ACUTE RHEUMATIC FEVER

Dr Romila Chimoriya
Lecturer
Department of Pediatrics
INTRODUCTION
 Rheumatic fever is a immunological disorder that may occur following group A beta
hemolytic streptococcal  pharyngitis in children

 Antibodies produced against selected streptococcal cell wall proteins and sugar react
with connective tissues of body as well as heart and result in rheumatic fever.

 The annual incidence of acute rheumatic fever in some developing countries exceeds 50
per 100,000 children, and very high rates are also seen in ethnic minority populations
within Australia and New Zealand

 In developed countries, the incidence of ARF is much lower probably due to improved
hygienic standards and routine use of antibiotics for acute pharyngitis
EPIDEMIOLOGY
 Worldwide, rheumatic heart disease remains the most common form of
acquired heart disease in all age-groups, accounting for up to 50% of all
cardiovascular disease and 50% of all cardiac admissions in many
developing countries.

 Rheumatic fever-5-15 years ,first episode rare before 3 years or after 30

years of age

 Mitral valve disease and chorea-females

 Aortic valve disease-males

 ARF can present with several different clinical findings within
2-4 weeks of a group A streptococcal (GAS) tonsillopharyngitis
 In the aboriginal population of Australia where RF is endemic,
GAS impetigo is common but GAS pharyngitis is uncommon
 Results in a generalised inflammatory response affecting brain,
joints, skin, subcutaneous tissues and the heart

 Group A beta hemolytic streptococcus: M types 1, 3, 5, 6, 18, 29

 Other predisposing factor:

 Family history of rheumatic fever
 Low socio-economic status
 Overcrowding
PATHOGENESIS
 The pathogenic link between a GAS infection of the
upper respiratory tract and an attack of acute rheumatic
fever, characterized by organ and tissue involvement , is
still not clear

 Several theories of the pathogenesis of acute rheumatic

fever and rheumatic heart disease have been proposed

 Two theory are seriously considered: the cytotoxic

theory and the immunologic theory
CYTOTOXIC THEORY
 The cytotoxic theory suggests that a GAS toxin may be
involved in the pathogenesis of acute rheumatic fever and
rheumatic heart disease

 GAS produces several enzymes that are cytotoxic for

mammalian cardiac cells, such as streptolysin O, which
has a direct cytotoxic effect on mammalian cells in tissue
culture

 One of the major problems with the cytotoxic hypothesis

is its inability to explain the latent period between GAS
pharyngitis and the onset of acute rheumatic fever
IMMUNOLOGIC THEORY
 An immune-mediated pathogenesis :The antigenicity of a large variety of GAS products
and constituents and the immunologic cross reactivity between GAS components and
mammalian tissues also lends support to this hypothesis

 Antibody cross-reactivity

 Type II hypersensitivity reaction and is termed molecular mimicry

 Common antigenic determinants are shared between certain components of GAS (M

protein, protoplast membrane, cell wall group A carbohydrate, capsular hyaluronate) and
specific mammalian tissues (e.g., heart, brain, joint)

 For example, certain M proteins (M1, M5, M6, and M19) share epitopes with human
tropomyosin and myosin.

 Additionally, the involvement of GAS superantigens such as pyrogenic exotoxins in the

pathogenesis of acute rheumatic fever has been proposed
CLINICAL FEATURES
POLYARTHRITIS
 Arthritis - 70% of patients
 involves larger joints, particularly
the knees, ankles, wrists, and elbows

 Migratory, rarely involving a single joint for more than 2 days

 Dramatic response to even small doses of salicylates

 Arthritis is the earliest manifestation of acute rheumatic fever and may

correlate temporally with peak antistreptococcal antibody titers

 An inverse relationship between the severity of arthritis and the severity of

cardiac involvement
 Arthralgia

-joint pain without objective signs of inflammation

-common in rheumatic recurrences and patients with RHD

in developing countries
CARDITIS

Carditis -50-60%

Rheumatic carditis is characterized by pancarditis, with active

inflammation of myocardium, pericardium, and endocardium.

Endocarditis (valvulitis) is a universal finding in rheumatic

carditis, whereas the presence of pericarditis or myocarditis is
variable

Myocarditis: Tachycardia out of proportion to the degree of

fever

Pericarditis: friction rub, effusion, precordial chest pain

 Signs of CHF in case of severe carditis

 Echocardiographic findings include pericardial effusion, decreased

ventricular contractility, and aortic and/or mitral regurgitation

 Cardiomegaly on x-ray chest indicative of severity of rheumatic carditis or

CHF

 The major consequence of acute rheumatic carditis is chronic, progressive

valvular disease, particularly valvular stenosis, which can require valve
replacement
 ERYTHEMA MARGINATUM
 Erythema marginatum is a rare (<3% of patients) but
characteristic rash of acute rheumatic fever

 Erythematous, serpiginous, macular lesions with pale

centers that are not pruritic

 Site: trunk, inner proximal portion of the extremities

 Never on face

 Evanescent: disappears on cold exposure and reappears

with heat
 SUBCUTANEOUS NODULE
 <1%
 Hard, painless, non pruritic, freely movable swelling
and 0.2 to 2 cm in diameter

