Rheumatic Fever

Etiology.

Group A b-hemolytic Streptococcus is the agent causing acute rheumatic fever,

serotypes of group A streptococci (e.g., M types 1, 3, 5, 6, 18, 24) are isolated from
patients with acute rheumatic fever

Epidemiology.

A sequelae of group A streptococcal pharyngitis. Rheumatic fever is observed in the age

group susceptible to group A streptococcal infections, from 5–15 yr of age. Over
crowding, is frequent in certain group of population.
streptococcal skin infection does not result in acute rheumatic fever, but infection of the
upper respiratory tract or the skin may lead to another non-suppurative complication of
streptococcal infection, acute post streptococcal glomerulonephritis. Difference in
Rheumatogenic potential of “skin strains” and “throat strains,” can explain the
phenomenon.

Untreated or inadequately treated infection, leads to rheumatic fever. Carriers are at

reduced risk for development of acute rheumatic fever and they cause spread of the
organism to close family or school contacts.

M types 1, 3, 5, 6, and 18. are associated with rheumatic fever.

Pathogenesis.

Two theories are

1. a toxic effect produced by an extracellular toxin of group A streptococci on target
organs such as myocardium, valves, synovium, and brain;
2. an abnormal immune response by the human host.

Antibodies cause the immunologic damage. The latent period, 1–3 wk between the onset
of the actual group A streptococcal infection and the onset of symptoms of acute
rheumatic fever, supports immunologic mechanism
The M protein is responsible for the organism's ability to resist phagocytosis. M protein
shares amino acid sequences with some human tissues.
In Sydenham’s chorea, common antibodies to antigens are found in the group A
streptococcal cell membrane and the caudate nucleus of the brain.

Clinical manifestations. Modified Jone’s Criteia

Carditis.- mild or severe carditis, leading to heart failure

Pancarditis involves the pericardium, epicardium, myocardium, and endocardium

Carditis results in chronic changes. Valvular insufficiency, most frequently affecting
mitral and the aortic valve .. Isolated involvement of the aortic valve is rare. in chronic
stage, scarring of the valve orcalcified valve tissue may lead to stenosis. A combination
of insufficiency and stenosis is found.
pericarditis, pericardial effusion, and arrhythmias (usually first-degree heart block, third-
degree or complete heart block may occur).
Polyarthritis.

The arthritis of is tender. Refuse even bed sheets or clothing to cover an affected joint.
The joints are red, warm, and swollen. Migratory and affects several joints: the elbows,
knees, ankles, and wrists. Rare in the fingers, toes, or spine. Effusions may be present.
Aspirate = polymorphonuclear leukocytosis is found - no specific laboratory findings in
the synovial fluid.

The arthritis does not result in chronic joint disease. After anti-inflammatory therapy is
begun, the arthritis may disappear in 12–24 hr. Untreated, it may persist for a week or
more. Because of treatment with anti-inflammatory drugs, the migratory nature does not
develop.

Chorea

Sydenham chorea, occurs much later than other manifestations. Choreoathetoid

movements may begin insidiously. The period following pharyngitis may be several
months, and the movements are often very difficult to detect at the onset. Deterioration in
their handwriting. Emotional lability is a frequent finding. Sydenham chorea may affect
all four extremities or may be unilateral. frequently it is the only symptom of rheumatic
fever. It usually disappears within weeks to months. It may recur. Pronator sign, Bishop’s
sign, milk maid sign, hung up reflex, poor handwriting, no abnormal movement in sleep.

Erythema Marginatum

Major manifestation, very difficult to diagnose. Nonspecific pink macules that are seen
over the trunk, later in its fully developed form, blanching occurs in the middle of the
lesions, sometimes with fusing of the borders, resulting in a serpiginous-looking lesion.
This rash can be made worse with application of heat, but characteristically it is transient
– that is disappears in a few hours. The rash does not itch. It often occurs in patients with
chronic carditis. The rash of erythema marginatum can be mistaken for the rash seen with
Lyme disease.

