An acute febrile illness that is characterized by

  • Fleeting pains in the joints (arthritis)
  • Inflammation of the heart muscle (carditis), and
  • Abnormal movements performed by the patient (chorea)

The condition occurs mostly in children (average age 6 to 14 years old), following streptococcal bacterial infections.

Why does this happen and how may it affect the patients health?

In essence, rheumatic fever is an immunologic response of the patients body, following an untreated, acute throat infection, caused by a streptococcal organism. The immune system of the body mistakenly attacks the patients own tissues. In particular, it causes inflammatory damage to the tissues of the heart, brain, skin and joints.

Almost half of the patients who have had acute rheumatic fever will develop it again. Prevention is possible through following a strict regime of prophylaxis (preventative measures). The regime is specifically designed to prevent recurrent infections.

There may be a genetic predisposition, making certain individuals more susceptible to rheumatic fever.

Increased incidence occurs with low standards of living and crowded conditions. The patients develop combinations of certain distinct clinical symptoms and signs, which are used as criteria for making the diagnosis. The Jones criteria for guidance in diagnosis of rheumatic fever includes combinations of the following

Major manifestations

  • Inflammation of the heart muscle (carditis)
  • Inflammation of joints (arthritis)
  • Inflammation of brain tissue (chorea)
  • Development of a skin rash (erythema marginatum)
  • Formation of nodules (lumps) under the skin

Minor manifestations

  • Previous history of rheumatic fever
  • Joint pains
  • Increased ESR
  • Increased white cell count
  • Abnormal ECG when measuring heart rhythm

Theories regarding disease development

  • Toxic effect of streptococcal organism on host tissues
  • Abnormal immune response
  • Direct infection by Streptococcus, group A

What symptoms may the patient experience?

The patient may experience combinations of symptoms and manifestations under the Jones criteria. The condition may present with variations of these symptoms and combinations.

Average age of onset is between 6 to 14 years.

Most commonly the patient will complain of painful, swollen joints that may be warm to touch. Knees are especially affected. The patient may have a sustained fever. There is a history of a throat infection within the 3 weeks before onset of joint pains and fever.

When the heart is affected

  • The heart rate may be fast
  • The heart may enlarge
  • There may be abnormal heart sounds due to valve damage, or
  • The heart sac may become inflamed (pericarditis); this may also cause a so-called pericardial friction rub

Characterised by abnormal movements of the body and limbs this condition, chorea (Sydenhams chorea) is due to effects that occur on the central nervous system.

  • Skin rash (red lesions, 2 cm in diameter; mainly occurs on the trunk - Erythema marginatum).
  • Nodules under the skin (rare).

For making the diagnosis, two major Jones criteria or one major criterion and two minor criteria including evidence of a recent streptococcal infection are required.

How is the diagnosis made and what special investigations are required?

  • Complete blood count
  • Increased white blood cell count
  • ESR (erythrocyte sedimentation rate increased)
  • Throat swab for culture and sensitivity
  • Chest X-ray
  • ECG to evaluate heart
  • Antistreptolysin-O titre (elevated)
  • C-reactive protein
  • Rapid antigen detection test
  • Anti-hyaluronidase test

What is the treatment and prognosis?

  • Bed rest
  • Aspirin
  • Penicillin V for prophylaxis to prevent recurrent attacks must be administered on a continual basis.
  • Erythromycin may also be used.
  • Steroid medications (e.g. Prednisone) may be required if the patient presents with severe carditis (accompanied by heart failure).
  • Tetrabenazine may help for chorea.

Treat any congestive heart failure with appropriate treatment.

Secondary prophylaxis (prevention)

  • Benzathine Penicillin G, intra-muscular every 4 weeks, or
  • Sulfadiazine: 1 gram daily per mouth, or
  • Penicillin V: 250 mg twice daily, per mouth

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