CME REVIEW ARTICLE
Acute Rheumatic Fever
Revised Diagnostic Criteria
Kelsey L. Rhodes, DO, Malia M. Rasa, MD, MS, and Loren G. Yamamoto, MD, MPH, MBA
Abstract: The Jones criteria of 2 major criteria or 1 major plus 2 minor
criteria that have been classically used to establish the diagnosis have been
significantly modified in 2015 by the American Heart Association. The
criteria now include the utilization of echocardiography and Doppler color
flow mapping as diagnostic tools for carditis, along with defining criteria in
relation to overall population risk, delineating low- versus moderate-high
risk populations. Monoarthritis and polyarthralgia are now major criteria
for moderate- to high-risk groups.
Key Words: acute rheumatic fever, rheumatic heart disease, Jones criteria,
chorea, erythema marginatum, subcutaneous nodules, arthritis, arthralgia,
carditis, valvulitis
(Pediatr Emer Care 2018;34: 436–442)
TARGET AUDIENCE
This article is intended for physicians, nurse practitioners,
and other providers who care for children with disease and condi-
tions related to rheumatic fever and rheumatic heart disease.
LEARNING OBJECTIVES
After completion of this CME article, the reader should be
better able to:
1. Apply the clinical Jones criteria used to make the diagnosis of
acute rheumatic fever.
2. Stratify the Jones criteria based on population risk groups.
3. Interpret laboratory testing and diagnostic studies to confirm
acute rheumatic fever.
CASE
A 10-year-old Polynesian male sought care in the emergency
department for severe left ankle pain. He gave a history that a
younger cousin has rolled over his ankle with a tricycle. An
x-ray was negative. He was noted to be febrile, but this was attrib-
uted to a concurrent viral infection. He was treated with acetamin-
ophen and an elastic wrap. He returned 2 days later with severe
pain in his right ankle and foot. This area was extremely tender
to touch with very painful range of motion and minimal swelling.
His left ankle pain had largely resolved. He was also noted to have
a grade 2/6 holosystolic murmur at the apex radiating into his
axilla. An electrocardiogram showed a third-degree atrioventricular
Pediatric Residents (Rhodes, Rasa) and Professor of Pediatrics (Yamamoto), Depart-
ment of Pediatrics, University of Hawaii John A. Burns School of Medicine; Pediat-
ric Residents (Rhodes, Rasa) and Chief of Staff and Pediatric Emergency Physician
(Yamamoto), Kapi'olani Medical Center for Women and Children, Honolulu, HI.
Disclosure: The authors, faculty, and staff in a position to control the content of
this CME activity and their spouses/life partners (if any) have disclosed that
they have no financial relationships with, or financial interest in, any
commercial organizations pertaining to this educational activity.
Reprints: Loren G. Yamamoto, MD, MPH, MBA, Department of Pediatrics,
University of Hawaii John A. Burns School of Medicine, 1319 Punahou St,
7th Floor, Honolulu, HI 96826 (e‐mail: Loreny@hawaii.edu).
Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.
ISSN: 0749-5161
436 www.pec-online.com
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(AV) block. An echocardiogram confirmed mitral insufficiency
with good myocardial contractility and minimal congestive heart
failure. His aortic valve was normal. There was no pericardial ef-
fusion. His laboratory test results showed a leukocytosis and
highly elevated C-reactive protein (CRP) and erythrocyte sedimenta-
tion rate (ESR). A brain natriuretic peptide assay was highly ele-
vated owing to congestive heart failure. When asked, he did
confirm that he had a sore throat 3 weeks ago, but he did not tell
his mother about it. He was hospitalized for acute rheumatic fever
with mitral valvulitis and congestive heart failure. Streptococcal
serologies subsequently returned positive. The positive major
criteria were migratory polyarthritis and carditis/valvulitis, and
the positive minor criteria were fever, leukocytosis, elevated in-
flammatory markers. Another related finding but not part of
classic Jones criteria was third-degree AV block (classically
first-degree AV block is a minor criterion).
