Rheumatic Fever in Emergency Medicine Clinical Presentation

Updated: Jul 19, 2021
  • Author: Steven J Parrillo, DO, FACOEP, FACEP; Chief Editor: Barry E Brenner, MD, PhD, FACEP  more...
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Acute rheumatic fever (ARF) is associated with 2 distinct patterns of presentation. The first pattern of presentation is sudden onset. It typically begins as polyarthritis 2-6 weeks after streptococcal pharyngitis and is usually characterized by fever and toxicity. The second pattern is insidious or subclinical onset, which may occur if the initial abnormality is mild carditis.

Age at onset influences the order of complications. Younger children tend to develop carditis first, whereas older patients tend to develop arthritis first.


Physical Examination

Diagnosis of acute rheumatic fever (ARF) requires a high index of suspicion. Guidelines of diagnosis from the American Heart Association (AHA) include major and minor criteria (ie, modified Jones criteria), which were updated in 2015. Laboratory evidence of a preceding group A streptococcal infection is needed whenever possible. Without it, the diagnosis of ARF is in doubt, except in patients with chorea, which may be the sole initial manifestation of ARF, and rarely in patients with indolent rheumatic carditis with insidious onset and slow progression. [26]

The AHA suggests that diagnostic criteria may be applied differently, depending on the rate of ARF or rheumatic heart disease (RHD) in the local population. This can help avoid overdiagnosis in low-incidence populations and underdiagnosis in high-risk ones. The AHA defines low risk as an ARF incidence of < 2 per 100,000 school-aged children (usually 5–14 years old) per year or an all-age prevalence of RHD of ≤1 per 1000 population per year. Children not clearly from a low-risk population are at moderate to high risk, depending on their reference population. [26]

Criteria for the diagnosis of initial ARF are the presence of two major manifestations or one major and two minor manifestations. For recurrent ARF, the criteria are two major manifestations, one major and two minor manifestations, or three minor manifestations. [26]

Major manifestations comprise the following:

  • Carditis, clinical and/or subclinical (ie, detected by echocardiography)
  • Arthritis
  • Chorea
  • Erythema marginatum
  • Subcutaneous nodules

In patients from low-risk populations, arthritis must be polyarthritis. For patients from moderate- and high-risk populations, either monoarthritis or polyarthritis qualifies; polyarthralgia may qualify if other causes have been excluded.

Minor manifestations in low-risk populations comprise the following:

  • Polyarthralgia
  • Fever ≥38.5°C
  • Acute phase reactions: Erythrocyte sedimentation rate (ESR) ≥60 mm in the first hour and/or C-reactive protein (CRP) level ≥3.0 mg/L
  • Prolonged PR interval, after accounting for age variability (unless carditis is a major criterion)

Minor manifestations in moderate- and high-risk populations comprise the following:

  • Monoarthralgia
  • Fever ≥38°C
  • ESR ≥30 mm/h and/or CRP ≥3.0 mg/dL
  • Prolonged PR interval, after accounting for age variability (unless carditis is a major criterion)

Numerous authors have suggested that changes to the Jones Criteria may be in order. For example, some have suggested that echocardiography be performed in all suspected cases in order to avoid both underdiagnosis and overdiagnosis. Carapetis and Currie suggest that cases are missed because some patients have only monoarthritis and not polyarthritis. [27]  They would like to see monoarthritis become a major criterion. The same authors suggest that the set point of fever at 38ºC might be too high. Rayamajhi et al suggest that arthralgia be changed from a minor to a major Jones criterion. [28]

As mentioned above, there are authorities who suggest that less stringent echocardiographic criteria for the diagnosis of rheumatic valvular disease will increase the number of cases diagnosed. [21]

Karacan et al found several asymptomatic rhythm disturbances in children with ARF. Those without carditis often had accelerated junctional rhythm. Those with carditis often had premature contractions. The group suggests a role for 24-hour electrocardiography. [29]