Chronic Rheumatic Fever
A chronic inflammatory disease affecting the heart, joints, and other organs following streptococcal infection
Quick Facts
- Type: Inflammatory Disease
- ICD-10: I00-I02
- Prevalence: Rare in developed countries
- Age: Most common in children 5-15 years
Overview
Chronic rheumatic fever is a serious inflammatory disease that can affect multiple organs, particularly the heart, joints, brain, and skin. It develops as a delayed autoimmune response to an untreated or inadequately treated Group A streptococcal infection, typically strep throat or scarlet fever. This condition represents the chronic or recurrent form of acute rheumatic fever and can lead to permanent damage to the heart valves, known as rheumatic heart disease.
The disease occurs when the body's immune system mistakenly attacks its own tissues after being triggered by streptococcal antigens that share molecular similarities with human proteins. This phenomenon, known as molecular mimicry, leads to cross-reactive antibodies and T-cells that target various organs. The most serious complication is damage to the heart valves, which can result in mitral or aortic valve stenosis or regurgitation, ultimately leading to heart failure if left untreated.
While rheumatic fever has become rare in developed countries due to improved living conditions, access to healthcare, and early treatment of streptococcal infections, it remains a significant health problem in developing nations and among certain populations. The chronic form can develop after repeated episodes of acute rheumatic fever or as a result of ongoing low-grade inflammation. Early recognition and appropriate treatment are crucial for preventing progressive cardiac damage and other serious complications.
The prognosis for chronic rheumatic fever varies depending on the severity of organ involvement, particularly cardiac damage. With proper medical management, including long-term antibiotic prophylaxis and treatment of complications, many patients can lead relatively normal lives. However, those with severe rheumatic heart disease may require surgical intervention and face a more guarded prognosis.
Symptoms
The symptoms of chronic rheumatic fever can vary widely depending on which organs are affected and the severity of involvement. Unlike acute rheumatic fever, chronic symptoms may be more subtle and develop gradually over time.
Cardiac Symptoms
Additional Cardiac Manifestations
- Palpitations - awareness of heartbeat or irregular rhythm
- Sharp chest pain - may indicate pericarditis
- Fatigue and weakness, especially with physical activity
- Swelling of legs, ankles, or abdomen (heart failure signs)
- Dizziness or fainting spells
Joint Symptoms (Arthritis)
- Migratory joint pain affecting large joints (knees, ankles, elbows, wrists)
- Joint swelling, warmth, and redness
- Stiffness, particularly in the morning
- Pain that moves from one joint to another
- Temporary limitation of joint movement
Neurological Symptoms (Sydenham's Chorea)
- Involuntary, jerky movements of face, hands, and feet
- Difficulty with coordination and fine motor skills
- Emotional instability and mood changes
- Speech difficulties
- Muscle weakness
- Problems with handwriting or drawing
Skin Manifestations
- Erythema marginatum - pink or red rash with clear centers
- Subcutaneous nodules - painless bumps under the skin
- Rash typically on trunk and inner surfaces of limbs
- Nodules commonly found over bony prominences
General Symptoms
- Recurrent fever, often low-grade in chronic cases
- Weight loss and poor appetite
- General malaise and feeling unwell
- Night sweats
- Abdominal pain
- Nosebleeds (epistaxis)
Causes
Chronic rheumatic fever is caused by an autoimmune response triggered by Group A streptococcal infection. Understanding this complex pathophysiology is crucial for both prevention and treatment.
Primary Trigger: Streptococcal Infection
The initial cause is infection with Group A Streptococcus (Streptococcus pyogenes):
- Strep throat: Most common initial infection
- Scarlet fever: Streptococcal infection with rash
- Impetigo: Superficial skin infection
- Cellulitis: Deeper skin and soft tissue infection
- Other streptococcal infections: Pneumonia, sepsis
Autoimmune Mechanism
The disease develops through molecular mimicry and cross-reactivity:
- Molecular mimicry: Streptococcal proteins resemble human proteins
- Cross-reactive antibodies: Antibodies attack both bacteria and human tissues
- T-cell activation: Cellular immune response against self-antigens
- Inflammatory cascade: Ongoing inflammation in multiple organs
Targeted Tissues
Specific human proteins that cross-react with streptococcal antigens:
- Cardiac myosin: Heart muscle protein leading to carditis
- Valve collagen: Heart valve proteins causing valve damage
- Brain proteins: Neural antigens resulting in chorea
- Joint proteins: Synovial tissue antigens causing arthritis
Progression to Chronic Disease
Several factors contribute to chronicity:
- Repeated infections: Multiple streptococcal exposures
- Incomplete treatment: Inadequate antibiotic therapy
- Persistent inflammation: Ongoing autoimmune response
- Genetic susceptibility: Individual immune system variations
- Environmental factors: Poor living conditions, malnutrition
Rheumatogenic Strains
Certain streptococcal strains are more likely to cause rheumatic fever:
- M protein types 1, 3, 5, 6, 14, 18, 19, and 24
- Highly mucoid strains
- Strains with strong cross-reactive antigens
- Epidemic strains in certain geographic regions
Risk Factors
Several factors increase the likelihood of developing chronic rheumatic fever following streptococcal infection.