Acute rheumatic fever (ARF) is a delayed autoimmune medi-
ated process after infection with group A streptococcus (GAS).
Acute rheumatic fever occurs approximately 2 weeks after initial
group A strep pharyngitis. Initial presentation includes a spectrum
of manifestations, such as carditis, arthritis, chorea, and skin
findings. This cluster of manifestations is described as the Jones
criteria. Damage to the heart, most commonly valvular inflamma-
tion, scarring, and stenosis, can cause lasting effects, known as
rheumatic heart disease (RHD), which is a significant cause of
morbidity and mortality.1
The Jones criteria have been used since 1944 for the diagno-
sis of acute rheumatic fever. Initial modifications were made in
1992, which were reconfirmed in 2000 by the American Heart
Association (AHA).2 The most recent modification to the Jones
criteria were made by the AHA in 2015 and include utilization
of echocardiography and Doppler color flow mapping as diagnos-
tic tools for carditis, along with defining criteria in relation to
overall population risk, delineating low- versus moderate-high risk
populations. This statement also assigned for the first time a clas-
sification of recommendations and level of evidence according to
the AHA classification system.3
EPIDEMIOLOGY
The overall incidence of acute rheumatic fever has been de-
clining in most developed countries, including North America
and Europe, over the recent decades.4,5 This is thought to be attrib-
uted to improved access to antibiotics, improved hygiene, reduced
overcrowding along with other socioeconomic changes, and
possibly shifting GAS serotypes3,6 with variable rheumatogenic
virulence. Areas with a higher burden of disease typically include
areas of poverty and limited resources, such as underdeveloped
countries. However, certain indigenous populations within devel-
oped countries also see a higher incidence of ARF.2 For instance,
children aged 5 to 14 years of the indigenous populations in Australia
have reported one of the highest incidences of ARF in the world,
whereas the overall incidence for the country remains on par with
North America and Europe. Similarly, the Maori and Pasifika
populations of New Zealand living in low resource areas have
Pediatric Emergency Care • Volume 34, Number 6, June 2018
Pediatric Emergency Care • Volume 34, Number 6, June 2018 Acute Rheumatic Fever

reported a higher incidence of ARF than in non-Maori and non-
Pasifika children.1 Higher incidence worldwide has also been
reported in Africa, the Pacific basin, South America, Asia,
and parts of the Middle East.7–12
Acute rheumatic fever is most commonly associated with pre-
ceding pharyngitis with the highest incidence in children between
5 and 15 years of age.13,14 There is also some controversy surround-
ing impetigo as a possible cause of acute rheumatic fever and RHD,
as areas with a high prevalence of RHD also have a high burden of
GAS impetigo and a low incidence of GAS pharyngitis.15
Even with improvements in detection and early treatment of
streptococcal pharyngitis and ARF, 350,000 people per year die
from ARF and/or RHD worldwide.
DIAGNOSIS
The Jones criteria are categorized into major and minor
criteria. Group A streptococcus infection is required in addition
to 2 major or 1 major plus 2 minor criteria, to make a diagnosis
of ARF. In the 2015 revision of the Jones Criteria, major criteria
differ depending on risk stratification into low- or moderate- to
high-risk populations. Low-risk populations are defined as those
with an incidence of ARF less than 2 per 100,000 school-aged
children, or less than or equal to 1 per 1000 all-age RHD prevalence
per year.3
Prior GAS infection can be proven by (1) increased or rising
antistreptolysin O titer, or other streptococcal antibodies; (2) a
positive throat culture for group A beta-hemolytic streptococci;
and (3) a positive rapid group A streptococcal carbohydrate anti-
gen test in a patient with high probability of streptococcal pharyn-
gitis.3 According to the current criteria, history or presence of
clinically diagnosed scarlet fever is not considered to be sufficient
evidence for GAS infection.16
hence one of the major guideline changes for diagnosis in the
2015 update.
