Rheumatic fever

Overview

Rheumatic fever, an inflammatory disease impacting the brain, skin, joints, and heart, is a rare complication of untreated group A streptococci bacterial infections. This autoimmune response triggers the immune system to mistakenly target its tissues, causing inflammation in organs like the heart, joints, skin, and central nervous system.Symptoms include high fever, sore joints, chest pain, and fatigue. While these symptoms often resolve spontaneously, there's a risk of lasting damage to the heart, leading to rheumatic heart disease.Rheumatic fever risk factors include a recent untreated or poorly treated strep throat infection, especially in children aged 5-15, and living in crowded or low-income conditions where such infections are more common. Repeated strep infections increase the likelihood of developing rheumatic fever.The primary treatment for rheumatic fever involves antibiotics. Additionally, anti-inflammatory medications or corticosteroids may be prescribed to reduce inflammation and manage symptoms.

Key Facts

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Symptoms

Symptoms usually appear 1 to 5 weeks after a bacterial throat infection and may vary among individuals. Common symptoms include:

  • Sore throat
  • Swollen, red tonsils
  • Fever
  • Joint and muscle pain, particularly in the knees and ankles
  • Chest pain
  • Shortness of breath
  • Fatigue
  • Small painless lumps under the skin
  • Jerky, uncontrollable body movements (Sydenham chorea), most often in the hands, feet, and face

In severe cases:

  • Large joints
  • Heart failure
  • Behavioral disturbances

Know the difference: Scarlet fever vs Rheumatic fever

Scarlet fever is characterized by a pink, bumpy rash due to a group A streptococci infection in the throat or skin and is usually treated with antibiotics. However, if scarlet fever is not effectively treated, it may progress into rheumatic fever, a more serious condition.

Cause

Rheumatic fever can stem from untreated throat or skin infections, primarily caused by group A Streptococcus bacteria, including:

  • Strep throat
  • Scarlet fever
  • Impetigo

The immune system, responding to the bacterial infection, may mistakenly attack healthy tissues, leading to inflammation and potential damage to the heart, joints, skin, and organs. If the antibodies attack the heart, they can cause the heart valves to swell, which may result in scarring of the valve "doors" (called leaflets or cusps).

Certain risk factors can increase your likelihood of developing rheumatic fever, as discussed in the next section.

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RiskFactors

Rheumatic fever is now relatively rare in developed countries, thanks to the widespread use of antibiotics to treat streptococcal infections. The risk factors for rheumatic fever include:

  • Strep throat infection: The primary risk factor for rheumatic fever is an untreated or inadequately treated streptococcal throat infection (strep throat). Certain strains of streptococcal bacteria, particularly Group A Streptococcus, are more likely to lead to rheumatic fever.
  • Demographic factors:
    • Age
    • Gender
    • Family history

    Note: Rheumatic fever is most common in children between the ages of 5 and 15. It is also more common in females, with a relative risk of 1.6 to 2.0 compared with males.

  • Genetic predisposition: Both a person's vulnerability and the specific genetics of streptococcal bacteria play important roles in how these bacteria interact with the body, increasing the risk of infection.
  • Environmental factors:
    • Low socioeconomic status
    • Poor sanitation
    • Crowded living conditions
    • Limited access to healthcare
    • Undernutrition

Diagnosis

The revised Jones criteria serve as a guiding framework for rheumatic fever diagnosis, incorporating diverse clinical and laboratory findings. It includes:

Revised Jones Criteria

To make a diagnosis, there must be evidence of recent streptococcal infection (confirmed by throat culture or rapid strep test) along with the presence of two major criteria or one major criterion and two minor criteria. The major and minor criteria include:

Major Criteria:

  • Carditis (inflammation of the heart)
  • Polyarthritis (inflammation of multiple joints)
  • Chorea (involuntary movements)
  • Erythema marginatum (a specific type of skin rash)
  • Subcutaneous nodules (small, painless nodules under the skin)

Minor Criteria:

  • Fever
  • Arthralgia (joint pain)
  • Elevated acute phase reactants (such as elevated CRP or ESR)
  • Prolonged PR interval on ECG

To make a diagnosis of rheumatic fever, there should be either:

  1. Two major criteria, or
  2. One major criterion and two minor criteria, along with evidence of recent streptococcal infection.

