PRIMEVIEW

ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

For the Primer, visit doi:10.1038/nrdp.2015.84

Acute rheumatic fever (ARF) results MANAGEMENT

EPIDEMIOLOGY Each year, there are an estimated
from group A Streptococcus (GAS) 470,000 ARF cases. There are 33 million
infection and typically affects the heart, prevalent RHD cases and 275,000 deaths
joints, central nervous system and skin. Penicillin treatment is the core of ARF
annually as a result of ARF and RHD. Most ARF management, to clear existing GAS infection
Repeated episodes of ARF, or a single cases occur in children aged 514 years.
and to prevent reinfection and future ARF
severe episode, can seriously damage the ercrowding
heart valves leading to rheumatic heart Ov episodes. For serious cases of valvular stenosis
disease (RHD) a chronic condition that Geography and regurgitation, cardiac surgery is carried out
Poverty to repair or replace the damaged valve. Women
can cause heart failure.
Sex with severe RHD who are planning to have
children should ideally have cardiac surgery
Although
Healthcare prior to conception, as pregnancy places
MECHANISMS ARF rates do
not differ according considerable physiological strain on the
to sex, more women heart. Indeed, RHD is coming to be
During a GAS infection in the throat, a cross- than men develop RHD recognized as a leading cause
reactive immune response can occur through a of obstetric mortality in the
process called molecular mimicry and can cause developing world.
ARF. Here, the immune system raises a response to
GAS antigens and a subset of the ensuing activated Penicillin prophylaxis is
B and T cells are also able to recognize self antigens usually recommended for
located at different sites in the body. In the heart, 10years after the last ARF episode
this cross-reactivity is mainly mediated by groupA to minimize the risk of developing RHD
carbohydrate-specific antibodies and leads to
carditis (inflammation), formation of lesions called
Aschoff bodies and tissue damage, in particular
ARF and RHD are most OUTLOOK
in the valves. Over time, cumulative damage
tothe heart valves can remodel their structure to common in developing
cause RHD, which initially involves regurgitation countries and are strongly Following the dramatic decline in the incidence of
incomplete closure of valve leaflets causing related to socioeconomic ARF and RHD in most western countries during the
blood to flow backwards through the valve and disadvantage associations twentieth century, which can be partly attributed
subsequently, as a result of scarring over time, that are probably functions of to improved housing conditions, research
stenosis reduced ability of the valve to open household overcrowding and interest in these diseases waned. However, the
thatimpedes blood flow. poor access to medical care substantial burden of ARF and RHD in lower-
income communities demands renewed focus
and investment. In particular, successful RHD
T B prevention requires a better understanding of its
DIAGNOSIS epidemiological landscape. This, in combination
with large-scale trials on screening and
Diagnosis of ARF is made using marginatum) and minor valves. Echocardiography is prophylactic strategies for ARF and RHD, would lay
Erythema the Jones Criteria a system manifestations (such as fever) an essential tool in evaluating the groundwork for effective interventions in GAS
Inammation
and valve
marginatum and
subcutaneous
of combining clinical features with serological confirmation of RHD, the diagnosis of which endemic regions. Ultimately, increased research
damage nodules including major manifestations recent GAS infection. Carditis involves finding valvular disease funding and the implementation of existing and
(carditis, arthritis, chorea, is detected in the majority of along with a history of ARF or emerging tools to combat ARF and RHD will
subcutaneous nodules and patients with ARF and primarily identifying typical features of require concerted efforts to advocate for the needs
a skin rash called erythema affects the mitral and aortic RHD regardless of history. of affected populations to policy makers.
Sydenhams chorea Arthritis

Article number: 15085; doi:10.1038/nrdp.2015.85; published online 14 Jan 2016

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