Chorea is described as an involuntary nonrhythmic move-
ment that is sometimes accompanied by concurrent muscular
weakness or emotional disturbances.20,21 Subcutaneous nodules
are firm and painless, and manifest at joint, scalp, and over spi-
nous processes of the thoracic and lumbar spine. Erythema
marginatum (Fig. 1) is a fairly rare manifestation of ARF. It is a
macular pink rash that is blanching with pale clearing and distinct
borders. It can appear on the trunk and extremities and rarely
involves the face.2
Minor Criteria
The minor criteria are also stratified by risk. Low-, moderate-,
and high-risk populations all have minor criteria of fever (>38.5°C)
and prolonged PR interval, as defined by age-specific norms (if
carditis is not otherwise a major criteria for diagnosis). For low-risk
populations, other minor criteria include polyarthralgia and ESR of
greater than 60 mm/h and/or CRP level of greater than 3.0 mg/dL
(ie, above institutional norm). For moderate- and high-risk popu-
lations, arthralgia may be monoarticular, ESR is greater than
30 mm/h, and/or CRP level is greater than 3.0 mg/dL. Note that
the ESR criteria differ between the groups, but the CRP values
do not differ. In addition, joint manifestations, whether arthritis or
arthralgia, may only be considered as major or minor criteria but
not as both in the same patient. The revised Jones criteria are shown
in Table 1 (adapted from the original).3
Recurrent Rheumatic Fever
In recurrent rheumatic fever, as seen in Table 1, 3 minor
criteria may be sufficient to make the diagnosis. However, other

Major Criteria
The major criteria for low-risk populations continue to be
carditis, polyarthritis, chorea, erythema marginatum, and subcuta-
neous nodules with the addition/revision that carditis may be
clinical and/or subclinical.3 In contrast, the major criteria for
moderate- and high-risk populations have changed to include
monoarthritis or polyarthralgia, as replacements for arthritis,
and carditis may currently be clinical or subclinical.
Carditis continues to be the most common serious manifesta-
tion of ARF, ranging from mild involvement to life-threatening
damage.2 Carditis may involve the endocardium, myocardium,
or pericardium, although valvulitis continues to be the most con-
sistent feature. Generally, carditis without valvular involvement is
rarely, if ever, considered rheumatic in nature.3 For example, iso-
lated pericarditis is more likely to be caused by etiologies other
than acute rheumatic fever. Traditionally, the diagnosis of valvuli-
tis was made through auscultation of a murmur, usually indicating
mitral regurgitation (holosystolic murmur at the apex radiating
into the axilla) or aortic valve regurgitation (diastolic murmur
heard at the base). However, technologic advances, mainly echo-
cardiography and Doppler flow techniques, have made the diagno-
sis of carditis possible even in the absence of physical examination
findings. This is now considered subclinical carditis and has been
included in the diagnostic criteria.3
The arthritis associated with ARF is typically described as a
migratory polyarthritis that most often involves the large joints
(knees, ankles, elbows, and wrists).16 It is generally self-limited
even when not treated; however, there is rapid improvement with
salicylates or nonsteroidal anti-inflammatory drugs. Several re- FIGURE 1. Erythema marginatum (courtesy of Dr Raul Rudoy;
ports have noted aseptic monoarthritis to be an important clinical copyright 2016, Loren Yamamoto, University of Hawaii
manifestation in areas with increased incidence of ARF,17–19 Department of Pediatrics).

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Rhodes et al
TABLE 1. The Revised Jones Criteria (Adapted From AHA 2015 Guidelines3)
Patients must have evidence of preceding GAS infection
For initial ARF:
• 2 Major manifestations or 1 major plus 2 minor manifestations
For recurrent ARF
• 2 Major or 1 major and 2 minor or 3 minor manifestations
Major criteria
Low-risk populations
• Carditis (clinical and/or subclinical)
• Arthritis (must be polyarthritis)
• Chorea
• Erythema marginatum
• Subcutaneous nodules
Minor criteria
Low-risk populations
• Polyarthralgia
• Fever >38.5°C
• ESR >60 mm/h and/or CRP >3.0 mg/dL
• Prolonged PR interval for age (carditis cannot be major criterion)
likely causes that tie together the clinical presentation must be ex-
cluded before making the diagnosis of recurrent ARF.