Other key aspects of the diagnostic process include:

1. Clinical Evaluation

  • Medical History: The doctor will review the patient's medical history, paying attention to recent or past episodes of streptococcal infection, especially strep throat.
  • Physical Examination: A thorough physical examination will be conducted to assess symptoms and look for signs of rheumatic fever.

2. Blood Tests

  • Inflammatory Markers: Blood tests like C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) are commonly used to detect signs of inflammation in the body. Elevated levels of these markers may indicate ongoing inflammation.
  • Antistreptolysin O (ASO) Titer: This blood test measures the level of antibodies (specifically antistreptolysin O) produced in response to a recent streptococcal infection. Elevated levels may suggest a recent or ongoing strep infection.

3. Throat Cultures

Throat cultures may be performed to identify the presence of streptococcal bacteria. However, it's important to note that the bacteria might no longer be present in the throat by the time symptoms of rheumatic fever develop. This is why antibody tests, like the ASO titer, are often used.

4. Imaging Tests

  • Electrocardiogram (ECG or EKG): It helps identify abnormal heart rhythms (arrhythmias) and can indicate if there is any damage or enlargement of the heart.
  • Echocardiogram: This test can reveal abnormalities in the heart's structure and function, including inflammation of the heart muscle (myocarditis) or damage to the heart valves.
  • Chest X-rays: These may be conducted to assess the extent of cardiac involvement or to rule out other conditions.

Prevention

Preventing rheumatic fever primarily involves prompt and effective treatment of streptococcal infections (GAS infections), particularly strep throat. Here are some preventive tips:

  • Consider prophylactic antibiotics: High-risk individuals are typically recommended monthly injections of penicillin to prevent rheumatic fever.
  • Do not take strep throat lightly: Strep throat is typically treated with antibiotics (such as penicillin or amoxicillin). Completing the full course of prescribed antibiotics is crucial to eliminate the bacteria.
  • Practice good hygiene:
    • Encourage regular handwashing to prevent the spread of infections.
    • Avoid sharing utensils, drinking glasses, or personal items that may harbor bacteria.
  • Maintain a healthy lifestyle: Encourage a healthy lifestyle with a balanced diet and regular physical activity to promote overall well-being.
  • Foods to include:
    • Fish rich in omega-3 fatty acids
    • Whole grains
    • Fruits and vegetables
    • Lean proteins
    • Low-fat dairy
    • Nuts and seeds
  • Foods to limit/eliminate:
    • Processed foods
    • Excessive salt
    • Sugary beverages
    • Saturated and trans fats
  • Supplements that can help:
    • Omega-3 fish oil
    • Vitamin D
    • Calcium
    • Magnesium
  • Create awareness: Educate people, especially parents and caregivers, about the symptoms of strep throat and the importance of seeking medical attention.

Treatment

The treatment of rheumatic fever aims to address the infection, alleviate symptoms, manage inflammation, and prevent recurrence. The following approaches are commonly employed:

1. Medications

  • Antibiotics: These are the first choice to treat streptococcal throat infections. Penicillin is the most commonly used drug, which includes:
    • Penicillin V (Phenoxymethylpenicillin)
    • Penicillin G (Benzylpenicillin)
  • Anti-inflammatory drugs: Helps to reduce inflammation, fever, and pain. Commonly used medications include:
    • Ibuprofen
    • Naproxen
    • Aspirin
  • Note: Do not administer aspirin to a child without specific instructions from a doctor. It is avoided in children due to the risk of Reye's syndrome, a rare but serious condition that causes swelling in the liver and brain, often linked to aspirin use in children recovering from viral infections.
  • Corticosteroids: Prescribed if the patient does not respond to initial anti-inflammatory medications or if there is inflammation of the heart. Prednisone is most commonly used.
  • Anti-seizure medications: These medicines are prescribed to manage severe involuntary movements associated with Sydenham chorea. Examples include:
    • Valproic Acid
    • Carbamazepine
    • Haloperidol
    • Risperidone

2. Long-term medication for heart support

Drug options for managing heart failure may include using angiotensin-converting enzyme (ACE) inhibitors (like Lisinopril and Enalapril) and taking diuretics (like Furosemide and Spironolactone).

