Rheumatic Fever

Rheumatic fever is an inflammatory disease that can occur due to an untreated infection caused by the group A Streptococcus bacterium. This condition triggers an autoimmune response, where the body's immune system mistakenly attacks its tissues, leading to inflammation in different body areas. While rheumatic fever can affect anyone, it is most commonly seen in children between the ages of 5 and 15.

What is Rheumatic Fever?

Rheumatic fever is a serious complication that can arise from untreated streptococcal infections, such as strep throat or scarlet fever. The disease is characterised by an autoimmune response, where the body's immune system inadvertently attacks healthy tissues, causing inflammation. This inflammation can affect various parts of the body, particularly the joints, heart, skin, and brain. Prompt treatment of streptococcal infections is crucial in preventing the development of rheumatic fever.

Although rheumatic fever is not categorised into distinct types, it can manifest in various ways depending on the body systems affected. Some of the types of rheumatic fever include:

Arthritis

Joint pain and inflammation are common manifestations of arthritis, often affecting the knees, ankles, elbows, and wrists.

Carditis

Carditis is the inflammation of the heart, which can lead to rheumatic heart disease (RHD) if not properly managed.

Sydenham's Chorea

This condition is characterised by jerky, uncontrollable movements, particularly of the face, hands, and feet.

Erythema Marginatum

Erythema marginatum is a rare rash that can appear as pink rings with clear centres.

Subcutaneous Nodules

Subcutaneous nodules are small, painless lumps under the skin near the joints.

Recognising these different manifestations of the disease is essential for accurate diagnosis and appropriate rheumatic fever treatment, which can help prevent the development of long-term complications, such as RHD.

The early signs of rheumatic fever include:

• Sudden onset of symptoms 1 to 6 weeks after an untreated strep throat infection

• High fever

• Painful and inflamed joints, especially in the knees, elbows, ankles, and wrists

• Raised, red skin rashes on the chest, back, or stomach

• Small nodules (bumps) under the skin over affected joints

Rheumatic fever can present with a range of symptoms affecting various parts of the body, including the joints, heart, skin, and nervous system.

Joint Pain and Swelling

The most common symptoms include joint pain and swelling, where the joints become painful, warm, swollen, and red. The pain often migrates from one joint to another, a condition known as migratory arthritis.

High Fever

A high fever is another frequent symptom of rheumatic fever.

Carditis

In some cases, inflammation of the heart (carditis) can occur, causing chest pain, palpitations, and shortness of breath.

Skin Manifestations

These may include a raised, red rash called erythema marginatum or small nodules under the skin.

Sydenham's Chorea

This is a nervous system disorder characterised by jerky, uncontrollable movements of the face, hands, and feet.

Other Symptoms

Other general symptoms may include fatigue, unexplained weight loss, and abdominal pain.

While rheumatic fever does not have distinct stages in the traditional sense, the progression of the disease can be understood in terms of its development and potential complications:

Initial Infection

The onset of rheumatic fever typically occurs 1 to 5 weeks after an untreated streptococcal infection, such as strep throat or scarlet fever.

Onset of Symptoms

Symptoms can include fever, painful and inflamed joints (arthritis), fatigue, and sometimes a rash or nodules under the skin. Heart-related symptoms can also occur, including chest pain and shortness of breath.

Potential for Complications

If not managed promptly, rheumatic fever can lead to serious complications, including RHD, which can weaken the heart valves and lead to heart failure or other cardiac issues.

Understanding these stages can help in the early detection and management of rheumatic fever.

Rheumatic fever is a serious complication that can develop following an untreated strep throat infection caused by group A Streptococcus bacteria. When the body's immune system responds to the streptococcal infection, it may mistakenly attack healthy tissues, leading to widespread inflammation in the joints, heart, skin, and nervous system.

The primary rheumatic fever cause is the failure to manage strep throat infections promptly and effectively with antibiotics. As the immune system produces antibodies to fight the infection, these antibodies can cross-react with the body's own tissues, causing an autoimmune response that leads to inflammation and damage. This autoimmune reaction triggered by the streptococcal infection is the underlying mechanism responsible for the development of rheumatic fever.

A person's age, living conditions, and genetic predisposition are a few factors that influence the risk of getting the disease.

Who's at Risk?

• Children aged 5 to 15 are most susceptible to developing rheumatic fever

• Individuals living in overcrowded or unsanitary conditions

• Those with a family history of rheumatic fever

• People in developing countries with limited access to healthcare and antibiotics

• Individuals with untreated or recurrent strep throat infections

Rheumatic fever can lead to several serious and long-lasting complications, particularly affecting the heart and other body systems. Some of these complications include:

Rheumatic Heart Disease

It is the most common and severe complication involving scarring and thickening of the heart valves, which can lead to narrowed or leaky valves (valve stenosis or regurgitation). This can result in heart failure as the heart has to work harder to pump blood.

Valve Damage

This is another complication, with stenosis reducing blood flow and regurgitation, causing backflow of blood across the valve due to improper closure. The mitral valve is most commonly affected, but other valves can also be involved.

Other Cardiac Complications

Other complications may include heart muscle damage, heart failure, and atrial fibrillation (AFib).

Neurological Complications

Conditions such as Sydenham's chorea (St. Vitus' Dance) can cause rapid, jerky movements, usually on one side of the body, leading to uncontrollable twitching, muscle weakness, and emotional outbursts.

