Rheumatic Fever

Rheumatic fever is an inflammatory disease that can develop as a complication of untreated or poorly treated strep throat caused by group A Streptococcus bacteria. Although rare in developed countries, rheumatic fever remains a significant health concern in developing nations, where poor access to healthcare and antibiotics leads to higher rates of infection. Left untreated, it can lead to severe complications, particularly rheumatic heart disease, which affects the heart valves and can cause long-term disability or death.

This blog will explore the causes, symptoms, complications, diagnosis, treatment, and prevention of rheumatic fever. It will also discuss the impact of this disease globally, with particular emphasis on its connection to poverty, poor living conditions, and limited access to healthcare.

Understanding Rheumatic Fever: Causes and Risk Factors

It is not an infection itself but rather an autoimmune response to a bacterial infection, specifically a throat infection caused by Streptococcus pyogenes, commonly known as group A Streptococcus (GAS). When this infection goes untreated or is inadequately treated, the body’s immune system may mistakenly attack its own tissues, particularly the heart, joints, skin, and brain. This misdirected immune response is what causes the symptoms of rheumatic fever.

1. Causes and Mechanism

Rheumatic fever develops as a result of untreated or improperly treated streptococcal pharyngitis (strep throat). When a person has strep throat, the immune system produces antibodies to fight the GAS bacteria. However, in some individuals, these antibodies can cross-react with the body’s tissues due to molecular mimicry. The proteins on the streptococcal bacteria are similar to those found in human tissues, particularly the heart, joints, skin, and nervous system. As a result, the immune system attacks these tissues, leading to the inflammation and damage characteristic of the disease.

2. Risk Factors

• Age: It primarily affects children between the ages of 5 and 15, although it can occur at any age.
• Socioeconomic Status: Children from low-income families or those living in overcrowded conditions are at higher risk due to increased exposure to the bacteria and limited access to medical care.
• Geographical Location: It is more common in developing countries where access to healthcare and antibiotics is limited. Poor living conditions, inadequate sanitation, and limited education about infectious diseases contribute to the higher incidence of the disease in these areas.
• Previous History: Individuals who have had rheumatic fever are at a higher risk of recurrence, especially if they are exposed to streptococcal infections again.

Rheumatic Fever symptoms

The symptoms usually appear 1 to 5 weeks after the initial strep throat infection. They can vary widely depending on the parts of the body affected, but the most common symptoms include:

1. Fever: A high-grade fever often accompanies other symptoms.

2. Joint Pain and Swelling: Polyarthritis, or inflammation of multiple joints, is a hallmark of rheumatic fever. The joints, particularly the knees, ankles, elbows, and wrists, become swollen, tender, and painful. The inflammation often migrates from one joint to another, a characteristic known as migratory arthritis.

3. Cardiac Symptoms: Rheumatic fever can lead to carditis, or inflammation of the heart. The valves of the heart, particularly the mitral and aortic valves, may become inflamed and damaged. Symptoms of carditis include chest pain, shortness of breath, and palpitations. Carditis is one of the most serious complications of rheumatic fever, as it can lead to rheumatic heart disease.

4. Chorea: Also known as Sydenham’s chorea or St. Vitus’ dance, this symptom involves involuntary, jerky movements of the arms, legs, and face. It occurs when the central nervous system is affected by the autoimmune response.

5. Skin Manifestations: Erythema marginatum, a rare but distinctive rash, can appear on the trunk and limbs. The rash is characterized by pink or red rings that spread outward while the center clears. Subcutaneous nodules, small, painless lumps under the skin, may also appear, particularly near joints.

6. Fatigue and Weakness: Generalized feelings of fatigue and weakness are common, especially in cases involving carditis.

Complications

If left untreated or inadequately managed, rheumatic fever can lead to long-term complications, primarily affecting the heart.

1. Rheumatic Heart Disease (RHD)

Rheumatic heart disease is the most serious complication of rheumatic fever. It occurs when the inflammation caused by rheumatic fever leads to scarring of the heart valves, particularly the mitral and aortic valves. This scarring can cause the valves to narrow (stenosis) or leak (regurgitation), leading to impaired blood flow through the heart. Over time, this can result in heart failure, stroke, arrhythmias, and other cardiovascular complications.

2. Chronic Arthritis

While the joint pain and inflammation caused by rheumatic fever typically resolve over time, some individuals may develop chronic arthritis, particularly if they experience recurrent episodes of rheumatic fever.

3. Neurological Complications

Sydenham’s chorea, if severe or left untreated, can result in long-term neurological problems, including coordination issues and emotional disturbances.

