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Acute rheumatic fever G043

Last amended 
9 May 2019
Current RMA Instruments
Reasonable Hypothesis SOP53 of 2019
Balance of Probabilities SOP54 of 2019
Changes from previous Instruments

SOP Bulletin 208

ICD Coding
  • ICD-9-CM Codes: 390,391.0,391.2,391.8,391.9,392.0,392.9
  • ICD-10-AM Codes: I00, I01, I02
Brief description

Acute rheumatic fever is a condition that develops generally two to four weeks after a streptococcal throat infection. It causes inflammation that may involve the joints, heart, skin and brain.  The condition remains common in children in developing countries and in disadvantaged communities with poor access to health care.  The condition frequently goes undetected and may only become known in retrospect, due to the later development of chronic rheumatic heart disease (which is covered by a separate SOP). 

Confirming the diagnosis

The diagnosis of rheumatic fever is generally made based on the acute clinical manifestations (e.g. carditis, arthritis, central nervous system involvement), using appropriate criteria, together with laboratory confirmation of the prior beta-haemolytic streptococcus infection.  That laboratory confirmation can be at the time of the infection, or later, using serology.  The diagnosis may also be made in cases where indolent carditis, detected on echocardiography performed months after a beta-haemolytic streptococcal pharyngitis, is the only manifesation.  Similarly, chorea (a movement disorder) may be the only manifestation and can also provide a basis for (delayed)diagnosis. 

The relevant medical specialist is an infectious diseases physician.

Additional diagnoses covered by SOP
  • Recurrent acute rheumatic fever
  • Acute rheumatic carditis
Conditions not covered by SOP
  • rheumatic heart disease*
  • rheumatic aortic valve incompetence or stenosis*
  • rheumatic mitral valve incompetence or stenosis*
  • rheumatic pulmonary valve disease*
  • rheumatic tricuspid valve incompetence or stenosis*
  • chronic rheumatic myocarditis*
  • chronic rheumatic pericarditis*

* all covered by rheumatic heart disease SOP

Clinical onset

The usual presentation is an acute illness occurring 2 to 4 weeks after a bacterial (streptococcal) pharyngitis. Major manifestations are: carditis and valvulitis; migratory polyarthritis, predominantly involving the large joints; central nervous system involvement (eg, Sydenham chorea); subcutaneous nodules and erythema marginatum (a type of rash).  Minor manifestations include arthralgia (joint pain) and fever.

Clinical worsening

The only SOP worsening factor is for inability to obtain appropriate clinical management.  Acute phae management consists of anti-inflammatory therapy, antibiotic therapy, and heart failure management, if required.