Musculoskeletal Complications

Diseases like Jaccoud arthropathy affect the fingers, toes and wrists, causing arthritic-like symptoms and increased risk of dislocation.

Other Complications

These include an increased risk of stroke, pulmonary hypertension, fluid surrounding the heart (pericardial effusion), and an enlarged heart.

Prompt diagnosis and treatment are crucial to prevent these potentially life-threatening complications.

Prevention of rheumatic fever is primarily focused on managing the underlying strep throat infection promptly.

• Managing strep throat with antibiotics as soon as it is diagnosed can prevent the development of rheumatic fever.

• Environmental and public health measures such as reducing overcrowding and improving sanitation can help prevent the spread of strep bacteria.

• For individuals who have had rheumatic fever, long-term antibiotic prophylaxis may be recommended to prevent recurrent strep infections and reduce the risk of further complications. However, it is important to adhere to the prescribed antibiotic regimen for effective prevention.

Diagnosing rheumatic fever involves a combination of clinical assessment, laboratory tests, and imaging studies. The diagnosis is based on the modified Jones criteria, which consider major and minor manifestations along with evidence of a recent strep infection.

Major manifestations include:

• Carditis (inflammation of the heart)

• Polyarthritis (inflammation of multiple joints)

• Chorea (neurological disorder causing jerky movements)

• Erythema marginatum (distinctive rash)

• Subcutaneous nodules (lumps under the skin)

Minor manifestations include:

• Fever

• Arthralgia (joint pain)

• Elevated inflammatory markers [erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)]

• Prolonged PR interval on electrocardiogram (ECG)

Evidence of a recent strep infection is confirmed through:

• Positive throat culture for group A Streptococcus

• Elevated or rising antistreptococcal antibody titre

Additional diagnostic tests may include echocardiography to assess heart valve function and detect any signs of RHD. Early and accurate diagnosis is essential for timely treatment and prevention of complications.

The management of rheumatic fever involves a multifaceted approach aimed at managing the acute symptoms, eradicating the strep infection, and preventing recurrences and long-term complications.

1. Antibiotic Therapy

   • Penicillin or amoxicillin to eradicate the strep infection

   • Long-term prophylaxis with penicillin or erythromycin to prevent recurrent infections

2. Anti-inflammatory Medications

   • Aspirin or naproxen to reduce inflammation and relieve joint pain and fever

   • Corticosteroids (e.g. prednisone) for severe carditis or chorea

3. Supportive Care

   • Bed rest during the acute phase to reduce strain on the heart

   • Monitoring of heart function with ECG and echocardiography

   • Management of heart failure symptoms if present

4. Anticonvulsant Medications

   • Valproic acid or carbamazepine for chorea

5. Surgical Interventions

   • Valve repair or replacement surgery may be necessary for severe RHD

6. Long-term Follow-up

   • Regular check-ups to monitor heart function and valve status

   • Continued antibiotic prophylaxis to prevent recurrences

   • Dental hygiene to prevent bacterial endocarditis

Medication for Rheumatic Fever

The treatment of rheumatic fever involves several medications targeting different aspects of the disease.

• To eradicate the inciting streptococcal infection, benzathine benzylpenicillin G (BPG) is the preferred option.

• Alternatively, phenoxymethylpenicillin can be given orally.

• For patients with penicillin hypersensitivity, erythromycin is prescribed orally.

• To manage the symptoms of arthritis and arthralgia, naproxen is preferred due to its safety profile and convenience.

• Ibuprofen is also well-tolerated, although there is less experience with its use in acute rheumatic fever compared to naproxen.

• For initial analgesia, paracetamol (acetaminophen) is preferred during diagnostic uncertainty to avoid masking symptoms.

• Tramadol can also be used cautiously in children under 12 years due to variable metabolism.

A comprehensive treatment plan tailored to the individual patient's needs is crucial for optimal outcomes and prevention of long-term sequelae of rheumatic fever.

If you or your child experience symptoms suggestive of rheumatic fever, such as joint pain, fever, chest pain, or uncontrolled movements, it is essential to seek medical attention promptly. Early diagnosis and treatment are crucial in preventing the progression of the disease and potential long-term complications, such as RHD.

Your doctor will perform a thorough physical examination and may order tests, including blood tests, ECG, and echocardiogram, to confirm the diagnosis of rheumatic fever and assess the extent of any cardiac involvement. If diagnosed with the disease, your doctor will prescribe appropriate rheumatic fever medications and advise on the necessary follow-up care and preventive measures to reduce the risk of recurrent episodes.

• Rheumatic fever is an inflammatory disease that can develop as a complication of untreated or inadequately treated strep throat or scarlet fever.

• Common symptoms of rheumatic fever include fever, joint pain, chest pain, and uncontrolled movements (Sydenham's chorea).

• Diagnosis of rheumatic fever is based on a combination of signs, symptoms, and test results, including blood tests, ECG, and echocardiogram.

• Treatment of rheumatic fever involves antibiotics to eradicate the streptococcal infection, anti-inflammatory medications to manage joint symptoms, and supportive care for other manifestations.

• Regular follow-up care and adherence to preventive measures, including long-term antibiotic prophylaxis, are crucial to reduce the risk of recurrent episodes and further damage to the heart valves.