Rheumatic Fever criteria

Diagnosing rheumatic fever can be challenging, as its symptoms overlap with those of other conditions, and there is no single test for the disease. Instead, doctors rely on a combination of clinical criteria, laboratory tests, and patient history to make a diagnosis. The most widely used diagnostic tool is the Jones Criteria, which divides the signs and symptoms of the disease into major and minor criteria.

1. Major Criteria (According to the modified Jones Criteria):

• Carditis (inflammation of the heart)
• Polyarthritis (migratory arthritis affecting large joints)
• Chorea (involuntary movements)
• Erythema marginatum (rash)
• Subcutaneous nodules (small lumps under the skin)

2. Minor Criteria:

• Fever
• Arthralgia (joint pain without swelling)
• Elevated acute phase reactants (e.g., elevated C-reactive protein or erythrocyte sedimentation rate)
• Prolonged PR interval on electrocardiogram (ECG)

A diagnosis of rheumatic fever is usually made if two major criteria, or one major and two minor criteria, are present along with evidence of a recent streptococcal infection (e.g., a positive throat culture or elevated streptococcal antibodies).

Rheumatic Fever treatment

The primary goals of treatment for rheumatic fever are to eradicate the streptococcal infection, reduce inflammation, and prevent long-term complications, particularly rheumatic heart disease.

1. Antibiotic Therapy

Penicillin is the antibiotic of choice for treating the underlying streptococcal infection. A single injection of intramuscular benzathine penicillin is often used to ensure compliance, though oral antibiotics may also be prescribed. After the initial treatment, long-term antibiotic prophylaxis is recommended to prevent recurrent episodes of rheumatic fever. This usually involves monthly injections of benzathine penicillin or daily oral penicillin for several years, depending on the patient’s risk of recurrence.

2. Anti-inflammatory Medications

Nonsteroidal anti-inflammatory drugs (NSAIDs) such as aspirin or ibuprofen are commonly used to reduce inflammation and relieve pain associated with joint inflammation. In cases of severe carditis, corticosteroids may be prescribed to control inflammation.

3. Treatment of Cardiac Complications

Patients with carditis may require additional treatment, including diuretics or other medications to manage heart failure symptoms. In severe cases, surgical intervention, such as valve repair or replacement, may be necessary to address valve damage.

4. Supportive Care for Chorea

For patients with chorea, supportive care and medications such as anticonvulsants or sedatives may be used to manage involuntary movements.

Prevention

Preventing rheumatic fever is primarily focused on preventing streptococcal infections and ensuring prompt and adequate treatment when they occur.

1. Primary Prevention

Primary prevention involves the early recognition and treatment of strep throat with antibiotics to prevent the development of rheumatic fever. Education on the importance of seeking medical attention for sore throats, especially in children, is critical. In areas where the disease is common, mass antibiotic treatment programs may be used to reduce the prevalence of streptococcal infections.

2. Secondary Prevention

Secondary prevention involves the long-term use of antibiotics to prevent recurrent episodes of the disease in individuals who have already had the disease. This is particularly important for those who have developed rheumatic heart disease, as recurrent episodes of rheumatic fever can worsen heart valve damage.

The Global Impact of Rheumatic Fever

Rheumatic fever and rheumatic heart disease are most prevalent in developing countries, where poverty, overcrowding, and limited access to healthcare contribute to the persistence of streptococcal infections and inadequate treatment. According to the World Health Organization (WHO), an estimated 15.6 million people worldwide are affected by rheumatic heart disease, with 282,000 new cases and 233,000 deaths annually. The burden of disease falls disproportionately on children and young adults in low- and middle-income countries.

Efforts to reduce the global burden of rheumatic fever and rheumatic heart disease focus on improving access to healthcare, including the availability of antibiotics and diagnostic services. Public health initiatives aimed at reducing poverty, improving living conditions, and increasing awareness of the importance of early treatment for strep throat are also critical in reducing the incidence of this preventable disease.

Rheumatic fever is a serious and potentially life-threatening disease that primarily affects children and young adults in low-resource settings. It develops as a complication of untreated strep throat and can lead to long-term complications, particularly rheumatic heart disease. Early diagnosis and treatment of strep throat are essential to preventing rheumatic fever, and long-term antibiotic prophylaxis is necessary to prevent recurrence in those who have had the disease.

Global efforts to reduce the burden of rheumatic fever must focus on improving access to healthcare and education, particularly in developing countries where the disease remains prevalent. By addressing the root causes of poverty, overcrowding, and inadequate healthcare, we can reduce the incidence of this preventable disease and improve the lives of millions of people worldwide.