Rheumatic Fever (RF): Causes, Symptoms, Diagnosis, and Management
Rheumatic Fever (RF) is a serious inflammatory disease that develops in susceptible individuals following an untreated or poorly treated Group A Streptococcus (Strep) throat infection. It is an autoimmune reaction where the body's immune system mistakenly attacks healthy tissues, primarily affecting the heart, joints, skin, and brain. Timely antibiotic treatment of Strep throat is crucial for prevention and minimizing long-term cardiac damage.
Key Takeaways
RF is triggered by an untreated Group A Strep infection.
The disease primarily damages heart valves (Rheumatic Heart Disease).
Diagnosis relies on the revised Jones Criteria (Major and Minor signs).
Prevention requires prompt antibiotic treatment of Strep throat.
Long-term management involves continuous antibiotic prophylaxis.
What is Rheumatic Fever and how common is it?
Rheumatic Fever (RF) is an inflammatory disease that occurs as a delayed, non-suppurative complication of an untreated Group A Streptococcus (GAS) infection, typically strep throat. This condition involves multiple body systems, including the heart, joints, skin, and brain, usually developing two to four weeks after the initial infection. While RF is rare in high-income countries, it remains a major public health concern in regions like Africa, South Asia, and the Pacific, predominantly affecting children and adolescents aged 5 to 15 years.
- RF is an inflammatory disease involving the heart, joints, skin, and brain.
- It develops 2–4 weeks after an untreated Strep throat infection.
- Incidence is rare in high-income countries but high in developing regions.
- Most affected demographic includes children and adolescents (5–15 years).
How does Rheumatic Fever develop in the body?
Rheumatic Fever develops through a mechanism known as molecular mimicry, where the immune response intended to fight the Group A ß-hemolytic Streptococcus (S. pyogenes) mistakenly attacks the host's own tissues. The M protein found on the surface of the Strep bacteria closely resembles proteins in human tissue, leading to cross-reactivity. Antibodies and T-cells generated against the bacteria subsequently attack vulnerable sites, causing inflammation in the heart (pancarditis), joints (migratory arthritis), brain (Sydenham chorea), and skin.
- Triggered by Group A ß-hemolytic Streptococcus (S. pyogenes) infection.
- Molecular Mimicry occurs when the M protein resembles human tissue.
- Cross-reactivity causes antibodies/T-cells to attack host tissues.
- Inflammation sites include the heart, joints, brain, and skin.
What are the primary causes and risk factors for Rheumatic Fever?
The primary cause of Rheumatic Fever is an antecedent infection with Group A Streptococcus (GAS), which initiates the autoimmune response. However, not everyone who gets strep throat develops RF; certain risk factors increase susceptibility. These factors include age, as children between 5 and 15 years are most vulnerable, and environmental conditions such as overcrowding and poor living standards, which facilitate the spread of GAS. Crucially, untreated or recurrent strep throat significantly elevates the risk of developing RF.
- Primary Cause: Group A Streptococcus infection.
- Risk Factor: Age (Children 5–15 years).
- Risk Factor: Untreated or recurrent strep throat infections.
- Risk Factor: Poor access to healthcare and antibiotics.
- Risk Factor: Overcrowding and poor living conditions.
- Risk Factor: Genetic predisposition.
What are the key signs and symptoms of Rheumatic Fever?
The clinical presentation of Rheumatic Fever is highly variable, manifesting as a combination of major and minor symptoms used for diagnosis under the Jones Criteria. Major manifestations include carditis, which affects up to 50% of cases and can involve all layers of the heart, and migratory polyarthritis, affecting 80% of patients, where pain moves from joint to joint. Less common major signs include Sydenham chorea (involuntary movements), erythema marginatum (a non-itchy rash), and subcutaneous nodules. Minor manifestations include fever, joint pain (arthralgia), and elevated inflammatory markers.
- Major Manifestations: Carditis, Migratory polyarthritis, Sydenham's chorea, Erythema marginatum, Subcutaneous nodules.
- Minor Manifestations: Fever, Arthralgia, Raised acute-phase reaction (ESR, CRP).
- Minor Manifestations: Prolonged P-R interval on ECG.
How is Rheumatic Fever diagnosed using clinical criteria and tests?
Diagnosis of Rheumatic Fever relies primarily on the revised Jones Criteria, which requires evidence of a preceding Strep infection combined with either two major manifestations or one major and two minor manifestations. Clinicians gather a detailed history, noting any antecedent sore throat, which is present in about 70% of older children. Laboratory tests are crucial for confirming inflammation and past Strep exposure, although throat cultures are often negative during the acute RF phase. Radiological tests, particularly echocardiography, are essential for assessing heart valve damage and confirming carditis.
- Diagnosis uses Jones Criteria (Major and Minor manifestations).
