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Rheumatic fever

Rheumatic fever is an inflammatory disease that can develop as a complication of a streptococcal throat infection (such as strep throat) caused by the bacteria Streptococcus pyogenes. It primarily affects children between the ages of 5 and 15 years but can also occur in adults. If left untreated, the condition can cause serious damage to the heart, joints, skin, and brain.

Rheumatic fever is an inflammatory disease that can develop as a complication of a streptococcal throat infection (such as strep throat) caused by the bacteria Streptococcus pyogenes. It primarily affects children between the ages of 5 and 15 years but can also occur in adults. If left untreated, the condition can cause serious damage to the heart, joints, skin, and brain.

Key Features of Rheumatic Fever:

  1. Autoimmune Reaction: Rheumatic fever occurs as a result of an autoimmune response where the body’s immune system mistakenly attacks its own tissues after fighting a strep infection. The antibodies produced to fight the bacteria can cross-react with the body's tissues, leading to inflammation and damage.
  2. Affects Multiple Organs: Rheumatic fever can affect several organs, most notably the heart (leading to rheumatic heart disease), joints, nervous system, and skin.

Symptoms:

The symptoms of rheumatic fever can vary, but they typically appear 2 to 4 weeks after a strep throat infection and may include:

  1. Carditis (Heart Involvement):
    • Rheumatic heart disease is the most serious complication, where the heart valves are damaged, leading to long-term heart problems.
    • Symptoms include shortness of breath, fatigue, chest pain, and palpitations.
    • In severe cases, the heart valves can become scarred and narrowed, leading to heart failure.
  2. Migratory Arthritis (Joint Involvement):
    • Pain and swelling in large joints, such as the knees, ankles, elbows, and wrists, often shifting from one joint to another.
    • The arthritis tends to be temporary and resolves within a few weeks.
  3. Erythema Marginatum (Skin Rash):
    • A distinctive, non-itchy rash with raised, red borders and pale centers, often found on the trunk, arms, and thighs.
    • The rash is usually not painful or itchy, and it may come and go.
  4. Subcutaneous Nodules:
    • Small, firm, painless lumps or nodules under the skin, often over bones or joints.
    • These nodules typically resolve on their own over time.
  5. Chorea (Sydenham's Chorea):
    • A neurological manifestation of rheumatic fever, characterized by involuntary, jerky movements (also known as St. Vitus' dance).
    • Chorea can affect the face, hands, and feet and may cause difficulty in speaking, swallowing, and walking.
    • This is typically temporary, but in some cases, it may last for months.
  6. Fever and Malaise:
    • Fever is one of the hallmark symptoms, often accompanied by fatigue, weakness, and a general feeling of being unwell.

Causes:

Rheumatic fever is caused by a Group A streptococcal infection, usually a throat infection (strep throat). The body’s immune response to the infection triggers inflammation in the body. While strep throat is common, only some individuals, particularly those who are not treated with antibiotics, develop rheumatic fever as a complication.

Diagnosis:

The diagnosis of rheumatic fever is made based on a combination of symptoms, physical examination, and laboratory tests:

  1. Jones Criteria: The diagnosis of rheumatic fever is often based on the Jones criteria, which lists major and minor criteria. A diagnosis is typically made if there is evidence of a recent strep infection (confirmed by a throat culture or rapid strep test) and at least two major criteria or one major and two minor criteria.
  2. Major Criteria include:
    • Carditis
    • Migratory arthritis
    • Erythema marginatum
    • Subcutaneous nodules
    • Chorea
  3. Minor Criteria include:
    • Fever
    • Arthralgia (joint pain without swelling)
    • Elevated inflammatory markers (such as ESR and CRP)
    • Prolonged PR interval on ECG (suggesting heart involvement)
  4. Throat Culture or Rapid Strep Test: To confirm the presence of a recent strep infection.
  5. Echocardiogram: An ultrasound of the heart to assess if there is any damage to the heart valves, which is a key feature of rheumatic heart disease.
  6. Blood Tests: These may show elevated levels of inflammation markers (such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR)), and a test for antistreptolysin O (ASO) antibodies, which can indicate a recent strep infection.

Treatment:

The treatment of rheumatic fever focuses on eliminating the strep infection, controlling inflammation, and preventing complications, particularly rheumatic heart disease.

  1. Antibiotics:
    • Penicillin or another antibiotic is given to eliminate the streptococcal bacteria, usually for a prolonged period to prevent future strep infections and recurrences of rheumatic fever.
    • In some cases, azithromycin or other antibiotics are used for patients allergic to penicillin.
  2. Anti-inflammatory Medications:
    • Aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs) may be used to reduce inflammation, especially for joint pain and carditis.
    • In more severe cases of heart involvement, corticosteroids (e.g., prednisone) may be used.
  3. Chorea Treatment:
    • If chorea is present, medications such as haloperidol or diazepam may be used to control involuntary movements.
  4. Prolonged Antibiotic Prophylaxis:
    • To prevent recurrent episodes of rheumatic fever, patients may need to receive long-term antibiotics, often penicillin, for several years, depending on the severity of the disease and the presence of heart damage.

Complications:

  1. Rheumatic Heart Disease: This is the most serious complication, where inflammation and scarring of the heart valves occur, leading to long-term heart problems. Over time, this can cause valvular stenosis (narrowing) or regurgitation (leakage), leading to heart failure or other cardiovascular issues.
  2. Chronic Arthritis: Some individuals may experience recurring arthritis or joint pain.
  3. Increased Risk of Recurrent Infections: Without proper treatment, recurrent strep throat infections can increase the risk of further episodes of rheumatic fever, leading to worsening damage to the heart and other organs.

Prevention:

Rheumatic fever is largely preventable by promptly treating strep throat with antibiotics. It is essential to ensure that children and individuals with a strep throat infection receive the appropriate treatment to prevent the development of rheumatic fever. If someone has had rheumatic fever in the past, long-term antibiotics may be recommended to prevent future infections.

Prognosis:

The prognosis for rheumatic fever depends on the severity of the disease and the organs affected. Many children recover fully with appropriate treatment, but in severe cases where the heart is involved, the risk of rheumatic heart disease and long-term complications is significant. Early detection and treatment are key to preventing these complications.

If you suspect rheumatic fever or if you've had a recent strep throat infection followed by symptoms such as joint pain, skin rashes, or difficulty breathing, it's important to seek medical attention promptly for appropriate diagnosis and treatment.