Rheumatic fever

Rheumatic fever is an inflammatory disease that occurs following a Group A streptococcal infection, (such as strep
throat or scarlet fever). Believed to be caused by antibody cross-reactivity that can involve the heart, joints, skin,
and brain,[1] the illness typically develops two to three weeks after a streptococcal infection. Acute rheumatic fever
commonly appears in children between the ages of 5 and 15, with only 20% of first-time attacks occurring in
adults. The illness is so named because of its similarity in presentation to rheumatism.

Diagnosis

Rheumatic heart disease at autopsy with characteristic findings (thickened mitral valve, thickened chordae

Modified Jones criteria were first published in 1944 by T. Duckett Jones, MD. They have been periodically revised by

the American Heart Association in collaboration with other groups. According to revised Jones criteria, the diagnosis

of rheumatic fever can be made when two of the major criteria, or one major criterion plus two minor criteria, are

present along with evidence of streptococcal infection. Exceptions are chorea and indolent carditis, each of which by

itself can indicate rheumatic fever.

Major criteria

 Migratory polyarthritis: a temporary migrating inflammation of the large joints, usually starting in the legs and

migrating upwards.

 Carditis: 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 over bones or tendons. They commonly

appear on the back of the wrist, the outside elbow, and the front of the knees.

 Erythema marginatum: 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 it 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.
Minor criteria

 Fever

 Arthralgia: Joint pain without swelling

 Raised Erythrocyte sedimentation rate or C reactive protein

 Leukocytosis

 ECG showing features of heart block, such as a prolonged PR interval

 Supporting evidence of Streptococcal infection: elevated or rising Antistreptolysin O titre or DNAase.

 Previous episode of rheumatic fever or inactive heart disease

 Abdominal pain

 Nose bleeds
Pathophysiology

Rheumatic fever is a systemic disease affecting the peri-arteriolar connective tissue and can occur after an untreated

Group A Beta hemolytic streptococcal pharyngeal infection. It is believed to be caused by antibody cross-reactivity.

This cross-reactivity is a Type II hypersensitivity reaction and is termed molecular mimicry. Usually, self reactive B

cells remain anergic in the periphery without T cell co-stimulation. During a Strep. infection, mature antigen

presenting cells such as B cells present the bacterial antigen to CD4-T cells which differentiate into helper T2 cells.

Helper T2 cells subsequently activate the B cells to become plasma cells and induce the production of antibodies

against the cell wall of Streptococcus. However the antibodies may also react against the myocardium and joints[9],

producing the symptoms of rheumatic fever.

Group A streptococcus pyogenes has a cell wall composed of branched polymers which sometimes contain M

protein that are highly antigenic. The antibodies which the immune system generates against the M protein may cross

react with cardiac myofiber protein myosin,heart muscle glycogen and smooth muscle cells of arteries,

inducing cytokine release and tissue destruction. However, the only proven cross reaction is with

perivascular connective tissue.This inflammation occurs through direct attachment of complement and Fc receptor-

mediated recruitment of neutrophils and macrophages. Characteristic Aschoff bodies, composed of swollen

eosinophilic collagen surrounded by lymphocytes and macrophages can be seen on light microscopy. The larger
macrophages may become Aschoff giant cells. Acute rheumatic valvular lesions may also involve a cell-mediated

immunity reaction as these lesions predominantly contain T-helper cells and macrophages

In acute RF, these lesions can be found in any layer of the heart and is hence called pancarditis. The inflammation

may cause a serofibrinous pericardial exudates described as “bread-and-butter”pericarditis, which usually resolves

without sequelae. Involvement of the endocardium typically results in fibrinoid necrosis and verrucae formation along

the lines of closure of the left-sided heart valves. Warty projections arise from the deposition, while subendothelial

lesions may induce irregular thickenings called MacCallum plaques.

Chronic rheumatic heart disease is characterized by repeated inflammation with fibrinous resolution. The cardinal

anatomic changes of the valve include leaflet thickening, commissural fusion and shortening and thickening of the

tendinous cords.

Treatment

The management of acute rheumatic fever is geared toward the reduction of inflammation with anti-inflammatory

medications such as aspirin or corticosteroids. Individuals with positive cultures for strep throat should also be treated

with antibiotics. Aspirin is the drug of choice and should be given at high doses of 100 mg/kg/day. One should watch

for side effects like gastritis, salicylate poisoning etc. 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. Monthly injections of Longacting Penicillin must be given for a period of 5 years in

patients having one attack of Rheumatic fever. If there is evidence of carditis, the length of Penidure therapy may be

up to 40 years. Another important cornerstone in treating rheumatic fever includes the continual use of low dose

antibiotics (such as penicillin, sulfadiazine, or erythromycin) to prevent recurrence.