- History taking includes antecedent sore throat history.
- Laboratory tests include CRP, ESR, and ASO titre.
- Radiological tests include ECG (for arrhythmias) and 2D Echocardiograph (for valve damage).
What is the standard medical management for Rheumatic Fever?
Medical management focuses on three key areas: eradicating the residual Strep infection, controlling inflammation, and managing specific symptoms. Antibiotics, typically Penicillin, are used immediately to eliminate any remaining Group A Strep, followed by long-term prophylaxis to prevent recurrence. Anti-inflammatory drugs, such as NSAIDs or salicylates, treat fever and joint pain, while corticosteroids like Prednisone are reserved for moderate to severe carditis. If heart failure develops due to severe carditis, specific treatments like diuretics and ACE inhibitors are initiated, and surgical valve repair or replacement may be necessary in chronic cases.
- Eradicate Strep using antibiotics (Penicillin for 5 years or until age 21).
- Use NSAIDs/Salicylates for fever and pain relief.
- Administer Corticosteroids for moderate/severe carditis.
- Treat Sydenham chorea with antiseizure drugs.
- Manage heart failure and consider valve repair/replacement.
What are the potential long-term complications of Rheumatic Fever?
The most significant and chronic complication of Rheumatic Fever is Chronic Rheumatic Heart Disease (RHD), which results from permanent damage to the heart valves, particularly the mitral valve, leading to stenosis or regurgitation. Recurrent episodes of RF significantly worsen this heart damage. Long-term RHD increases the risk of serious cardiac issues, including heart failure, atrial fibrillation (AFib), and pulmonary hypertension. Acute complications during the initial phase primarily involve severe carditis, which can rapidly lead to heart failure, and the debilitating, though temporary, migratory polyarthritis.
- Chronic Rheumatic Heart Disease (RHD) is the main long-term outcome.
- Permanent valve damage (Mitral stenosis/regurgitation is common).
- Increased risk of Heart failure, AFib, and Pulmonary Hypertension.
- Acute complications include severe carditis and migratory polyarthritis.
What is the role of nursing care in managing Rheumatic Fever patients?
Nursing management during the acute phase focuses on reducing cardiac workload and monitoring for complications. This involves enforcing strict bed rest, especially if carditis is present, and closely monitoring vital signs and signs of heart failure. For patients experiencing Sydenham chorea, providing a quiet and safe environment is essential. Nurses are also responsible for administering medications, including the initial Strep eradication antibiotics and long-term monthly Penicillin injections for prophylaxis, while providing crucial health education to ensure the patient completes the full antibiotic course and adheres to the chronic care plan.
- Enforce bed rest to reduce cardiac workload (with carditis).
- Monitor Vitals and signs of heart failure.
- Provide a quiet environment if chorea is present.
- Administer long-term prophylaxis via monthly Penicillin injections.
- Offer psychosocial support and health education.
How can Rheumatic Fever be prevented at different stages?
Prevention of Rheumatic Fever is categorized into three levels. Primary prevention aims to stop the initial attack by ensuring prompt and complete treatment of all Group A Strep infections, often through health education about the dangers of untreated sore throats and improving living conditions to reduce transmission. Secondary prevention is critical for preventing recurrence in those who have already had RF, involving continuous antibiotic prophylaxis, typically monthly Penicillin injections, and regular medical follow-up. Tertiary prevention focuses on minimizing disability caused by established RHD through rehabilitation and lifestyle modifications.
- Primary Prevention: Health Education on sore throat dangers and improving living conditions.
- Secondary Prevention: Continuous Prophylaxis (Monthly Penicillin) and regular follow-up.
- Tertiary Prevention: Rehabilitation and limiting strenuous activity if the heart is affected.
Frequently Asked Questions
What is the main trigger for Rheumatic Fever?
The main trigger is an untreated or poorly treated infection with Group A Streptococcus (Strep throat). RF develops 2 to 4 weeks later due to an autoimmune reaction called molecular mimicry.
Which part of the body is most seriously affected by RF?
The heart is the most seriously affected organ. Inflammation (carditis) can lead to permanent damage to the heart valves, resulting in Chronic Rheumatic Heart Disease (RHD).
What are the Jones Criteria used for?
The Jones Criteria are used to diagnose Rheumatic Fever. They require evidence of a preceding Strep infection plus a specific combination of major manifestations (like carditis or arthritis) and minor signs (like fever).
How is recurrence of Rheumatic Fever prevented?
Recurrence is prevented through secondary prophylaxis, which involves continuous, long-term antibiotic treatment, typically monthly injections of Penicillin, often lasting for many years.
Why is bed rest important during the acute phase of RF?
Bed rest is crucial, especially if carditis is present, because it helps reduce the workload on the inflamed heart muscle, minimizing the risk of developing acute heart failure.