Depending on the severity, heart surgery may be required to repair or replace damaged valves.

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3. Supportive care

Bed rest is often recommended during the acute phase of rheumatic fever, especially when the heart is involved.

Adequate hydration is essential, and maintaining a balanced and healthy diet can support the recovery process.

HomeCare

Home remedies should complement conventional treatments, and their effectiveness and safety may vary. Always consult a doctor before considering anything new. Some herbal supplements that can help alleviate symptoms include:

  • Ginger (Adrak): Has anti-inflammatory and analgesic effects. It may help manage pain and inflammation associated with rheumatic fever.
  • How to use: Incorporate ginger into your diet through ginger tea or add it to your meals like curry or stir-fry.

  • Garlic (Lehsun): Known for its anti-inflammatory properties and is a natural source of quercetin, an antihistamine.
  • How to use: Consuming raw or crushed garlic a month or two before hay fever season may help reduce symptoms.

  • Turmeric (Haldi): Contains curcumin, known for its anti-inflammatory properties. It may help reduce inflammation and alleviate symptoms.
  • How to use: It can be consumed by adding it to warm milk or incorporating it into your meals.

  • Willow bark: Contains salicin, a compound similar to aspirin, which may help with pain relief and inflammation.
  • How to use: It can be used in capsule form, following recommended dosages.

    Note: Avoid if you have allergies to aspirin or are taking blood-thinning medications, and consult with a doctor.

  • Green tea: Rich in antioxidants, green tea may have anti-inflammatory properties that could potentially be beneficial.
  • How to use: Enjoy as a beverage or take green tea extract supplements.

  • Cinnamon (Dalchini): Has anti-inflammatory properties and may offer mild relief in managing symptoms of rheumatic fever.
  • How to use: Sprinkle on foods like oatmeal, yogurt, or tea for flavor and potential health benefits.

Remember! Prioritize ample rest, hydration, and a balanced diet with prescribed pain relievers for recovery.

Complications

Rheumatic fever can lead to several complications, particularly if the initial streptococcal infection is not promptly treated. Some of the complications include:

  • Rheumatic heart disease (RHD): A severe complication that can result in permanent damage to the heart valves, leading to long-term heart problems.
  • Heart valve damage: Rheumatic fever often targets heart valves, causing inflammation and scarring that may lead to valve stenosis (narrowing) or regurgitation (leaking).
  • Heart failure: Severe damage to heart valves can eventually lead to heart failure, where the heart is unable to pump blood effectively.
  • Atrial fibrillation: Irregular heart rhythms, such as atrial fibrillation, can occur due to the impact of rheumatic heart disease on the heart's electrical system.
  • Pulmonary hypertension: Elevated blood pressure in the main arteries of the heart, commonly associated with advanced rheumatic heart disease.
  • Embolic stroke: This can be a complication of rheumatic fever, arising from the migration of blood clots or debris formed due to heart valve damage to the brain's blood vessels.
  • Jaccoud arthropathy (joint problems): This causes joint deformities due to recurrent bouts of arthritis, potentially resulting in long-term joint damage and deformities.
  • Subcutaneous skin lesions: Erythema marginatum, a rash with characteristic borders, may appear on the trunk or arms.
  • Increased susceptibility: Greater vulnerability to recurrent episodes of rheumatic fever if exposed to streptococcal infections.
  • Sydenham's chorea: A neurological complication characterized by involuntary movements and muscle weakness.
  • Sudden cardiac death: The abrupt, unexpected loss of heart function, typically caused by an electrical disturbance in the heart.

