Rheumatic fever is an autoimmune inflammatory disease that may develop after an infection with Streptococcus bacteria (such as strep throat or scarlet fever). The disease can affect the heart, joints, skin, and brain.
Acute rheumatic fever commonly appears in children between the ages of 6 and 15.
The illness is so named because of its similarity in presentation to rheumatism.
Rheumatic fever is common worldwide and is responsible for many cases of damaged heart valves.
Jones Criteria
Jones criteria are guidelines decided on by the American Heart Association to help doctors clinically diagnose rheumatic fever. Two major criteria or one major and two minor plus a history of a streptococcal throat infection are required to make the diagnosis of rheumatic fever.
The major criteria for diagnosis include
- arthritis in several joints (polyarthritis),
- heart inflammation (carditis),
- nodules under the skin (subcutaneous nodules or Aschoff bodies),
- rapid, jerky movements (Sydenham’s chorea), and
- skin rash (erythema marginatum).
The minor criteria include
- fever,
- high ESR (erythrocyte sedimentation rate, an laboratory sign of inflammation),
- joint pain (arthralgia),
- EKG changes, and
- other laboratory findings (elevated C-reactive protein, elevated or rising streptococcal antigen test).
Pathophysiology
Acute rheumatic fever is a sequela of a previous group A streptococcal infection, usually of the upper respiratory tract. This is an autoimmune response secondary to molecular mimicry following group A streptococcal pharyngitis.
The disease involves the heart, joints, central nervous system (CNS), skin, and subcutaneous tissues. It is characterized by an exudative and proliferative inflammatory lesion of the connective tissue, especially that of the heart, joints, blood vessels, and subcutaneous tissue.
Clinical Features
Acute rheumatic fever (ARF) is associated with 2 distinct patterns of presentation. The first pattern of presentation is sudden onset. It typically begins as polyarthritis 2-6 weeks after streptococcal pharyngitis and is usually characterized by fever and toxicity. If the initial abnormality is mild carditis, ARF may be insidious or subclinical.
Age at onset influences the order of complications. Younger children tend to develop carditis first, whereas older patients tend to develop arthritis first.
The common signs and symptoms associated with the disease are :
- Abdominal pain
- Fever
- Heart (cardiac) problems, which may not have symptoms, or may result in shortness of breath and chest pain
- Joint pain, arthritis (mainly in the knees, elbows, ankles, and wrists)
- Joint swelling; redness or warmth
- Nosebleeds
- Skin nodules
- Skin rash (erythema marginatum)
- Skin eruption on the trunk and upper part of the arms or legs
- Eruptions that look ring-shaped or snake-like
- Sydenham chorea (emotional instability, muscle weakness and quick, uncoordinated jerky movements that mainly affect the face, feet, and hands).
Diagnosis
The patient must have a history of an infection with group A streptococcal bacteria, either by laboratory documentation (a positive rapid strep test) or positive strep culture, and must have two major or one major and two minor Jones criteria findings for the diagnosis of rheumatic fever.
Management
Acute rheumatic fever (ARF) is usually preventable if antibiotics are initiated within 9 days of the onset of streptococcal infection.
The management of acute rheumatic fever is in the reduction of inflammation with anti-inflammatory medications such as aspirin or corticosteroids.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 and also monitor for Reye’s syndrome which may occur in children due to aspirin use. The risks, benefits and alternative treatments must always be considered when administering aspirin and aspirin-containing products in children and teenagers. Ibuprofen for pain and discomfort and corticosteroids for moderate to severe inflammatory reactions manifested by rheumatic fever should be considered in children and teenagers. 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.
Use of Antibiotics
Individuals with positive cultures for strep throat should also be treated with antibiotics. Monthly injections of long acting penicillin must be given for a period of five years in patients having one attack of rheumatic fever. If there is evidence of carditis, the length of 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.
Consultations
Because of the many clinical features of acute rheumatic fever (ARF), consider consulting a cardiologist, a rheumatologist, and a neurologist.
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Carditis is not only a major clinical finding, but it also is the cause of much of the disability.
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Arthritis is one of the major manifestations.
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Movement disorders associated with acute rheumatic fever may be difficult to differentiate from those of other clinical problems.
