Box 1: World Health Organization revised Jones criteria for the diagnosis of rheumatic fever1
Diagnostic categoriesCriteria
Primary episode of rheumatic fever*Two major or 1 major and 2 minor manifestations plus evidence of a preceding group A streptococcal infection
Recurrent episode of rheumatic fever in a patient without established rheumatic heart diseaseTwo major or 1 major and 2 minor manifestations plus evidence of a preceding group A streptococcal infection
Recurrent episode of rheumatic fever in a patient with established rheumatic heart diseaseTwo minor manifestations plus evidence of a preceding group A streptococcal infection
Rheumatic choreaOther manifestations or evidence of group A streptococcal infection not required
Chronic valve lesions of rheumatic heart disease (patients presenting for the first time with pure mitral stenosis or mixed mitral valve disease and aortic valve disease)§No other criteria required for diagnosis of rheumatic heart disease
Major manifestations
  • carditis

  • polyarthritis

  • chorea

  • erythema marginatum

  • subcutaneous nodules

Minor manifestations
  • fever

  • polyarthralgia

  • elevated acute phase reactants (erythrocyte sedimentation rate or leukocyte count)

Supporting evidence of a preceding streptococcal infection within the last 45 d
  • electrocardiogram (prolonged P–R interval)

  • elevated or rising antistreptolysin O or other streptococcal antibody titre, or

  • a positive throat culture, or

  • a rapid antigen test for group A streptococci or recent scarlet fever

  • * Patients may present with polyarthritis (or with only polyarthralgia or monoarthritis) and with several (3 or more) other minor manifestations, together with evidence of recent group A streptococcal infection. Some of these cases may later turn out to be rheumatic fever. It is prudent to consider them as cases of “probable rheumatic fever” (once other diagnoses are excluded) and advise regular secondary prophylaxis. Such patients require close follow up and regular examination of the heart. This cautious approach is particularly suitable for patients in vulnerable age groups in high incidence settings.

  • Infective endocarditis should be excluded.

  • Some patients with recurrent attacks may not fulfill these criteria.

  • § Congenital heart disease should be excluded.

  • Reproduced with permission from Rheumatic fever and rheumatic heart disease: report of a WHO expert consultation, Geneva, 29 October – 1 November 2001. No. 923 of WHO Technical Report Series. Geneva: World Health Organization; 2001, available http://whqlibdoc.who.int/trs/WHO_TRS_923.pdf?ua=1 (accessed 2013 Nov. 20).