Rheumatic fever is an inflammatory disease that may develop 2 - 3 weeks after a Group
‘A’ streptococcal infection (such as strep throat or scarlet fever).
It is believed to be caused by antibody cross-reactivity and can involve the heart, joints,
skin, and brain.
Epidemiology
Acute rheumatic fever commonly appears in children ages 5 through 15 years, with only
20% of first time attacks occurring in adults.
Rheumatic fever is common worldwide and responsible for many cases of damaged heart
valves.
The rate of development of rheumatic fever in individuals with untreated streptococcal
infection is estimated to be 3%.
The incidence of recurrence with a subsequent untreated infection is substantially greater
(about 50%). The rate of development is far lower in individuals who have received
antibiotic treatment.
The recurrence of rheumatic fever is relatively common in the absence of maintenance of
low dose antibiotics, especially during the first three to five years after the first episode
Heart complications may be long-term and severe, particularly if valves are involved.
Pathology
• Rheumatic fever is a systemic disease affecting the peri-arteriolar connective tissue and
can occur after an untreated Group A streptococcal pharyngeal infection.
• It is believed to be caused by antibody cross-reactivity which is a Type II hypersensitivity
reaction.
Clinical Features
• Rheumatic fever is a systematic illness presenting with
Painful swollen joints
Chest pain
Fatigue
Shortness of breath
Faint pink or red rash
Jerky body movements (Chorea)
Fever
Arthritis in about 75% of all patients
• Other features includes
Skin rashes
Carditis
Neurological features
Diagnosis and Complications of Rheumatic Fever
Diagnosis
• Clinical diagnosis of rheumatic fever can be done using modified Jones criteria which
consists of major and minor criteria as follows.
Two major criteria, or one major and two minor criteria, when there is also evidence
of a previous streptococcal infection, support the diagnosis of rheumatic fever.
Exceptions are chorea and carditis, each of which by itself can indicate rheumatic
fever.
• The major criteria used are
Joints (Migratory Polyarthritis)
Temporary migrating inflammation of the large joints
Usually starting in the legs and migrating upwards
-Carditis (in 50 – 60% of cases)
Inflammation of the heart muscle which can manifest as congestive heart failure
with shortness of breath, pericarditis with a rub, or a new heart murmur.
-Subcutaneous nodules
Painless, firm collections of collagen fibers on the back of the wrist, the outside
elbow, and the front of the knees.
These now occur infrequently.
-Erythema Marginatum
A long lasting rash that begins on the trunk or arms as macules and spreads
outward to form a snakelike ring while clearing in the middle.
This rash never starts on the face and is made worse with heat.
-Sydenham's chorea (St. Vitus' dance)
A characteristic series of rapid movements without purpose of the face and arms
This can occur very late in the disease.
• The minor Criteria include
Fever
Arthralgia: Joint pain without swelling
Laboratory abnormalities: increased erythrocyte sedimentation rate & leukocytosis
Electrocardiogram abnormalities: a prolonged PR interval
Evidence of Group ‘A’ Streptococcal infection: elevated or rising Antistreptolysin O
titre
Previous rheumatic fever or inactive heart disease
Complications
• The main complication of rheumatic fever is rheumatic heart disease as described below.
During a first rheumatic fever attack, about half of people develop heart
inflammation, but this doesn't always result in permanent damage.
Most people with rheumatic fever recover fully after six weeks.
In some cases however, one or more of the heart's valves may be scarred.
Permanent heart damage due to rheumatic fever is known as rheumatic heart disease
In many cases, heart damage is not discovered until years later.
In rare cases of rheumatic fever, the inflammation causes so much damage to the heart
muscle that it leads to heart failure. If there's serious impairment to the function of
affected heart valves, surgery may be needed to repair or replace the damaged valve
or valves.
• Other complications include
Joints in 60 –75% of cases
Often, several joints are affected with painful swelling, redness and sensation of
heat.
These affect large joints usually symmetrical starting from lower limbs moving
upward.
-Brain
If acute rheumatic fever affects the brain, loss of coordination and uncontrolled
movement of the limbs and face may occur.
These movements are called chorea - from the Greek word "choreia," which
means ‘choral dance.’
They are also called sydenham's chorea, rheumatic chorea or St. Vitus' dance.
Chorea occurs in about one in 10 rheumatic fever cases.
Chorea usually subsides or disappears within weeks to months.
Skin
Subcutaneous nodules - a form of aschoff bodies: painless, firm collections of
collagen fibers on the back of the wrist, the outside elbow, and the front of the
knees.
These now occur infrequently.
Erythema marginatum in < 5% of cases
A long lasting rash that begins on the trunk or arms as macules and spreads
outward to form a snake-like ring while clearing in the middle.
This rash never starts on the face and is made worse with heat.
Treatment and Prevention of Rheumatic Fever
• Medical therapy is directed toward eliminating the group A streptococcal pharingitis
(if still present), suppressing inflammation from the autoimmune response and providing
supportive treatment for congestive heart failure.
• Patients with features of rheumatic fever should be referred to hospitals for specialized
investigations and treatment. However, in primary health facility (dispensary and health
centres) pre-referral management may be done. This includes the following
Relieving fever and pain by offering Paracetamol and or NSAID- e.g. Asprin
Bed rest
Prophylactic penicillin
• The management of acute inflammatory manifestation of rheumatic fever is geared
toward the reduction of inflammation with anti-inflammatory medications such as
aspirin or corticosteroids.
• Aspirin is the drug of choice and this will usually relieve the symptoms of arthritis
rapidly and a prompt response (within 24 hours) helps to confirm the diagnosis
Reasonable starting dose is 60 mg/kg per day, divided into six doses.
In adults, 100 mg/kg per day may be needed up to the limits of tolerance or a
maximum of 8g per day.
Aspirin should be continued until the ESR has fallen and then gradually tailed off.
• Individuals with positive cultures for Streptococci should also be treated with antibiotics.
• Steroids are reserved for cases where there is evidence of involvement of heart.
• The use of steroids may prevent further scarring of tissue and may prevent development
of sequelae such as Mitral stenosis.
Prevention
• Prevention of recurrence is achieved by eradicating the acute infection and prophylaxis
with antibiotics, also early detection and treatment of sore throat and skin infection.
• Monthly injections of long acting Penicillin must be given for a period of 5 years after the
last attack or 18 years of age (whichever is longer) in patients with rheumatic fever
without proven carditis.
Usually Benzathine penicillin at a dose of 1.2 million units every 4 weeks
If there is evidence of carditis, the length of Benzathine penicillin may be up to 25
years or 10 years after the last attack (whichever is longer)                                             
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