Infection

Patients with positive cultures for Streptococcus pyogenes should be treated with penicillin as long as allergy is not

present. This treatment will not alter the course of the acute disease.

The most appropriate treatment stated in Oxford Handbook of Clinical Medicine for Rheumatic fever is

Benzylpenicillin.

Inflammation

Patients with significant symptoms may require corticosteroids. Salicylates are useful for pain.

Heart failure

Some patients develop significant carditis which manifests as congestive heart failure. This requires the usual

treatment for heart failure: diuretics and digoxin. Unlike normal heart failure, rheumatic heart failure responds well to

corticosteroids.
Prevention

Prevention of recurrence is achieved by eradicating the acute infection and prophylaxis with antibiotics. The American

Heart Association recommends daily or monthly prophylaxis continue long-term, perhaps for life.

Screening school-aged children for sore throats also aid in prevention.

Epidemiology

Rheumatic fever is common worldwide and responsible for many cases of damaged heart valves. In Western

countries, it became fairly rare since the 1960s, probably due to widespread use of antibiotics to

treat streptococcus infections. While it is far less common in the United States since the beginning of the 20th

century, there have been a few outbreaks since the 1980s. Although the disease seldom occurs, it is serious and has

a mortality of 2–5%.

Rheumatic fever primarily affects children between ages 5 and 15 years and occurs approximately 20 days after strep

throat or scarlet fever. In up to a third of cases, the underlying strep infection may not have caused any symptoms.

The rate of development of rheumatic fever in individuals with untreated strep 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. Persons who have suffered a case of

rheumatic fever have a tendency to develop flare-ups with repeated strep infections.

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.

Survivors of Rheumatic fever often have to take penicillin to prevent streptococcal infection which could possibly lead

to another case of Rheumatic fever that could prove fatal.

What are rheumatic heart disease and rheumatic fever?

Rheumatic (roo-MAT'ik) heart disease is a condition in which the heart valves are damaged by rheumatic fever.

Rheumatic fever begins with a strep throat (also called strep pharyngitis). Strep throat is caused by Group
A Streptococcusbacteria. It is the most common bacterial infection of the throat.

Rheumatic fever is an inflammatory disease. It can affect many of the body's connective tissues — especially those
of the heart, joints, brain or skin. Anyone can get acute rheumatic fever, but it usually occurs in children five to 15
years old. The rheumatic heart disease that results can last for life.

The incidence of rheumatic fever/rheumatic heart disease is low in theUnited States and most other developed
countries. However, it continues to be the leading cause of cardiovascular death during the first five decades of life in
the developing world.

What are the symptoms of strep throat?

Symptoms include (but are not limited to):

 sudden onset of sore throat

In some people, strep throat is very mild with just a few symptoms. Also, sore throats are caused more often by
viruses than by a strep infection. Viral throat infections don’t raise the risk of rheumatic fever and are not treatable
with antibiotics.

What are the symptoms of rheumatic fever?

Symptoms may include:

 fever
 painful, tender, red swollen joints
 pain in one joint that migrates to another one
 heart palpitations
 chest pain 
 shortness of breath
 skin rashes
 fatigue
 small, painless nodules under the skin 

The symptoms of rheumatic fever usually appear about three weeks after the strep throat.

How can I prevent rheumatic heart disease?

The best defense against rheumatic heart disease is to prevent rheumatic fever from ever occurring. By treating strep
throat with penicillin or other antibiotics, doctors can usually stop acute rheumatic fever from developing.
People who've already had rheumatic fever are more susceptible to recurrent attacks and heart damage. That's why
they're given continuous monthly or daily antibiotic treatment, maybe for life. If their heart has been damaged by
rheumatic fever, they're also at increased risk for developing infective endocarditis (also known as bacterial
endocarditis), an infection of the heart's lining or valves.

In 2007, the American Heart Association updated its guidelines for prevention of endocarditis and concluded that
there is no convincing evidence linking dental, gastrointestinal or genitourinary tract procedures with the development
of endocarditis. The prophylactic use of antibiotics prior to a dental procedure is now recommended ONLY for those
patients with the highest risk of adverse outcome resulting from endocarditis, such as patients with a prosthetic
cardiac valve, previous endocarditis, or those with specific forms of congenital heart disease. The guidelines no
longer recommend prophylaxis prior to a dental procedure for patients with rheumatic heart disease unless they also
have one of the underlying cardiac conditions listed above. 

Antibiotic prophylaxis solely to prevent endocarditis is no longer recommended for patients who undergo a
gastrointestinal or genitourinary tract procedure.