By Santa J. Bartholomew M.D. FAAP, FCCM
The incidence of ARF is 8-51/100,000 people worldwide. It most commonly affects children ages 5-15 years after having a Group A Streptococcal infection (Strep throat). Overcrowding and poverty are directly proportional to the incidence of ARF as strep can sweep through a community and untreated can lead to acute rheumatic fever. The incidence of untreated Group A strep leading to Acute Rheumatic Fever is approximately 3% and recurrence is 50% in children who have had ARF. Timely treatment of strep throat can prevent acute rheumatic fever and is the most effective way to prevent all rheumatic heart disease (RHD).
Pathophysiology
Group A Strep (GAS) is also known as streptococcus pyogenes is a gram-positive bacterium with more than 100 different serotypes but only serotype B causes disease. A protein on the outside of the cell called Protein M helps the bacterium cling to the skin of the host (typically a child) and cause illness. This protein causes a large inflammatory response and the clinical symptoms that the children experience. The immune response causes the child’s body to make proteins that can attack the muscle of the heart. This usually occurs 10 days to 5 weeks after the Group A strep pharyngitis.
The immune response causes the child’s body to make proteins that can attack the muscle of the heart.
Clinical Diagnosis
GAS pharyngitis (strep throat) rarely affects children younger than 3 years old. Typically, children experience a sore throat, fever and headache. Some children may have nausea, vomiting and abdominal pain. On physical examination the physician may find exudate in the throat and on the tonsils but not always, redness or erythema. There are often swollen lymph nodes in the neck and sometimes petechiae on the soft portion of the palate. The uvula at the back of throat may be red and swollen. There maybe a fine red, sandpaper like rash over the child’s skin. It is also helpful to know if the child was near another recently diagnosed with strep throat.
Evidence of GAS infection can be obtained by drawing blood for titers in the blood to group A strep, in the acute setting throat swabs can help make the diagnosis of strep throat and thus quick treatment initiated avoiding ARF.
Diagnosis of ARF
Acute rheumatic fever is diagnosed using modified Jones criteria that were fist published in 1944 and have undergone multiple revisions through the years the last time in 2015 and are still used today.
The initial diagnosis of ARF is positive if there are 2 major criteria or 1 major and 2 minor criteria present along with evidence of persistent streptococcal infection. Further the presence of Sydenham Chorea is by itself enough to diagnose acute rheumatic fever. Sydenham chorea is a movement disorder in which the child has purposeless involuntary movements that disappear when the child is asleep. Face and hands are mostly involved and can only be suppressed very briefly. Children have deterioration in their handwriting, they can have slurred speech and emotional lability.
The Modified Jones Criteria (2015)
| Major Criteria | Minor Criteria |
|---|---|
| Carditis-Clinical and/or subclinical | Polyarthalgia |
| Arthritis Polyarthritis only | Fever (≥ 38.5ºC) |
| Chorea | ESR ≥ 60 mm/h and/or CRP ≥ 3.0 mg/dL |
| Subcutaneous nodules | Prolonged PR interval after accounting for age variability |
Erythema marginatum
| Major Criteria | Minor Criteria |
|---|---|
| Carditis-Clinical and/or subclinical | Monocarthralgia |
| Arthritis Monoarthritis or polyarthritis Polyarthralgia | Fever (≥ 38ºC) |
| Chorea | ESR ≥ 30 mm/h and/or CRP ≥ 3.0 mg/dL |
| Subcutaneous nodules | Prolonged PR interval after accounting for age variability |
Erythema marginatum
CRP, C-reactive protein: ESR, erythrocyte sedimentation rate. For all patient populations with evidence of preceding Group A streptococcal infection. Diagnosis of initial ARF: 2 major manifestations or 1 major plus 2 minor manifestations.
Treatment
Primary Treatment
Prevention of the primary streptococcal infection to avoid ARF completely with the use of oral penicillin or IM doses of penicillin G, or like antibiotics. Prevention of repeated GAS infections is the most efficient way to prevent progression of ARF to rheumatic fever.
Secondary Treatment
Supportive and is not based on robust clinical trials, the mainstay is anti-inflammatory agents that will help with joint and cardiac inflammation but to date there is little evidence that these change outcome.
- ARTHRITIS
- Ambulatory restriction for arthritis for 6 weeks
- High dose aspirin for typically 1-4 weeks
- On occasion corticosteroids are used for persistent inflammation
- ACUTE CARDITIS
- Restricted activity for 4-6 weeks
- Aspirin
- Prednisone and/or Immunoglobulin in severe cases
- Treatment of heart failure associated with carditis
- Surgical procedures for valve problems associated with severe RHD.
- SYDENHAM CHOREA
- Usually resolves spontaneously in 4-5 weeks
- Dopamine antagonists have been used with some success.
- Carbamazepine and Valproate have been used for prolonged movement problems.
ARF and RHD continue to be a major health problem in the United States and around the world. The most common clinical features of ARF are arthritis, chorea and carditis. ARF can be controlled by a combination of prevention, prompt recognition and treatment of Strep infections and improving access to health care.