Demographic Risk Factors
- Age: Most common in children aged 5-15 years
- Geographic location: Higher rates in developing countries
- Socioeconomic status: More common in lower socioeconomic groups
- Population density: Overcrowded living conditions
- Indigenous populations: Higher susceptibility in certain ethnic groups
Genetic Factors
- Family history: Increased risk with affected family members
- HLA associations: Certain HLA types (DR7, DQA1, DQB1)
- Immune system variations: Individual differences in immune response
- Complement deficiencies: Inherited immune system defects
Environmental Risk Factors
- Poor hygiene: Limited access to clean water and sanitation
- Malnutrition: Compromised immune system function
- Overcrowding: Schools, households, institutions
- Limited healthcare access: Delayed or inadequate treatment
- Climate: Temperate climates with seasonal variations
Medical Risk Factors
- Previous rheumatic fever: Highest risk factor for recurrence
- Untreated strep throat: Primary precipitating factor
- Incomplete antibiotic treatment: Inadequate bacterial eradication
- Immunocompromised state: HIV, diabetes, other conditions
- Chronic tonsillitis: Recurrent streptococcal infections
Behavioral Risk Factors
- Poor medication compliance: Not completing antibiotic courses
- Delayed medical care: Ignoring symptoms of strep throat
- Close contact settings: Schools, daycare, military barracks
- Sharing personal items: Utensils, drinking glasses
Regional Variations
Risk varies significantly by geographic region:
- High-risk regions: Sub-Saharan Africa, South Asia, Pacific Islands
- Moderate-risk regions: Eastern Europe, Latin America
- Low-risk regions: North America, Western Europe, Australia
- Indigenous populations: Higher rates in Aboriginal communities worldwide
Diagnosis
Diagnosing chronic rheumatic fever can be challenging as there is no single diagnostic test. The diagnosis is based on clinical criteria, laboratory tests, and evidence of previous streptococcal infection.
Jones Criteria (Modified)
The diagnosis requires evidence of antecedent streptococcal infection plus:
Major Criteria
- Carditis: Heart inflammation (clinical or subclinical)
- Arthritis: Migratory polyarthritis of large joints
- Chorea: Sydenham's chorea (involuntary movements)
- Erythema marginatum: Characteristic skin rash
- Subcutaneous nodules: Painless nodules over bony prominences
Minor Criteria
- Fever: Temperature ≥38.5°C (101.3°F)
- Arthralgia: Joint pain without objective signs
- Elevated acute phase reactants: ESR ≥60 mm/hr, CRP ≥3.0 mg/dL
- Prolonged PR interval: On ECG (age-adjusted)
Laboratory Tests
Evidence of Streptococcal Infection
- Antistreptolysin O (ASO): Elevated titer (≥320 Todd units in adults)
- Anti-DNase B: Alternative streptococcal antibody test
- Streptozyme test: Multiple streptococcal antibodies
- Throat culture: May be negative in chronic cases
- Rapid strep test: Quick antigen detection
Inflammatory Markers
- Erythrocyte sedimentation rate (ESR): Usually elevated
- C-reactive protein (CRP): Acute phase reactant
- White blood cell count: May be elevated
- Complement levels: May be altered
Cardiac Evaluation
- Echocardiography: Essential for detecting carditis and valve abnormalities
- Electrocardiogram (ECG): May show PR prolongation, arrhythmias
- Chest X-ray: May reveal cardiomegaly or heart failure
- Cardiac MRI: For detailed cardiac assessment if needed
Additional Studies
- Joint imaging: X-rays or ultrasound of affected joints
- Brain MRI: If chorea is present
- Skin biopsy: Rarely needed for diagnosis
- Blood cultures: To rule out infective endocarditis
Diagnostic Criteria
For initial episode:
- 2 major criteria OR
- 1 major + 2 minor criteria
- Plus evidence of antecedent streptococcal infection
For recurrent episodes:
- 2 major criteria OR
- 1 major + 2 minor criteria OR
- 3 minor criteria
- Plus evidence of antecedent streptococcal infection
Differential Diagnosis
- Infective endocarditis
- Juvenile idiopathic arthritis
- Systemic lupus erythematosus
- Kawasaki disease
- Viral myocarditis
- Drug-induced lupus
- Tourette syndrome (for chorea)
Treatment Options
Treatment of chronic rheumatic fever focuses on controlling inflammation, managing complications, preventing recurrences, and providing long-term care for affected organs.