DIFFERENTIAL DIAGNOSIS
The diagnosis of ARF continues to be largely clinical. If a pa-
tient meets some but not all of the Jones criteria, alternative diag-
noses should be considered.
For a patient presenting with arthritis testing positive for
GAS infection, but without further criteria for ARF, consider the
diagnosis of poststreptococcal reactive arthritis. Poststreptococcal
reactive arthritis is characterized by an additive arthritis of large,
small, and axial joints with poor response to nonsteroidal anti-
inflammatory drug therapy occurring 7 to 10 days after GAS in-
fection.22 This is thought to be a separate entity from the arthritis
of ARF, although it has a similar pathology and it can be associated
with carditis.22 Other considerations include reactive and septic ar-
thritis, juvenile idiopathic arthritis, or other autoimmune/connective
tissue disorders.23
Chorea may have varying differentials including Wilson dis-
ease, toxin or drug ingestion, intracranial pathology such as tumor
or infection, or autoimmune disorders.23 One further differential
for chorea secondary to GAS infection is pediatric autoimmune
neurologic disorder associated with streptococci (PANDAS).
This diagnosis is unique because it is immunologically similar to
Sydenham chorea seen with ARF through production of antineuronal
antibodies but differs slightly in presentation.24 Pediatric autoimmune
neurologic disorder associated with streptococci most commonly pre-
sents with abrupt onset of tics, obsessive-compulsive symptoms, and
fine choreiform movements usually of the fingers and toes.25 This is
in contrast to the more obvious choreoathetoid involuntary move-
ments of Syndenham chorea.
Alternative diagnoses for carditis without other criteria for
ARF may include infective endocarditis, congenital heart disease,
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Pediatric Emergency Care • Volume 34, Number 6, June 2018
Moderate- and high-risk populations
• Carditis (clinical and/or subclinical)
• Arthritis
○ Monoarthritis or polyarthritis
○ Polyarthralgia
• Chorea
• Erythema marginatum
• Subcutaneous nodules
Moderate- and high-risk populations
• Monoarthralgia
• Fever >38.5°C
• ESR >30 mm/h and/or CRP >3.0 mg/dL
• Prolonged PR interval for age
(carditis cannot be major criterion)
or pericarditis secondary to infectious agents or connective
tissue disorders.23
Note that other listed entities in the differential diagnosis
such as Lyme disease, Kawasaki disease, and systemic lupus
erythematosus all have joint, heart, and skin manifestations.
Some of these have brain manifestations as well.
MANAGEMENT
Patients with a high suspicion of ARF are generally hospital-
ized for further diagnostic evaluation and treatment. The goals of
management include the confirmation of the diagnosis (which has
long-term implications), eradication of GAS, prophylaxis against
reoccurrence of ARF, management of carditis, and symptomatic
treatment of arthritis and chorea.1
Penicillin is the mainstay of treatment and prophylaxis for
ARF. Initial treatment with either oral penicillin for a 10-day course
or an intramuscular (IM) benzathine penicillin injection is recom-
mended.1 Generally, IM treatment is preferred over oral treatment
because of problems with medication adherence.26 Prophylaxis
with IM benzathine penicillin injections every 3 to 4 weeks should
be initiated immediately after treatment. Penicillin prophylaxis is
associated with reduced severity of rheumatic valvular lesions
over time.21,23 The duration of prophylaxis is not standardized
and depends on the extent of involvement or severity of illness.
