture of 1898, Walter Butler Cheadle offered a full description of the RF syndrome as we know it today: carditis, polyarthritis, and chorea as well as subcutaneous nodules and erythema marginatum Thus RF was first described about 120 years ago Subsequently, the pathognomonic and distinctive microscopic nodules of rheumatic carditis were described in 1904 by Ludwig Aschoff.8–12 The first report of a possible connection between a bacterial infection and RF had been suggested by Mantle in 1887, but it was not until early 1930s that the causal relationship between infection by the β-hemolytic streptococcus and RF was established.13–15 From then on, data about the disease was gathered in many other fields Todd, in 1932,16 introduced a method for measuring one of the antibodies developed by the human body after the contact with the bacteria Then, a year later, Rebecca Lancefield17 classified Streptococcus into five distinct groups Subsequently,18 continuous administration of sulfanilamide was shown to prevent recurrences followed in 195019 and 195120 with demonstration that adequate treatment of streptococcal pharyngitis with penicillin prevented the disease As early as 1944, Thomas Duckett Jones had proposed a set of clinical and laboratorial data to guide and reduce the overdiagnosis of RF.21 The Jones criteria were subsequently modified and updated by the Committee of the American Heart Association.22–27 Subclinical carditis, diagnosed by echocardiography and Doppler, has been included in the last revision, published in the year 2015 Jones criteria have long been recognized as guidelines for the diagnosis of the first episode of the acute phase The knowledge of the action of antibiotics in preventing the disease, and the systematization of the diagnosis by means of the important Jones criteria, heralded a new era of studies Epidemiology RF has a universal distribution, although significant differences in the rates of incidence and prevalence depend on the interaction of characteristics of the etiologic agent and its human host, besides environmental and socioeconomic conditions RF has almost disappeared in developed countries; it remains rampant in countries and regions characterized by poverty, overcrowding, and lack of adequate health care Due to the causal relationship of streptococcal pharyngotonsillitis and RF, the epidemiology of the two diseases is closely related As with most diseases, factors responsible for the occurrence of RF may pertain to the host, the environment, or the pathogen, or a combination of all three A genetic predisposition has been previously suggested based on association of rheumatic heart disease with certain haplotypes such as HLA DR2, DR4, DR1, and DRw6; however it has not been convincingly proven.28 A meta-analysis of twin studies showed a concordance risk of RF of 44% in monozygotic and 12% in dizygotic twins.29 RF is more frequent among children and adolescents between the ages 5 and 15 years, and has a peak of incidence around the ages of 8 to 9 years These ages coincide with the peak of streptococcal pharyngotonsillitis in school-aged children; this infection is found to be less common in late adolescence and in adults Likewise, RF is uncommon in children younger than 4 years and exceedingly rare under the age of 2 years.30–32 Data from Brazil revealed that 2.5% of patients had their first episode before age 3 years.33 In a study from India, Aschoff bodies were found in onetenth of autopsied cases, the majority from patients between 16 and 40 years of age, indicating a recent attack of carditis.34 Under special circumstances, such as focal epidemics of streptococcal infections in military populations or closed institutions, the incidence of RF can increase in adults.35,36 RF occurs in all populations, and shows equal frequency in both genders The exception is Sydenham chorea, which is more common in females, and is hardly ever seen in males after puberty.37 RF has usually been reported as a disease of the temperate climates, but currently it is more prevalent in warm tropical climates, especially in low- and middle-income countries Similarly, the influence of seasonal variation in the rates of incidence is now less defined, but in general follows that of streptococcal infections, which are most commonly observed in late winter and early spring.38,39 The disease is reputed to be more frequent in urban centers than in rural communities, but this is probably due to overcrowding.40 Although data thus far are inconclusive, it has been suggested that there is an increased susceptibility to RF in certain ethnic groups The aboriginals of Australia's Northern Territory, and the Polynesians, both from rural areas, show a markedly increased incidence of the disease.38,41 In Hawaii, wide differences in the prevalence of chronic rheumatic heart disease have been documented in Samoan schoolchildren when compared with white Hawaiians.42 Similarly, higher frequencies of RF and chronic rheumatic heart disease have been found in the Maori population of New Zealand,4 and among black schoolchildren in South Africa.43 Data from India show a more severe or malignant disease, with multivalve involvement and congestive heart failure even in the first attack of RF.44 Many factors, however, can overlap and interfere in the calculations of the incidence of RF and the prevalence of chronic rheumatic heart disease, since different rates can be found under diverse environmental conditions for any given population In this context, besides the role played by the bacteria and human host, other factors, such as differences in patterns of living conditions and streptococcal exposure, in addition to quality of and access to health care, are important and can impact the geographical distribution of the disease, as well as the severity of its sequels All over the world, pharyngotonsillitis is one of the most prevalent infections caused by the β-hemolytic streptococcus It accounts for up to one-third of the throat infections in children, and up to one-tenth in adults.36,45 Although streptococcal infections are very frequent, only a few individuals develop RF It is calculated that under endemic conditions, 0.3% of untreated infections, and 3% in epidemics, will lead to a first episode of RF.46 Despite the fact that group A Streptococcus has continued to constitute about 30% of all pharyngitis in children over the past five decades, the occurrence of RF in industrialized countries has fallen to a great extent.47 The reasons for this selective decline are not entirely clear It is presumed to be linked to change in the epidemiology of Streptococcus pharyngitis with a shift from rheumatogenic to nonrheumatogenic strains In some of the Aboriginal population of the Northern Territory of Australia, RF has occurred secondary to pyoderma rather than pharyngitis,48 and group C and G Streptococci rather than group A Streptococci.49 An analysis of emm type distribution shows a variation in the molecular epidemiology of group A Streptococcus (GAS) infections in Africa and the Pacific in comparison with that observed in high-income countries.50,51