Antibiotic Therapy
Acute Treatment
- Penicillin: Drug of choice for streptococcal eradication
- Oral penicillin V: 500mg twice daily for 10 days
- Intramuscular penicillin G: Single dose for compliance issues
- Alternatives: Erythromycin, azithromycin, or clindamycin for penicillin-allergic patients
Secondary Prophylaxis
Long-term antibiotic prophylaxis to prevent recurrence:
- Benzathine penicillin G: 1.2 million units IM every 3-4 weeks
- Oral penicillin V: 250mg twice daily
- Sulfadiazine: 1g daily for patients >27kg
- Duration: Varies based on cardiac involvement and age
Anti-inflammatory Treatment
Salicylates
- Aspirin: High-dose for arthritis (80-100 mg/kg/day in children)
- Monitoring: Salicylate levels, liver function
- Duration: Until inflammation subsides (typically 4-8 weeks)
Corticosteroids
- Indication: Severe carditis or heart failure
- Prednisolone: 1-2 mg/kg/day initially
- Tapering: Gradual reduction over 2-3 weeks
- Monitoring: Blood glucose, blood pressure, side effects
Cardiac Management
Heart Failure Treatment
- ACE inhibitors: Reduce afterload and prevent remodeling
- Diuretics: Manage fluid overload
- Beta-blockers: Improve cardiac function (when stable)
- Digoxin: For severe heart failure or atrial fibrillation
Valve Disease Management
- Medical management: First-line for most patients
- Anticoagulation: For atrial fibrillation or mechanical valves
- Surgical repair: For severe valve dysfunction
- Valve replacement: When repair is not possible
Treatment of Specific Manifestations
Chorea Management
- Haloperidol: 0.5-2mg daily for severe cases
- Valproic acid: Alternative medication
- Supportive care: Safe environment, physical therapy
- Education: Family and school support
Arthritis Treatment
- NSAIDs: Effective for joint symptoms
- Rest: During acute phase
- Physical therapy: To maintain joint function
- Heat therapy: For pain relief
Supportive Care
- Bed rest: During acute phase with carditis
- Activity restriction: Gradual return to normal activities
- Nutritional support: Adequate calories and nutrients
- Psychological support: Counseling for chronic disease management
- School accommodations: Modified activities during recovery
Duration of Prophylaxis
Secondary prophylaxis duration depends on cardiac involvement:
- No carditis: 5 years or until age 21 (whichever is longer)
- Carditis without valve disease: 10 years or until age 21
- Carditis with valve disease: 10 years or until age 40
- Valve surgery: Lifelong prophylaxis
Prevention
Prevention of chronic rheumatic fever involves both primary prevention (preventing initial episodes) and secondary prevention (preventing recurrences in those already affected).
Primary Prevention
Preventing the initial episode by prompt treatment of streptococcal infections:
Early Recognition and Treatment
- Recognize strep throat symptoms: Sore throat, fever, swollen lymph nodes
- Seek prompt medical care: Within 9 days of symptom onset
- Complete antibiotic course: Full 10-day treatment even if feeling better
- Follow-up care: Return if symptoms persist or worsen
Public Health Measures
- Improved living conditions: Reduce overcrowding
- Better nutrition: Strengthen immune system
- Access to healthcare: Ensure availability of medical care
- Education programs: Community awareness about strep throat
- School health programs: Early detection and treatment
Secondary Prevention
Preventing recurrent episodes in those with previous rheumatic fever:
Antibiotic Prophylaxis
- Benzathine penicillin G: Most effective regimen
- Regular schedule: Every 3-4 weeks without fail
- Alternative oral regimens: For injection-intolerant patients
- Compliance monitoring: Regular follow-up and support
Lifestyle Modifications
- Good hygiene: Frequent handwashing, avoid sharing utensils
- Avoid exposure: Stay away from people with strep throat
- Dental prophylaxis: Antibiotics before certain dental procedures
- Regular medical care: Routine cardiac monitoring
Infection Control Measures
- Isolation: Stay home when infected until treated for 24 hours
- Hand hygiene: Wash hands frequently with soap and water
- Respiratory etiquette: Cover coughs and sneezes
- Environmental cleaning: Disinfect surfaces and objects
- Avoid sharing: Personal items, drinks, or utensils
High-Risk Population Strategies
- Family screening: Test family members of affected individuals
- School surveillance: Monitor for outbreaks in schools
- Community programs: Target high-risk populations
- Healthcare worker education: Recognize and treat strep throat promptly
Global Prevention Efforts
- WHO initiatives: Global strategy for rheumatic heart disease
- Vaccine development: Research into streptococcal vaccines
- Screening programs: Echocardiographic screening in endemic areas
- Healthcare infrastructure: Improve access to diagnosis and treatment
- Research funding: Support for prevention and treatment studies
Special Considerations
- Pregnancy: Continue prophylaxis with penicillin
- Dental procedures: May need additional antibiotic coverage
- Surgery: Perioperative antibiotic prophylaxis
- Travel: Ensure access to prophylaxis medications
When to See a Doctor
Recognizing when to seek medical attention is crucial for preventing serious complications of rheumatic fever and its progression to chronic disease.