Subcutaneous Nodules

observed in patients with severe carditis. pea-sized nodules are firm and nontender, and
there is no inflammation. They are seen on the extensor surfaces of the joints, such as the
knees and elbows, and over the spine.

Minor Manifestations.

The minor manifestations fever and arthralgia.

Arthralgia is present if a patient feels discomfort in a joint in the absence of
inflammation (e.g., pain, redness, warmth) on physical examination.
 Fever, usually < 101–102°F. If >103–104°F requires re-evaluation -consider other
diagnoses.

Diagnosis

Jones criteria, used to determine the diagnosis of acute rheumatic fever.

five major criteria
Sydenham chorea is the only symptom of rheumatic fever, - this symptom alone is
adequate to satisfy the Jones criteria.
minor criteria are symptoms and laboratory tests.
Arthralgia cannot be counted as a minor manifestation if arthritis is used as a major
manifestation.
Levels of acute-phase reactants, such as the ESR or CRP, may be elevated.
prolonged PR interval on the ECG
Echocardiogram is useful in evaluating rheumatic heart disease.

Evidence of Group A Streptococcal Infection

a positive throat culture

a history of scarlet fever
elevated streptococcal antibodies such as ASO, ADB, or AH.
diagnosis of rheumatic fever should not be considered in patients if there is no evidence
of a recent group A streptococcal infection except for chorea and certain carditis.
In three situations, acute rheumatic fever may be diagnosed even in the absence of two
major criteria or one major and two minor criteria, Rheumatic fever should be considered
if
1 chorea with no other likely cause
2 indolent carditis with no other likely cause.
3 recurrence of rheumatic fever with prior rheumatic fever or rheumatic heart
disease and with evidence of a recent streptococcal infection with one major
or two minor criteria.

Laboratory Findings
throat culture –
rapid antigen detection tests are available in USA. a positive result of a rapid antigen
detection test provides evidence of group A streptococci. If a rapid antigen detection test
result is negative, a throat culture should be obtained in patients in whom rheumatic fever
is suspected.

Streptococcal antibody tests - ASO test. Other tests - ADB test and the AH test.
Elevated ASO is clear evidence of a previous group A streptococcal infection, -a rise in
titer between acute and convalescent sera. The ASO test result reaches its peak 3–6 wk
after infection, whereas the ADB test result reaches its peak slightly later (6–8 wk
Acute-phase reactants ESR or CRP are elevated at the onset of acute rheumatic fever.
These tests are nonspecific
ECG = a first-degree heart block (prolonged PR interval), second- or third-degree block
In chronic rheumatic heart disease, ECG change due to valve lesion like left atrial
enlargement, may be evident.

chest x ray = cardiomegaly in carditis.

Echocardiography - valvular regurgitation
Differential Diagnosis

Juvenile rheumatoid arthritis

connective tissue diseases
Infective endocarditis
rash of Lyme disease may be confused with erythema marginatum.

Complications

development of rheumatic valvular heart disease.

mitral valve is most frequently involved,
the aortic and tricuspid valves also may be affected.
The tricuspid valve becomes involved in pulmonary hypertension.

Treatment

Four points- :
1 treatment of the group A streptococcal infection
2 use of anti-inflammatory agents to control the clinical manifestations of the
disease
3 supportive therapy, including management of congestive heart failure
4 Prevention

Ten days of an oral agent or a single intramuscular injection of 1,200,000 units of

benzathine penicillin G intramuscular benzathine penicillin G may cause rises in the
ESR, treat patients initially with oral penicillin, especially if you are monitoring the ESR
as a measure of the effectiveness of anti-inflammatory therapy

Three systemic manifestations of acute rheumatic fever are arthritis, carditis, and
Sydenham’s chorea.
Salicylates relief for the arthritis
Tender migratory polyarthritis can be relieved in 12–24 hr by the use of salicylates
Salicylates or other anti-inflammatory agents should be withheld until diagnosis is
definite. In painful arthritis the use of small doses of codeine Corticosteroids are not
indicated for arthritis
mild carditis without congestive heart failure, salicylates are indicated.
in congestive heart failure corticosteroids are required. The use of salicylates or
corticosteroids does not prevent future development of rheumatic heart disease.
total dose of 2.5 mg/kg/24 hr of prednisone divided into two doses
A short course of corticosteroids over 2–3 wk is sufficient,
Monitor clinically and on laboratory tests (e.g., ESR, CRP).
side effects occur, including some cushingoid changes and hypertension.
Studies on alternate-day steroids have not been carried out.
The dose should be tapered -NOT stopped abruptly.

