Key points
- Acute rheumatic fever is a delayed sequela of some group A streptococcal infections.
- It affects many organ systems and has a varied clinical presentation.
- One manifestation, carditis, may result in long-term disability or death.
- Treatment involves antibiotics, which help prevent recurrent episodes.
Overview
Cause
Acute rheumatic fever is a delayed sequela of pharyngitis and skin infections, such as impetigo, due to Streptococcus pyogenes. These bacteria are also called group A Streptococcus (group A strep bacteria).
Streptococcal pharyngitis or skin infection typically precedes the onset of acute rheumatic fever by 1 to 5 weeks.
The exact disease process isn't fully known. However, the disease is in part due to an autoimmune response to S. pyogenes infection involving multiple organ systems.
Manifestations by organ system
Organ systems involved commonly include the
- Cardiovascular system
- Central nervous system
- Integumentary system
- Musculoskeletal system
Cardiovascular system
Carditis is the major cardiac manifestation of acute rheumatic fever. It occurs in 50% to 70% of first episodes and is associated with valvulitis.
Central nervous system
Chorea, also called Sydenham's chorea or St. Vitus dance, is the major central nervous system manifestation. Chorea often appears after the other manifestations of acute rheumatic fever. It also can appear as the only manifestation of acute rheumatic fever.
Integumentary system
Subcutaneous nodules and erythema marginatum are the two major skin manifestations. Nodules are most commonly present in patients with carditis.
Musculoskeletal system
Polyarthritis is the major musculoskeletal manifestation.
Risk factors
Certain factors increase the risk of someone developing acute rheumatic fever.
The incidence of acute rheumatic fever is highest in children between the ages of 5 and 15 years. Acute rheumatic fever is rare in children 3 years of age and younger in the United States.
First-onset acute rheumatic fever is rare in adults, although recurrence may occur through adulthood.
Crowding increases the risk of spreading group A strep bacteria and thus increases the risk of developing acute rheumatic fever. These settings include:
- Daycare centers and schools
- Detention or correctional facilities
- Homeless shelters
- Military training facilities
Individuals with a history of acute rheumatic fever have an increased risk of recurrence with subsequent streptococcal infections. The risk of recurrence after streptococcal infection is highest within the first few years after the initial attack and then declines.
In approximately one-third of patients, acute rheumatic fever follows subclinical streptococcal infections or infections for which medical attention was not sought.
Clinical features
Clinical features
Acute rheumatic fever is usually characterized by fever.
Other symptoms depend on the affected organ systems.
Carditis
Clinical signs of carditis include
- Cardiomegaly
- Congestive heart failure
- New heart murmur (usually with mitral or aortic valvular disease)
- Pericardial effusion
- Pericardial friction rub
Additionally, a prolonged PR interval can be seen on electrocardiography.
Subclinical carditis may also be present. In these cases, classic murmurs may not be appreciated on physical exam. Valve disease is found solely by echocardiography/Doppler studies.
Chorea
Chorea is a neurological disorder characterized by abrupt, purposeless, non-rhythmic, involuntary movements. It's often associated with muscle weakness and emotional lability.
Erythema marginatum
Erythema marginatum is an erythematous, non-pruritic, non-painful macular lesion on the trunk or proximal extremities. Lesions are transient and tend to extend outward with central clearing and are often described as serpiginous.
Polyarthritis
The arthritis is typically migratory and involves the following large joints:
- Ankles
- Elbows
- Knees
- Wrists
Joint involvement may range from general arthralgia to a painful, inflammatory arthritis.
Subcutaneous nodules
Subcutaneous nodules are
- Firm
- Painless
- Variable in size
- Found over joint extensor surfaces (most common)
Size of subcutaneous nodules are typically between a few millimeters and 2 centimeters in diameter.
Diagnosis and testing
There's no definitive diagnostic test for acute rheumatic fever. Instead, the Jones Criteria and supplemental testing are used.
Treatment
Patients with acute rheumatic fever should start on therapy for the management of
- Acute rheumatic fever
- Cardiac failure
Therapy for acute rheumatic fever includes salicylates and anti-inflammatory medicines to relieve inflammation and decrease fever.
Antibiotics
These patients should also be started on antibiotics to eliminate any residual group A strep bacteria. Treatment is indicated regardless of the presence or absence of pharyngitis at the time of diagnosis.
Prognosis and complications
Prognosis
Untreated rheumatic fever increases a person's risk of recurrent attacks and worsens prognosis. Prognosis is related to the prevention of recurrent attacks, degree of cardiac valvular damage, and degree of overall cardiac involvement.
Complications
Rheumatic heart disease is the most important long-term sequela of acute rheumatic fever. It can cause disability or death.
Cardiac complications may vary in severity and include, but aren't limited to:
- Arrhythmias
- Congestive heart failure
- Endocarditis
- Pericarditis
- Stroke
- Valvular damage
Prevention
Reduce the spread of group A strep bacteria with standard infection control practices, including good hand hygiene and respiratory etiquette.
Preventing initial illness
Diagnosis and adequate treatment of group A strep pharyngitis and skin infections are the primary means of preventing acute rheumatic fever.
Preventing recurrent illnesses
Acute rheumatic fever frequently recurs with subsequent group A strep pharyngitis infections. Secondary prevention of rheumatic fever requires antibiotic prophylaxis to reduce the likelihood of recurrent attacks. Long-term prophylaxis duration should be individually tailored but is usually indicated at least until age 21 years.
Prophylaxis typically involves one of the following:
- An intramuscular injection of benzathine penicillin every 4 weeks
- Oral penicillin V twice daily
Sulfadiazine or oral macrolides can be taken daily by individuals who are allergic to penicillin.
The American Heart Association no longer recommends bacterial endocarditis prophylaxis unless the patient with rheumatic heart disease has a prosthetic valve.
Resources
Prevention guidelines
Prevention of rheumatic fever and diagnosis and treatment of acute streptococcal pharyngitis
American Heart Association and American Academy of Pediatrics
Prevention of infective endocarditis
American Heart Association