Streptococcal pharyngitis and rheumatic heart disease: the superantigen hypothesis revisited

• Published in: Infection, genetics and evolution genetics and evolution, 2018-07, Vol.61, p.160-175
• Main Authors: Hurst, Jacklyn R., Kasper, Katherine J., Sule, Akshay N., McCormick, John K.
• Format: Article
• Language: English
• Subjects: Acute rheumatic fever; Group A Streptococcus; Pharyngitis; Rheumatic heart disease; Streptococcus pyogenes; Superantigen
• ISSN: 1567-1348; 1567-7257
• DOI: 10.1016/j.meegid.2018.03.006

Streptococcus pyogenes is a human-specific and globally prominent bacterial pathogen that despite causing numerous human infections, this bacterium is normally found in an asymptomatic carrier state. This review provides an overview of both bacterial and human factors that likely play an important role in nasopharyngeal colonization and pharyngitis, as well as the development of acute rheumatic fever and rheumatic heart disease. Here we highlight a recently described role for bacterial superantigens in promoting acute nasopharyngeal infection, and discuss how these immune system activating toxins could be crucial to initiate the autoimmune process in rheumatic heart disease. •Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are autoimmune disorders driven by Streptococcus pyogenes pharyngitis•The molecular basis of acute rheumatic fever and rheumatic heart disease is still not completely understood•Haplotypes of MHC class II can be important risk factors for the development of ARF and RHD•Bacterial superantigens are potent T cell activators and are known to exacerbate autoimmunity in animal models•We propose streptococcal superantigens may be crucial yet understudied factors that initiate ARF/RHD autoimmune responses