Rheumatic Fever: Symptoms, Types, Causes and Treatment

Rheumatic fever is a serious inflammatory disease that can develop after an infection with group A streptococcus bacteria. It primarily affects children and young people, especially in low-resource communities, and can lead to long-term complications like rheumatic heart disease. Understanding its symptoms, types, causes, and treatment options is crucial for early recognition and effective management. In this comprehensive guide, we explore each of these aspects based on current research and clinical recommendations.

Symptoms of Rheumatic Fever

Rheumatic fever often presents with a constellation of symptoms that can affect multiple organ systems. Recognizing these signs early is essential for prompt treatment and prevention of long-term damage, especially to the heart. The symptoms usually appear two to three weeks after a streptococcal throat infection, but can sometimes manifest even without a noticeable sore throat.

Symptom	Description	Frequency/Severity	Sources
Joint Pain	Migratory, often severe, swelling, redness	Very common (major sign)	1 2 4
Carditis	Inflammation of the heart/valves	Up to 80% of cases	1 2 3 4
Chorea	Involuntary movements (Sydenham's chorea)	Less common, disabling	2 3
Rash	Erythema marginatum, ring-shaped	Uncommon	1 2 3
Nodules	Subcutaneous, firm, painless lumps	Rare	1 2 3

Table 1: Key Symptoms

Major and Minor Manifestations

Major Manifestations:

• Polyarthritis: Sudden onset pain, swelling, and redness in large joints (knees, ankles, elbows, wrists). The pain “migrates” from one joint to another.
• Carditis: Inflammation of the heart muscle and valves, often leading to murmurs, chest pain, shortness of breath, or heart failure. The mitral and aortic valves are most frequently affected, potentially causing regurgitation and chronic rheumatic heart disease 1 2 4.
• Sydenham's Chorea: Characterized by involuntary, jerking movements, muscle weakness, and emotional instability. It may cause significant disability, including difficulty speaking or walking, especially in children 3.
• Erythema Marginatum: A fleeting, pink, ring-shaped rash primarily on the trunk and limbs.
• Subcutaneous Nodules: Small, firm, painless lumps under the skin, typically over joints or the spine.

Minor Manifestations:

• Fever, elevated inflammatory markers (ESR, CRP)
• Arthralgia (joint pain without swelling)
• Prolonged PR interval on ECG

Symptom Progression and Complications

• Acute Phase: Symptoms develop 2–3 weeks after a strep throat infection.
• Chronic Effects: About half of those affected may develop chronic rheumatic heart disease, which can lead to arrhythmias, stroke, infective endocarditis, and pregnancy complications 1 2.
• Neuropsychiatric Impact: Sydenham's chorea can cause significant distress and impairment, including speech difficulties and functional decline 3.

Types of Rheumatic Fever

While rheumatic fever is usually discussed as a single disease, there are different ways it can present and progress. Understanding these types helps clinicians tailor management and anticipate complications.

Type	Characteristics	Typical Population	Sources
Acute	Sudden onset, after strep infection	Children 5–15 years	1 4 6
Recurrent	Repeat episodes after new strep	Anyone with prior RF	2 9 10
Chronic (RHD)	Long-term heart valve damage	Often adults, teens	1 2 4 5

Table 2: Types of Rheumatic Fever

Acute Rheumatic Fever

• Timing: Develops 2–3 weeks after untreated or inadequately treated group A streptococcal pharyngitis.
• Presentation: Classic symptoms as outlined above. Most often seen in children aged 5–15, especially in low- and middle-income countries and among indigenous populations 1 4 6.
• Prognosis: The inflammatory process can resolve over weeks to months, but risk of heart damage remains high 1.

Recurrent Rheumatic Fever

• Definition: New episodes of rheumatic fever triggered by subsequent strep infections in individuals with a previous history of the disease.
• Risk: People with prior rheumatic fever are at much higher risk of recurrence, especially if secondary antibiotic prophylaxis is not maintained 2 9 10.
• Complications: Each recurrence increases the risk of cumulative heart valve damage.

Chronic Rheumatic Heart Disease

• Description: Long-term damage to heart valves (mostly mitral and aortic) resulting from repeated or severe episodes of rheumatic fever.
• Manifestations: Can cause heart failure, arrhythmias, increased risk of stroke, infective endocarditis, and complications during pregnancy 1 2 4 5.
• Prevalence: At least 15 million people worldwide are estimated to have chronic rheumatic heart disease 1.

Causes of Rheumatic Fever

The root cause of rheumatic fever lies in the body’s immune response to a specific bacterial infection. However, various factors influence who gets sick and why.