90–120 mg/kg/24 hr in four divided doses

serum salicylate levels may be monitored to reduce the possibility of toxicity.
Liver function be monitored.
add salicylates to the corticosteroids, when the dose of steroid tapered, to prevent the
possibility of rheumatic rebound.
The salicylates should be given during the last week of corticosteroid therapy and
continued for 4 wk after the steroids have been discontinued.
The duration of salicylate therapy depends on response and clinical course.

Congestive heart failure - Diuretics in severe congestive heart failure.

digitalis may be used, although usually in small doses.
bed rest -Strict bed rest is not needed. Bed rest is indicated for - - carditis and congestive
heart failure, but prolonged bed rest is unnecessary.
keep patients at bed rest until the ESR approaches normal and congestive heart failure has
been controlled.
The treatment of Sydenham chorea –Diazepam is prescribed for patients with mild
chorea. In patients severe chorea, haloperidol -severe toxic reactions to this drug have
been reported. Sodium valproate is nowadays used

There is no specific therapy for Erythema Marginatum or the subcutaneous nodules of

acute rheumatic fever.

Prevention

primary prophylaxis
secondary prophylaxis.

Primary prophylaxis refers to antibiotic treatment of the streptococcal upper respiratory

tract infection to prevent an initial attack of rheumatic fever. Antibiotic therapy up to 1
wk after onset of the streptococcal sore throat can prevent rheumatic fever. Ten full days
of oral therapy is essential if the oral method is used.

Secondary prophylaxis refers to the prevention of colonization or infection of the upper

respiratory tract with group A b-hemolytic streptococci in individuals who have already
had a previous attack of acute rheumatic fever. Patients who receive antibiotics
continuously and who do not have group A streptococcal infections do not have
recurrences of rheumatic fever. The recommended methods of secondary prevention =
regular
monthly injections of intramuscular benzathine penicillin G,
daily administration of oral penicillin,
daily administration of oral sulfadiazine,
daily oral administration of erythromycin (for individuals who cannot take any of
the previously recommended antibiotics).
sulfadiazine is effective in preventing colonization of the upper respiratory tract
 Regular injections of intramuscular benzathine penicillin G - better compliance.
Individuals at high risk for rheumatic recurrence should be given 1,200,000 units IM
every 3 wk
duration of secondary prophylaxis upto 5 yr after the most recent attack or when they
reach their 21st birthday, whichever comes last. Others recommend that for patients who
have significant rheumatic heart disease or who have a significant risk of contracting
group A streptococcal upper respiratory tract infection (e.g., medical professionals,
school teachers, those living in crowded conditions), the duration of secondary
prophylaxis should be longer. Some recommend that treatment be continued for at least
10 yr in patients with residual rheumatic valvular heart disease and at least until the age
of 40 yr (others recommend lifelong prophylaxis). Recommendations for each patient
must be individualized, depending on a patient's condition and the living and working
environment.

No streptococcal vaccine is available. Physicians and public health authorities must still
depend on the accurate and timely diagnosis and therapy of group A streptococcal upper
respiratory tract infections and avoidance of recurrent infections in known rheumatic
patients to prevent the crippling effects of rheumatic fever and rheumatic heart disease.

Acute Rheumatic Fever.

Pericarditis occurs in acute rheumatic fever as a component of pancarditis (see Chapters

184.1 and Chapters 444). It is associated with acute valvulitis. Pericarditis and other
manifestations of acute rheumatic pancarditis respond to therapy with steroids. Cardiac
tamponade is extremely rare.