Address for
Correspondence: Prof. Dr. KV Sahasranam MD, DM, Senior
Consultant Cardiologist, Baby Memorial Hospital, Kozhikode,
Kerala, India. PIN: 673004. E-mail:
ramani2911@gmail.com
Keywords: Acute Rheumatic Fever, Revised Jones
Criteria
Acute Rheumatic Fever (ARF) is still a major problem in some
developing and low middle income countries though the
incidence and prevalence has remarkably reduced in North
America and Europe. Ever since T. Duckett
Jones in 1944 proposed the Jones criteria, it was modified
and later revised by the American Heart Association (AHA) in
1992. This was reconfirmed in and AHA sponsored Work shop in
2002 [1]. No modification or change was suggested after the
introduction of Doppler Echocardiography (DE) for the
diagnosis of clinical and "subclinical carditis". When a
clinical diagnosis by the clinician fails to show evidence
of carditis, DE may pick up cases of "subclinical carditis".
Moreover, previous recommendations did not include the
Classification of Recommendations (Class I, II a, II b, and
III) and Levels of Evidence (Levels A, B and C). Recently,
however, a scientific statement from the American Heart
Association (AHA) has published an update to the Jones
criteria including the use of DE in the diagnosis of ARF
[2].
The pattern of disease also is different among different
populations. In the low and middle income countries and low
socioeconomic groups where prevalence is high, the disease
occurs throughout the year with no obvious outbreaks of
epidemic proportions whereas in the high socioeconomic
groups, the incidence is low but outbreaks may occur at
intervals. Low risk has been defined as having and ARF
incidence of less than 1 per 1000 population per year [2].
The major criteria for the diagnosis of ARF remain the same
namely, carditis, polyarthritis, chorea, subcutaneous
nodules and erythema marginatum. But in highly susceptible
populations, aseptic monoarthritis, polyarthralgia and low
grade fever should be factored in while making the diagnosis
[3]. Carditis, being the commonest major criteria (50-60%),
is often a pancarditis but mainly presents clinically as a
valvulitis. Subclinical carditis is diagnosed when the
classical clinical auscultatory signs are not seen but DE
shows evidence of mitral or aortic valvulitis. This is a new
development and more than 25 studies are available to
substantiate this. A review of 23 articles showed the
prevalence of subclinical carditis from 0 - 53% emphasizing
the high variability of the finding. Pooled evidence puts
the prevalence of subclinical carditis at 16.8% of ARF. So
much so, the Indian Working group has recommended DE to be
done in all confirmed cases of ARF without clinical evidence
of carditis [4].
The DE findings in pathological mitral regurgitation are
that it should be seen in at least two views, the jet length
of 2 cm or more, in at least one view, a peak velocity of
more than 3 m/s and a pansystolic jet in at least one
envelope. Those for pathological aortic regurgitation are to
be seen in at least two views, a jet length of 1 cm or more
in at least one view, a peak velocity of more 3 m/s and a
pan diastolic jet in at least one view [5]. Concurring with
the recent studies the AHA has suggested the "
Echocardiogram
with Doppler should be performed in all cases of confirmed
and suspected ARF (Class. I Level of Evidence B)". It
also recommends that "
It is reasonable to consider
performing echocardiogram/Doppler studies in any patient
with diagnosed or suspected ARF even if documented
carditis is not present on diagnosis (Class II a,
Level of Evidence C)" [2].
Coming to arthritis, in addition to the classical migratory
polyarthritis, aseptic monoarthritis may be an important
clinical presentation as reported from India, Australia and
Fiji [3, 6, 7, 8]. As high as 16-18% of confirmed cases of
ARF reported from Australia present as monoarthritis. Hence
the AHA has recommended that "
At present, consideration
that monoarthritis may be a part of ARF spectrum should be
limited to patients from moderate to high risk populations
(class I Level of evidence C)". The writing group also
recommends that
"Inclusion of polyarthralgia as a
major manifestation is applicable only for moderate or
high incidence populations and only after careful
consideration and exclusion of other causes of arthralgia
such as autoimmune, viral or reactive arthropathies (Class
II b, Level of Evidence C)" [2].
Whereas the recommendations on chorea and skin
manifestations are not changed, and ESR of more than 60
mm/hour and a C-reactive protein (CRP) of more than 3.0
mg/dL are considered typical of ARF. The evidence for a
preceding streptococcal infection also remains unchanged
from the previous recommendations [2]. The criteria for
Rheumatic fever recurrences also remain the same. The
recommendations also add that the addition of monoarthritis
and subclinical carditis as major manifestations and low
grade fever more 37.5 degrees Centigrad) as a minor
criterion with the increased sensitivity when considered in
high risk populations.
With respect to India, there have been reports that the
incidence and prevalence studies have reported a decline in
incidence of ARF and a decrease in prevalence of Rheumatic
Heart disease (RHD). The introduction of echocardiography /
Doppler and its easy availability in many urban and many
rural settings adds a different angle to the diagnosis of
ARF and RHD in India [9]. A working group of the Indian
Academy of Pediatrics brought out guidelines for the
diagnosis and treatment of ARF and RHD in 2008 in which,
subclinical carditis and its diagnosis by
echocardiography/Doppler has received a passing mention but
no criteria for the diagnosis have been
elaborated. Also they added that echocardiography /Doppler
is not mandatory but needs to be done to establish clinical
carditis [10].
Hence in conclusion, accepting the revision of AHA in 2015,
it is time that Pediatricians and Cardiologists in India
woke up to formulate their own guidelines for the diagnosis
of ARF adding Echocardiography and Doppler to the
armamentarium of tests in the diagnosis of ARF.
References
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the Jones Criteria workshop. Circulation, 2002; 106:
2521-2523.
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131:1806-18.
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