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Incidence of acute rheumatic fever in the world: a systematic review of population-based studies. Tibazarwa KB, Volmink JA, Mayosi BM. Heart. 2008 Dec;94(12):1534-40. Epub 2008 Jul 31. Review. According to the WHO, 15.6 million people worldwide are living with RHD. Of the 500 000 who develop ARF each year, 300 000 go on to develop RHD and 233 000 deaths are attributable each year to ARF/RHD. The are conservative estimates and the true burden of disease is thought to be even greater. This mortality rates are higher than those of rotaviruses, meningitis and hepatitis B and half of those with malaria. Rheumatic fever: neglected again. Watkins DA, Zuhlke LJ, Engel ME, Mayosi BM. Science. 2009 Apr 3;324(5923):37. No abstract available. The pathogenic pathway for rheumatic heart disease starts with the susceptible individual. Twin studies have demonstrated that a genetic component may exist. ( add link)(Repeated streptococcal infections are thought to prime a susceptible individual resulting in a heightened response to an infection with rheumatogenic streptococcus. This results in acute rheumatic fever usually 3 weeks after the initial tonsilllopharyngeal infection. The precise pathogenetic mechanism has not yet been defined, yet strong evidence exists that an autoimmune response mediates the development of RF/RHD in a susceptible host. Genetically determined host factors interacting with the molecular similarity of the streptococcal antigens and host tissues results in the involvement of brain, joints, tissues and heart. The pathogenesis of RF/RHD is a complex maze of events involving major histocompatibility antigens, potential tissue-specific antigens, antibodies and superantigenic activation . However it remains clear that environmental factors such as overcrowding, poor living conditions and poor access to health care is the most significant determinant of disease distribution. Lancet. 2005 Jul 9-15;366(9480):155-68. Acute rheumatic fever. Carapetis JR, McDonald
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