 Symmetrical

 Occur over body prominences- usually over the

extensor tendons of the hands, feet, elbows, scalp,
scapulae, and vertebrae

 Never occur as a sole manifestation

 Significant association with carditis

CHOREIC MOVEMENT
 Spontaneous purposeless movement
 10-15% of patients with acute rheumatic fever

 Isolated, frequently subtle, movement disorder

 More common in girls

 Auto-antibodies reacting with brain ganglioside

 Emotional lability, incoordination, poor school performance, uncontrollable movements, and

facial grimacing, all exacerbated by stress and disappearing with sleep, are characteristic
 Lasts for 4 to 18 month (average 7 months)

 Maneuvers to elicit features of chorea:

o Milkmaid's grip (irregular contractions of the muscles of the hands while squeezing the
examiner's fingers)
o Spooning and pronation of the hands when the patient's arms are extended
o Wormian darting movements of the tongue upon protrusion
o Examination of handwriting to evaluate fine motor movements

Rarely, leads to permanent neurologic sequelae

DIAGNOSIS
 2 Major manifestations plus Essential criteria

 1 major + 2 minor plus Essential criteria

 Exceptions to the Jones’s criteria

 Chorea may occur as a only manifestation of rheumatic fever.

 Indolent carditis may be the only manifestation in patients who come to

medical attention months after the onset of rheumatic fever

 Patient with rheumatic fever recurrence

REVISED JONES CRITERIA 2015 BY
AHA
 Low-Risk population -ARF incidence <2 per 100,000 school-age children
per year, or all-age RHD prevalence of <1 per 1000 population.

 Moderate/High-Risk population -ARF incidence >2 per 100,000 school-age

children per year, or all-age RHD prevalence of >1 per 1000 population.

 Initial attack: 2 major manifestations, or 1 major and 2 minor

manifestations, plus evidence of recent GAS infection.

 Recurrent attack: 2 major, or 1 major and 2 minor, or 3 minor

manifestations (the latter only in the Moderate/High-Risk population), plus
evidence of recent GAS infection .
REVISED JONES CRITERIA
TREATMENT
Bed rest
ANTIBIOTIC THERAPY:

 Intramuscular injection of benzathine penicillin single dose

-1.2 million unit(>27 kg)
-0.6 million unit(<27 kg)

 Oral penicillin V (250mg four times a day)-10 days

 Erythromycin (250mg four times a day )-10 days

 If penicillin: allergic, erythromycin

 After this initial course of antibiotic therapy, long-term antibiotic

prophylaxis should be instituted.
TREATMENT
Anti-inflammatory

 Patients with typical migratory polyarthritis and those with carditis without
cardiomegaly or congestive heart failure should be treated with oral
salicylates

 The usual dose of aspirin is -100 mg/kg/day in 4 divided doses PO for 2-

3weeks, followed by 60-70 mg/kg/day in 4 divided doses PO on symptom
resolution

 Total duration-12 weeks

 Naproxen in aspirin sensitive(10-20mg/kg/day)

TREATMENT
 Patients with carditis and more than minimal cardiomegaly and/or
congestive heart failure should receive corticosteroids

 The usual dose of prednisone is 2 mg/kg/day in 4 divided doses till ESR

normalises usually 2 weeks then taper over 2-4 weeks reduce dose by 2.5-
5mg every 3rd day

 When prednisone is being tapered, aspirin should be started at 50

mg/kg/day in 4 divided doses for 6 wk to prevent rebound of inflammation

 Supportive therapies for patients with moderate to severe carditis : digoxin,

fluid and salt restriction, diuretics, and oxygen
SYDENHAM CHOREA
 Sedatives may be helpful early in the course of chorea:
 Phenobarbital (16-32 mg every 6-8 hr PO) is the drug of
choice
 If phenobarbital is ineffective, then haloperidol (0.01-
0.03 mg/ kg/24 hr divided bid PO) or chlorpromazine
(0.5 mg/kg every 4-6 hr PO) should be initiated
 Some patients may benefit from a few week course of
corticosteroids
PROGNOSIS
 70% of patients with carditis during the initial episode of acute rheumatic
fever recover with no residual heart disease; contrast, patients with carditis
during the initial episode are likely to have carditis with recurrences, and
the risk for permanent heart damage increases with each recurrence

 Before antibiotic prophylaxis was available, 75% of patients who had an

initial episode of acute rheumatic fever had one or more recurrences during
their lifetimes

 20% of patients who present with “pure” chorea who are not given
secondary prophylaxis develop rheumatic heart disease within 20 yr.
Therefore, patients with chorea, even in the absence of other manifestations
of rheumatic fever, require long-term antibiotic prophylaxis
PRIMARY PREVENTION
 Prevention of initial attacks (primary prevention)
depends on identification and eradication of GAS
causing acute pharyngitis

 Appropriate antibiotic therapy instituted before the 9th

day of symptoms of acute GAS pharyngitis is highly
effective in preventing first attacks of acute rheumatic
fever
SECONDARY TREATMENT
Secondary prevention is directed at preventing acute GAS
pharyngitis in patients at substantial risk of recurrent acute
rheumatic fever