Cause	Description	Risk Factors/Notes	Sources
Streptococcus	Group A strep throat infection	Untreated/poorly treated	1 2 4 5
Autoimmune Rxn	Immune cross-reactivity with self-tissue	Molecular mimicry	2 5 6
Genetics	Family history increases risk	Not fully understood	7
Social Factors	Overcrowding, poverty, health access	Major risk amplifiers	5 7

Table 3: Causes and Risk Factors

Streptococcal Infection

• Group A Streptococcus: Rheumatic fever follows an untreated or inadequately treated throat infection (pharyngitis) caused by Streptococcus pyogenes (group A beta-hemolytic streptococcus) 1 2 4 5.
• Timing: Symptoms develop 2–3 weeks after the initial infection, often when throat symptoms have resolved 1.

Autoimmune Mechanism (Molecular Mimicry)

• How It Happens: The immune system mounts a response to strep antigens, some of which resemble human tissue (particularly heart, joints, brain, and skin). This leads to cross-reactive inflammation—essentially, the body attacks itself 2 5 6.
• Key Element: The bacterial M protein shares epitopes with cardiac tissue, making the heart especially vulnerable 6.

Genetic and Environmental Risk Factors

• Genetics: Having a family history of rheumatic fever significantly raises risk, suggesting a genetic predisposition 7.
• Social Determinants: Overcrowded living conditions, poverty, and limited access to healthcare all increase the risk by facilitating the spread of strep infections and delaying treatment 5 7.
• Other Infections: Recent evidence points to a possible link between skin infections and increased risk, especially in certain populations 7.

Treatment of Rheumatic Fever

Effective management of rheumatic fever centers on eradicating the causative bacteria, reducing inflammation, and preventing recurrences or long-term heart damage. Early intervention is crucial.

Treatment	Purpose	Comments/Alternatives	Sources
Antibiotics	Eradicate strep bacteria	Penicillin preferred; alternatives for allergies	9 10 12
Anti-inflammatories	Control inflammation/pain	Aspirin or corticosteroids	11
Bed Rest	Support recovery	Especially with carditis	11
Secondary Prophylaxis	Prevent recurrences	Long-term penicillin or alternatives	2 9 10
Surgery	Treat severe heart damage	For chronic rheumatic heart disease	2

Table 4: Treatment Options

Antibiotic Therapy

• Primary Goal: Eliminate group A streptococcal bacteria from the throat to halt the autoimmune response and prevent spread 9 10 12.
• First-line: Penicillin, either as oral penicillin V or a single intramuscular injection of benzathine penicillin, is preferred due to efficacy, safety, and cost. Streptococcal resistance to penicillin has not been documented 9 10 12.
• Alternatives: For those allergic to penicillin, cephalosporins, clindamycin, or macrolides (e.g., erythromycin) can be used 9 10 12.
• Timing: Early administration after diagnosis of strep throat can reduce the risk of developing rheumatic fever by up to 70–80% 8.

Anti-inflammatory Treatment

• Aspirin: Used to control joint pain and reduce fever. Particularly effective for arthritis without carditis 11.
• Corticosteroids: Recommended for patients with carditis (heart involvement) to reduce cardiac inflammation and improve outcomes. Hydrocortisone has shown greater benefits than aspirin alone in some cases 11.
• Combination Therapy: No additional benefit over individual agents 11.

Supportive Care and Bed Rest

• Adequate rest is advised, especially for those with carditis or severe symptoms, to support recovery and reduce cardiac workload 11.

Secondary Prevention (Long-term Prophylaxis)

• Who Needs It: Anyone with a history of rheumatic fever is at high risk for recurrence and must receive continuous antibiotic prophylaxis 2 9 10 12.
• Regimen: Regular intramuscular penicillin injections or oral penicillin; alternatives for allergies include sulfadiazine or macrolides 9 10 12.
• Duration: Depends on age, time since last episode, cardiac involvement, and exposure risk; can last years or even decades 9 10 12.

Management of Chronic Rheumatic Heart Disease

• Medical: Ongoing monitoring and management of heart failure, arrhythmias, and anticoagulation if needed.
• Surgical: Valve repair or replacement for advanced cases 2.

Emerging Prevention Strategies

• Vaccination: Research is underway for a group A strep vaccine, which could dramatically reduce the global burden of rheumatic fever in the future 2.

Conclusion

Rheumatic fever remains a significant health challenge, particularly in low-resource settings. Early detection, prompt treatment, and long-term prevention are essential to reduce both immediate symptoms and long-term complications.

Key Points Covered:

• Symptoms: Include joint pain, carditis, chorea, rash, and subcutaneous nodules, often appearing weeks after a strep throat infection.
• Types: Acute, recurrent, and chronic (rheumatic heart disease) forms, each with distinct features and challenges.
• Causes: Triggered by a group A streptococcal throat infection, with autoimmune mechanisms and risk factors like genetics, overcrowding, and poverty playing major roles.
• Treatment: Centers on antibiotics, anti-inflammatories, supportive care, and long-term prophylaxis to prevent recurrence; surgical intervention for severe heart damage.

By understanding and acting on these principles, healthcare providers and communities can work together to reduce the burden of rheumatic fever and its devastating complications.