Misconception: Rheumatic fever causes rheumatoid arthritis.
Reality: There is no direct link between rheumatic fever and rheumatoid arthritis. Although they may exhibit similar early symptoms and affect joints in children, rheumatic fever is a short-term condition lasting a few weeks, while rheumatoid arthritis is a lifelong disorder.

AlternativeTherapies

Complementary and alternative medicine (CAM) therapies may be considered for supportive care in rheumatic fever. It is important to seek medical advice before starting them. Here are some complementary measures that may be considered:

  • Acupuncture: Involves the insertion of thin needles into specific points on the body to promote balance and alleviate symptoms. It may help manage pain and inflammation.
  • Biofeedback: A technique that teaches individuals to control physiological processes such as heart rate and muscle tension. It may contribute to stress management and symptom relief.
  • Mind-body techniques: Practices such as meditation, relaxation exercises, and yoga may help manage stress and promote emotional well-being.
  • Massage therapy: May relieve muscle and joint discomfort but should be done with caution and under the guidance of a knowledgeable therapist.

Living With Disease

Managing rheumatic fever is an ongoing challenge, as exposure to streptococcal bacteria can trigger flare-ups. Managing this condition can induce stress, affecting overall well-being and quality of life, especially in children.

Coping strategies that can help

  • Seek professional guidance for tailored treatment and emotional support.
  • Collaborate with schools to address attendance and academic challenges.
  • Strictly adhere to prescribed medications for effective management.
  • Adapt daily activities to accommodate physical limitations.
  • Foster a resilient and supportive environment to mitigate the impact of rheumatic fever on a child's overall well-being.

Tips to manage rheumatic fever in children

  • Identify streptococcal triggers.
  • Encourage good hygiene practices.
  • Plan activities to avoid exposure to streptococcal infections.
  • Monitor for symptoms and seek medical attention promptly.
  • Consider prophylactic antibiotics as advised by the doctor.

Things to know about dental visits after rheumatic fever

  • Always inform any doctor, including dentists, about your child's history of rheumatic fever.
  • Ensure your child receives extra antibiotics before dental procedures to protect their heart valves.
  • Obtain a rheumatic fever wallet card from your doctor and share it with dentists or doctors before treatment.
  • Be cautious as dental work may introduce mouth bacteria to the bloodstream, potentially causing endocarditis (inflammation of the inner lining of the heart's chambers) in the heart valves.

Want to protect your child against throat infections? Read How.

References

National Organization For Rare Disease
Disease Review
2009 April 01
Autoimmune.org
2021
Jonathan R, et al.
Nature Reviews | Disease Primers
2016
Texas Heart Institute
2021
Autoimmune.org
2021
Ministry of Health and Family Welfare
Weinberg GA
MSD Manuals
2023
Centers for Disease Control and Prevention
2022
Arthritis Foundation
Chowdhury MDS, Koziatek CA, Rajnik M
StatPearls
2023 August 02
Dass C, Kanmanthareddy A
StatPearls
2023 July 25
ULUSOY H, GÜÇER TK, AKSU M, ARSLAN Ş, HABİBOĞLU A, AKGÖL G, et al.
Turkish League Against Rheumatism
1970
Centers for Disease Control and Prevention
2022

Frequently asked questions

Rheumatic fever is not inherited; it results from an immune response to a bacterial infection rather than genetic transmission. However, some individuals may have a genetic predisposition that increases their risk of developing the condition.
While rheumatic fever is more common in children, adults can also be affected, particularly if they had a previous episode in childhood. It is important for individuals of all ages to prevent and manage streptococcal infections to lower the risk of rheumatic fever.
Penicillin injections are required every 28 days for at least 10 years or until the age of 21, and potentially longer for cases involving heart valve damage.
Missing an injection increases the risk of strep throat and a recurrence of rheumatic fever. If an injection is missed, promptly contact your child's nurse to schedule the next injection as soon as possible.
Yes, it is advisable to schedule the injection early rather than late. Please coordinate with your nurse to arrange an early injection for your child.