Rheumatic fever is an inflammatory disease that can develop after an infection with Group A Streptococcus bacterium, the same bacteria that cause strep throat and scarlet fever. It primarily affects children aged 5 to 15 years old, but it can occur in adults as well. Rheumatic fever can cause permanent damage to the heart, particularly the valves, a condition known as rheumatic heart disease. It can also affect the joints, skin, and nervous system. Although rheumatic fever is rare in developed countries, it remains a major health problem in developing nations.
The primary cause of rheumatic fever is an untreated or inadequately treated strep throat or scarlet fever caused by Group A Streptococcus bacterium. The immune system, in its attempt to fight off the bacterial infection, mistakenly attacks healthy tissues, particularly in the heart, joints, skin, and brain. This autoimmune reaction can lead to the inflammation and symptoms associated with rheumatic fever.
Not everyone who has a strep throat infection will develop rheumatic fever. Certain factors increase the risk, including:
- Recurrent Strep Infections: People who have repeated episodes of strep throat are at higher risk.
- Family History: A genetic predisposition may play a role, as people with a family history of rheumatic fever are more likely to develop the disease.
- Environmental Factors: Overcrowded living conditions, poor sanitation, and limited access to medical care increase the risk of strep infections and, subsequently, rheumatic fever.
The symptoms of rheumatic fever typically appear 2 to 4 weeks after a strep throat or scarlet fever infection. The severity of symptoms varies, and they can affect multiple parts of the body. Common symptoms include:
- Fever: A high fever is often one of the first signs.
- Painful and Swollen Joints (Migratory Arthritis): The joints may become red, hot, swollen, and painful, particularly the larger joints such as the knees, ankles, elbows, and wrists. The pain often moves from one joint to another.
- Chest Pain and Shortness of Breath: Inflammation of the heart (carditis) can cause these symptoms. Carditis is the most serious complication of rheumatic fever and can lead to permanent heart damage.
- Fatigue and Weakness: General malaise and fatigue are common as the body responds to inflammation.
- Skin Rash (Erythema Marginatum): A distinctive, pinkish-red rash may develop, particularly on the trunk and upper limbs. The rash is typically not itchy and has a circular pattern with a clear center.
- Nodules under the Skin: Small, painless lumps may form under the skin, usually near joints.
- Uncontrolled Movements (Sydenham Chorea): Also known as St. Vitus’ dance, this condition involves jerky, involuntary movements of the hands, feet, and face. It can make simple tasks, like writing or eating, difficult.
Rheumatic fever can cause severe and lasting complications, particularly if it affects the heart. The most serious complication is rheumatic heart disease, which results from inflammation of the heart tissues, especially the valves. The damage caused by this inflammation can lead to scarring and narrowing of the heart valves, preventing them from functioning properly. This can lead to:
- Heart Valve Damage: Rheumatic heart disease can cause the valves to leak (regurgitation) or become narrowed (stenosis), which can lead to heart failure, arrhythmias, and other serious conditions.
- Endocarditis: Inflammation of the inner lining of the heart can increase the risk of infection, potentially leading to a life-threatening condition known as infective endocarditis.
- Heart Failure: Over time, the heart’s ability to pump blood effectively can be compromised, leading to heart failure.
Other complications of rheumatic fever include:
- Joint Damage: Although the arthritis associated with rheumatic fever typically resolves without long-term effects, in severe cases, it can lead to chronic joint problems.
- Nervous System Damage: Sydenham chorea, while usually temporary, can be debilitating and may persist for several months.
- Kidney Disease: In rare cases, rheumatic fever can cause inflammation in the kidneys, leading to complications like glomerulonephritis.
Diagnosing rheumatic fever involves a combination of medical history, physical examination, and laboratory tests. The most commonly used criteria for diagnosis are the Jones Criteria, which require evidence of a prior strep infection along with the presence of major and minor clinical findings.
- Medical History and Physical Exam: The doctor will ask about recent strep infections, symptoms of fever, joint pain, and heart-related symptoms. A thorough physical examination will help assess signs of inflammation, rash, and joint swelling.
- Throat Culture or Rapid Strep Test: These tests are used to confirm the presence of Group A Streptococcus bacteria. A throat culture involves swabbing the throat and growing the bacteria in a lab, while the rapid strep test provides quicker results.
- Blood Tests: Blood tests, such as the antistreptolysin O (ASO) titer, can detect antibodies produced in response to a strep infection. Elevated levels of these antibodies suggest a recent infection.
- Electrocardiogram (ECG): An ECG records the electrical activity of the heart and can help detect abnormalities caused by carditis.
- Echocardiogram: This imaging test uses sound waves to create pictures of the heart, allowing doctors to assess heart valve damage and other abnormalities.
The primary goals of treatment for rheumatic fever are to eliminate the strep infection, reduce inflammation, manage symptoms, and prevent long-term complications. Treatment typically involves:
- Antibiotics: A course of antibiotics, usually penicillin, is given to eliminate the strep bacteria. In some cases, long-term antibiotic therapy may be required to prevent recurrent infections, particularly in patients who have had rheumatic heart disease.
- Anti-inflammatory Medications: Nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin or ibuprofen, are used to reduce inflammation and relieve joint pain. In severe cases, corticosteroids may be prescribed to control inflammation, especially if the heart is affected.
- Anticonvulsants: If Sydenham chorea is present, anticonvulsant medications, such as valproic acid or carbamazepine, may be used to control involuntary movements.
- Bed Rest: Bed rest is often recommended during the acute phase of the illness to reduce strain on the heart, particularly in cases of carditis.
Preventing rheumatic fever involves prompt and effective treatment of strep throat infections. To reduce the risk of developing rheumatic fever:
- Treat Strep Throat Early: Any suspected case of strep throat should be diagnosed and treated with a full course of antibiotics. Even if symptoms improve, it’s crucial to complete the medication as prescribed.
- Prevent Recurrence: People who have had rheumatic fever in the past are at higher risk for recurrence. Long-term antibiotic prophylaxis (preventive antibiotics) is often recommended to prevent future strep infections, especially in patients with rheumatic heart disease.
- Improve Living Conditions: Addressing overcrowding and improving hygiene and sanitation can help reduce the transmission of strep infections, particularly in at-risk populations.
Rheumatic fever is a serious but preventable disease that can cause long-lasting damage to the heart and other organs. Early diagnosis and treatment are critical to preventing complications, especially rheumatic heart disease. By promptly treating strep throat infections and following appropriate preventive measures, the risk of rheumatic fever can be significantly reduced. Ongoing medical care and monitoring are essential for those who have had rheumatic fever to ensure optimal long-term outcomes.