Acute rheumatic fever (ARF) is a multisystem disease
resulting from
An autoimmune reaction to infection with group A
streptococcus.
Although many parts of the body may be affected,
almost all of the manifestations resolve completely.
The major exception is cardiac
valvular damage which may persist
ARF is mainly a disease of children age 5–14 years.
Initial episodes become less common in older
adolescents and young adults and are rare in persons
age >30 years.
By contrast, recurrent episodes of ARF remain
relatively common in adolescents and young adults
This pattern contrasts with the prevalence of RHD
which peaks between 25 and 40years.
More commonly affects females, sometimes up to
twice as frequently as males.
ARF is exclusively caused by infection of the upper
respiratory tract with group A streptococci
M-serotypes (particularly types 1, 3, 5, 6, 14, 18, 19, 24,
27, and 29)
ARF in high-incidence regions
A streptococcus has the potential to cause ARF. The
potential role of skin infection and of groups C and G
streptococci is currently being investigated.
Approximately 3–6% of any population may be
susceptible to ARF
latent period of ~3 weeks (1–5 weeks)
between the precipitating group A
streptococcal infection and the appearance of
the clinical features of ARF.
The exceptions are chorea and indolent
carditis, which may follow prolonged latent
periods lasting up to 6 months.
Many group A streptococcal infection is
commonly subclinical
The most common clinical features are
polyarthritis (present in 60–75% of cases)
carditis (50–60%)
The prevalence of chorea in ARF varies
substantially between populations, ranging
from <2 to 30%.
Erythema marginatum and subcutaneous
nodules are now rare, being found in <5%
of cases.
Heart involvement
Up to 60% of patients with ARF progress to RHD
The endocardium, pericardium or myocardium may be
affected.
Valvular damage is the hallmark of rheumatic carditis.
The mitral valve is almost always affected, sometimes together
with the aortic valve isolated aortic valve involvement is rare
Damage to the pulmonary or tricuspid valves is usually
secondary to increased pulmonary pressures resulting from left-
sided valvular disease.
Early valvular damage leads to regurgitation.
leaflet thickening, scarring, calcification, and valvular stenosis
may develop
Myocardial inflammation may affect electrical
conduction pathways, leading to P-R interval
prolongation(first-degree atrioventricular block
rarely higher level block) and softening of the first
heart sound.
People with RHD are often asymptomatic for many
years before their valvular disease progresses to
cause cardiac failure.
particularly in resource-poor settings, the diagnosis
of ARF is often not made, so children, adolescents,
and young adults may have RHD but not know it
Joint involvement
The most common form of joint involvement in ARF is arthritis
Objective evidence of inflammation, with hot, swollen, red, and/tender
joints, and involvement of more than one joint(polyarthritis).
Polyarthritis is typically migratory, moving from one joint to another
over a period of hours. ARF almost always affects the large joints
Most commonly the knees, ankles, hips, and elbows and asymmetric.
The pain is severe and usually disabling until anti-inflammatory
Medication is commenced Arthralgia
without objective joint inflammation usually affects large joints in
the same migratory pattern as polyarthritis. In some populations,
aseptic monoarthritis may be a presenting feature of ARF, which may,
in turn, result from early commencement of anti-inflammatory
medication
The joint manifestations of ARF are highly responsive
to salicylates and other nonsteroidal anti
inflammatory drugs (NSAIDs)
joint involvement that persists for more than 1 or 2
days after starting salicylates is unlikely to be due to
ARF.
Chorea
Sydenham’s chorea commonly occurs in the absence of
other manifestations, follows a prolonged latent period
after group A streptococcal infection and is found mainly
in females.
The chorea form movements affect particularly the
head (causing characteristic darting movements of the
tongue) and the upper limbs .They may be generalized
or restricted to one side of the body (hemi-chorea).
In mild cases, chorea may be evident only on careful
examination, whereas in the most severe cases, the
affected individuals are unable to perform activities of
daily living.
Skin manifestation
The classic rash of ARF is erythema marginatum which begins as
pink macules that clear centrally, leaving a serpiginous, spreading
edge.
The rash is evanescent, appearing and disappearing before the
examiner’s eyes. It occurs usually on the trunk, sometimes on the
limbs, but almost never on the face.
Subcutaneous nodules occur as painless, small (0.5–2 cm), mobile
lumps beneath the skin overlying bony prominences, particularly
of the hands, feet, elbows, occiput, and occasionally the vertebrae.
They are a delayed manifestation, appearing 2–3 weeks after the
onset of disease, last for just a few days up to 3 weeks, and are
commonly associated with carditis.
Fever occurs in most cases of ARF, although rarely
in cases of pure chorea.
Although high-grade fever (≥39°C) is the rule,
lower grade temperature elevations are no
uncommon. Elevated acute-phase reactants are also
present in most cases.
group A streptococcal infection is essential in making
the diagnosis of ARF
Because most cases do not have a positive throat swab
culture or rapid antigen test, serologic evidence is
usually needed.
The most common serologic tests are the anti-
streptolysin O (ASO) and anti-DNase B (ADB) titers.
Treatment
All patients with ARF should receive antibiotics
sufficient to treat the precipitating group A
streptococcal infection
Penicillin
Amoxicillin
These may be used for the treatment of arthritis, arthralgia,
and fever, once the diagnosis is confirmed. They are of no
proven value in the treatment of carditis or chorea. Aspirin
is the drug of choice
Naproxen at a dose of 10–20 mg/kg per day is a suitable
alternative to aspirin
Traditional recommendations for long-term bed rest,
once the cornerstone of management.
Instead, bed rest should be prescribed as needed while
arthritis and arthralgia are present and for patients
with heart failure.
Once symptoms are well controlled, gradual
mobilization can commence as tolerated
Prognosis
Untreated, ARF lasts on average 12 weeks.
With treatment, patients are usually discharged from
hospital within 1–2 weeks.
Inflammatory markers should be monitored every 1–
2weeks until they have normalized
(usually within 4–6 weeks)
echocardiogram should be performed after 1 month to
determine if here has been progression of carditis.
Cases with more severe carditis need close clinical and
secondary prophylaxis.
Secondary prophylaxis