The World Health Organization (WHO) gives general guidelines,
recommending prophylaxis for 5 years after the last episode or
until 18 years of age for patients without carditis, 10 years after
the last episode or until at least 25 years of age for patients with
mild carditis, or lifelong for those with severe valvular disease and
after valvular surgery.26
Anti-inflammatory medications have long been used for
carditis, along with antibiotics, as mentioned above. Aspirin is
considered first line therapy according the WHO guidelines,
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Pediatric Emergency Care • Volume 34, Number 6, June 2018
although there is no contemporary evidence to support one form
of anti-inflammatory medication over another. Naproxen may offer
similar efficacy to aspirin with less adverse effects.1 Corticosteroids
are thought to play a role during acute carditis, especially for pa-
tients with moderate-to-severe disease including heart failure, peri-
cardial effusion, or pancarditis.23 The WHO guidelines recognize
oral corticosteroids as a treatment option for those patients with
moderate-to-severe disease, and intravenous corticosteroids in life-
threatening circumstances.26 Other anti-inflammatory options
for severe carditis include intravenous immune globulin (IVIG) and
adrenocorticotropic hormone.1 However, a 2015 Cochrane review
found no significant evidence to suggest that corticosteroids,
IVIG, or aspirin are able to prevent cardiac disease in ARF pa-
tients, questioning their role in ARF for carditis. The same review
determined that there is no lifesaving role for corticosteroids in
patients with RHD.27 Furthermore, a study comparing oral versus
intravenous corticosteroids used in patients with cardiac failure
classes III and IV according to the New York Heart Association
classification found no significant difference in reducing the
course of disease severity between the 2 therapies.28 Those with
cardiac failure should follow typical management with diuretics
and vasodilators. Bed rest has also been a mainstay recommen-
dation, although this therapy has also not been proven.1
For patients with severe carditis with valvular involvement,
surgical intervention may be necessary. Surgery during the acute
phase is avoided if feasible, as it is associated with poor out-
comes.1 Generally, surgery is required secondary to significant
or symptomatic chronic rheumatic valve disease.26 Valve repair
is much preferred over valve replacement if possible. It is usually
reserved for patients with lone mitral valve involvement, as
success rates are improved and no long-term anticoagulation
is necessary23; however, it should be noted that the state of the
art of valve replacement is evolving rapidly.
Arthritis is generally managed symptomatically with anti-
inflammatory drugs.1 Naproxen and aspirin are most commonly
used, although naproxen offers less adverse effects with equiva-
lent efficacy to aspirin.23
Management of chorea is mainly supportive, unless there is
significant motor impairment and interference with daily life.1
Anticonsvulsant drugs can provide symptomatic relief and are
generally well tolerated. Neuroleptics are usually avoided owing
to extrapyramidal effects although these were commonly used
for treatment in the past. Oral corticosteroids, IVIG, and plasma
exchange are also possible treatment options.23
PROGNOSIS
The prognosis of patients with ARF and RHD is largely de-
termined by severity and reoccurrence of disease. Resolution of
RHD is possible and generally occurs within the first 12 months
after the initial episode for patients with mild to moderate
carditis.23 Mitral valve regurgitation alone is the most likely to re-
gress, whereas involvement of the aortic valve rarely shows full
resolution.1 Resolution is dependent upon compliance with
penicillin prophylaxis, as poor compliance and reoccurrence in-
crease the risk of complications of RHD and development of
chronic RHD.1
Although mitral and aortic regurgitation may be asymptom-
atic for years, progression of ARF to RHD, particularly in moder-
ate to severe cases, is related to continued scarring of the valves
and/or compensatory dilation of the left ventricle causing further
stretching of the mitral and aortic annulus.1,29,30 Rheumatic mitral
and aortic stenosis occur secondary to valvular scarring, fibrosis,
and calcification, with mitral stenosis associated more with recur-
rence of disease than with its initial presenting severity.1
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Acute Rheumatic Fever
Along with poor compliance and frequent reoccurrences,
other factors that predispose patients to chronic RHD are severe
carditis and young age with first episode.1 Chronic RHD increases
the patient's risk of complications such as heart failure, infective
endocarditis, complications during pregnancy, stroke, arrhythmia,
and premature death.23
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