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Rheumatic fever

Last updated: January 21, 2021

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Rheumatic fever is an inflammatory sequela involving the heart, joints, skin, and central nervous system (CNS) that occurs two to four weeks after an untreated infection with group A streptococcus (GAS). The pathogenic mechanisms that cause rheumatic fever are not completely understood, but molecular mimicry between streptococcal M protein and human cardiac myosin proteins is thought to play a role. Because of the structural similarities between the two proteins, antibodies and T cells activated to respond to streptococcal proteins also react with the human proteins, causing tissue injury and inflammation. In addition to nonspecific symptoms (e.g., fever, malaise, and fatigue), patients present with symptoms involving the heart (pancarditis), joints (migratory polyarthritis), skin (subcutaneous nodules, erythema marginatum), and/or CNS (Sydenham chorea). The diagnosis of acute rheumatic fever is primarily a clinical one, and is based on the Jones criteria. Diagnostic evaluation in acute rheumatic fever typically shows elevated inflammatory markers, positive antistreptococcal antibodies, and valvular damage on echocardiogram. The first-line treatment is penicillin combined with symptomatic anti-inflammatory treatment, typically with salicylates or glucocorticoids (if salicylates are not effective). Acute rheumatic fever may be complicated by progressive, permanent damage to the heart valves (especially the mitral valve), resulting in chronic rheumatic heart disease. Preventing the cardiac complications of rheumatic fever is the goal of both primary prophylaxis (i.e., antibiotic therapy for GAS pharyngitis) and secondary prophylaxis (antibiotic administration following an episode of acute rheumatic fever.

  • Delayed inflammatory complication of group A β-hemolytic streptococcal pharyngitis that usually occurs within 2–4 weeks of acute infection [1]
  • One of the nonsuppurative complications of GAS pharyngitis
  • Rheumatic heart disease refers to two clinical entities:
    • Acute pancarditis as a sequela of GAS infection
    • Chronic cardiac valvular changes as a complication of acute rheumatic fever
  • Peak incidence: 5–15 years of age [2]
  • Prevalence: more common in resource-limited countries [3]

Epidemiological data refers to the US, unless otherwise specified.

Rheumatic heart disease tends to involve the high-pressure valves (i.e., the mitral and aortic valves).

The symptoms of acute rheumatic fever can be remembered by reading the JONES criteria (see “Diagnostics” below) as an acronym that replaces the “o” with a heart: J = Joints, ♥ = Pancarditis, N = Nodules, E = Erythema marginatum, S = Sydenham chorea

Diagnosis of acute rheumatic fever is based on the Jones criteria, which primarily describe the clinical findings of the condition. Evidence of a preceding GAS infection is also preferred (unless carditis or chorea are present). Laboratory tests and imaging may be necessary to assess any outstanding Jones criteria.

Jones criteria [10]

Two major criteria or one major plus two minor criteria or three minor criteria are required for diagnosis.

Low risk population

High risk population
Major criteria
Minor criteria

Additional findings [1]

  • Cardiac involvement is the most important prognostic factor.
    • Early death from rheumatic fever is usually due to myocarditis rather than valvular defects.
    • Patients with a history of carditis (and possible post-inflammatory scarring and calcification) during an initial rheumatic fever episode are at high risk of developing valvular heart defects with recurrent episodes → rheumatic heart disease
  1. Steer A, Gibofsky A. Acute rheumatic fever: Clinical manifestations and diagnosis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/acute-rheumatic-fever-clinical-manifestations-and-diagnosis?source=search_result&search=rheumatic+fever&selectedTitle=1~118.Last updated: January 6, 2017. Accessed: February 13, 2017.
  2. Steer A, Gibofsky A. Acute rheumatic fever: Epidemiology and pathogenesis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/acute-rheumatic-fever-epidemiology-and-pathogenesis?source=search_result&search=rheumatic+fever+epidemiology&selectedTitle=1~118.Last updated: January 3, 2017. Accessed: February 13, 2017.
  3. Remenyi B, Carapetis J, Wyber R, Taubert K, Mayosi BM. Position statement of the World Heart Federation on the prevention and control of rheumatic heart disease. Nat Rev Cardiol. 2013; 10 (5): p.284-292. doi: 10.1038/nrcardio.2013.34 . | Open in Read by QxMD
  4. Dinkla K, Rohde M, Jansen WT, Kaplan EL, Chhatwal GS, Talay SR. Rheumatic fever-associated Streptococcus pyogenes isolates aggregate collagen. J Clin Invest. 2003; 111 (12): p.1905-1912. doi: 10.1172/JCI200317247 . | Open in Read by QxMD
  5. Munoz R, Morell V, da Cruz E, Vetterly CG. Critical Care of Children with Heart Disease: Basic Medical and Surgical Concepts. Springer ; 2010
  6. Gilbert DL. Sydenham chorea. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/sydenham-chorea?source=search_result&search=sydenham+Chorea&selectedTitle=1~10#H1.Last updated: September 2, 2016. Accessed: February 13, 2017.
  7. Zühlke L, Peters F. Clinical Manifestations and Diagnosis of Rheumatic Heart Disease. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-rheumatic-heart-disease.Last updated: January 23, 2018. Accessed: October 26, 2018.
  8. Izabela Szczygielska, Elżbieta Hernik, Beata Kołodziejczyk, Agnieszka Gazda, Maria Maślińska, and Piotr Gietka. Rheumatic fever – new diagnostic criteria. Rheumatology. 2018 .
  9. FRASER WJ, HAFFEJEE Z, COOPER K. Rheumatic Aschoff nodules revisited: an immunohistological reappraisal of the cellular component. Histopathology. 1995; 27 (5): p.457-461. doi: 10.1111/j.1365-2559.1995.tb00310.x . | Open in Read by QxMD
  10. Roberts S, Kosanke S, Terrence Dunn S, Jankelow D, Duran CMG, Cunningham MW. Pathogenic Mechanisms in Rheumatic Carditis: Focus on Valvular Endothelium. J Infect Dis. 2001; 183 (3): p.507-511. doi: 10.1086/318076 . | Open in Read by QxMD
  11. Armstrong C. AHA Guidelines on Prevention of Rheumatic Fever and Diagnosis and Treatment of Acute Streptococcal Pharyngitis. Am Fam Physician. 2010; 81 (3): p.346-359.
  12. Pichichero ME. Treatment and Prevention of Streptococcal Tonsillopharyngitis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. http://www.uptodate.com/contents/treatment-and-prevention-of-streptococcal-tonsillopharyngitis.Last updated: October 27, 2016. Accessed: February 13, 2017.
  13. Steer A, Gibofsky A. Acute rheumatic fever: Treatment and prevention. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. http://www.uptodate.com/contents/acute-rheumatic-fever-treatment-and-prevention.Last updated: January 4, 2017. Accessed: February 13, 2017.