Seek Emergency Care Immediately
- Severe shortness of breath or difficulty breathing
- Sharp chest pain that worsens with breathing
- Signs of heart failure (swelling, severe fatigue, inability to lie flat)
- Sudden weakness or paralysis
- Severe involuntary movements that interfere with daily activities
- High fever with severe throat feels tight
- Fainting or severe dizziness
- Rapid or irregular heartbeat with symptoms
Schedule Urgent Appointment
- Persistent fever lasting more than 3 days
- Severe sore throat with difficulty swallowing
- Joint pain that moves from one joint to another
- New or worsening chest tightness
- Increased heart rate at rest
- Unexplained rash, especially with other symptoms
- New involuntary movements or coordination problems
- Worsening fatigue or exercise intolerance
Routine Medical Consultation
- History of rheumatic fever needing prophylaxis monitoring
- Family history of rheumatic heart disease
- Recurrent strep throat infections
- Need for cardiac screening or monitoring
- Questions about antibiotic prophylaxis
- Planning for dental procedures or surgery
- Pregnancy planning with history of rheumatic fever
Strep Throat Symptoms Requiring Attention
- Sudden onset of severe sore throat
- Fever higher than 101°F (38.3°C)
- White patches or pus on the throat
- Swollen, tender lymph nodes in the neck
- Headache and body aches
- Nausea or vomiting (especially in children)
- Scarlet fever rash (red, sandpaper-like rash)
Follow-up Care Requirements
For patients with diagnosed rheumatic fever:
- Regular cardiology visits: Annual or as recommended
- Echocardiogram monitoring: To assess valve function
- Prophylaxis compliance: Ensure antibiotic adherence
- Dental consultation: Before any dental procedures
- Pregnancy planning: Preconception counseling
- Symptom monitoring: Report any new or worsening symptoms
Special Populations
Children and Adolescents
- School absence due to recurrent illnesses
- Behavioral changes or academic difficulties
- Growth or developmental concerns
- Difficulty with physical activities
Adults with Previous Rheumatic Fever
- Pre-pregnancy counseling and monitoring
- Career planning with cardiac limitations
- Insurance and disability considerations
- Transition of care from pediatric to adult cardiology
Frequently Asked Questions
While acute episodes of rheumatic fever can be treated and inflammation controlled, the damage to heart valves and other organs may be permanent. However, with proper long-term management including antibiotic prophylaxis and treatment of complications, many patients can live normal or near-normal lives.
The duration depends on whether you have heart involvement. Without heart damage, prophylaxis is typically for 5 years or until age 21. With heart involvement, it may be for 10 years or until age 40, and sometimes lifelong if you have severe valve disease or have had valve surgery.
Many women with rheumatic heart disease can safely have children, but pregnancy requires specialized care from a maternal-fetal medicine specialist and cardiologist. The safety depends on the severity of heart involvement and how well your heart function is controlled.
Rheumatic fever itself is not contagious, but the strep throat infection that causes it is contagious. People with rheumatic fever are not infectious to others, but they need to be careful to avoid getting new strep infections.
Not everyone with rheumatic heart disease needs surgery. The need for surgery depends on the severity of valve damage and how well your heart is functioning. Many people can be managed with medications alone, while others may eventually need valve repair or replacement.
References
- American Heart Association. Prevention of Rheumatic Fever and Diagnosis and Treatment of Acute Streptococcal Pharyngitis. Circulation. 2024.
- World Heart Federation. Rheumatic Heart Disease Guidelines. Global Heart. 2023.
- Gerber MA, et al. Prevention of Rheumatic Fever and Diagnosis and Treatment of Acute Streptococcal Pharyngitis. Pediatrics. 2023.
- Zühlke L, et al. Clinical Outcomes in 3343 Children and Adults with Rheumatic Heart Disease from 14 Low- and Middle-Income Countries. Circulation. 2024.
- National Institute for Health and Care Excellence. Rheumatic Fever: Diagnosis and Management. NICE Guidelines. 2023.