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Acute rheumatic fever

Last updated: June 23, 2021

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Acute rheumatic fever (ARF) is an inflammatory sequela involving the heart, joints, skin, and central nervous system (CNS) that occurs two to four weeks after an untreated infection with group A Streptococcus (GAS). The pathogenic mechanisms that cause rheumatic fever are not completely understood, but molecular mimicry between streptococcal M protein and human cardiac myosin proteins is thought to play a role. Because of the structural similarities between the two proteins, antibodies and T cells activated to respond to streptococcal proteins also react with the human proteins, causing tissue injury and inflammation. In addition to nonspecific symptoms (e.g., fever, malaise, and fatigue), patients present with symptoms involving the heart (carditis or valvulitis), joints (migratory polyarthritis), skin (subcutaneous nodules, erythema marginatum), and/or CNS (Sydenham chorea). The diagnosis of ARF is primarily clinical and based on the Jones criteria. Diagnostic evaluation in ARF typically shows elevated inflammatory markers, positive antistreptococcal antibodies, and valvular damage on echocardiogram. The first-line treatment is penicillin combined with symptomatic anti-inflammatory treatment, typically with salicylates or glucocorticoids (if salicylates are not effective). ARF may be complicated by progressive, permanent damage to the heart valves (especially the mitral valve), resulting in chronic rheumatic heart disease. Preventing the cardiac complications of ARF is the main goal of both primary prophylaxis (i.e., antibiotic therapy for GAS pharyngitis) and secondary prophylaxis (antibiotic administration following an episode of ARF).

  • Delayed inflammatory complication of group A β-hemolytic streptococcal pharyngitis that usually occurs within 2–4 weeks of acute infection [1]
  • One of the nonsuppurative complications of GAS pharyngitis
  • Rheumatic heart disease refers to two clinical entities:
    • Acute pancarditis as a sequela of GAS infection
    • Chronic cardiac valvular changes as a complication of acute rheumatic fever
  • Peak incidence: 5–15 years of age [2]
  • Prevalence: more common in resource-limited countries [3]

Epidemiological data refers to the US, unless otherwise specified.

Rheumatic heart disease tends to involve the high-pressure valves (i.e., the mitral and aortic valves).

The symptoms of acute rheumatic fever can be remembered by reading the JONES criteria (see “Diagnostics” below) as an acronym that replaces the “o” with a heart: J = Joints, ♥ = Pancarditis, N = Nodules, E = Erythema marginatum, S = Sydenham chorea

Approach [10][11]

Revised Jones criteria [10][11]

  • Diagnostic criteria for patients with laboratory findings of a preceding GAS infection
    • Initial episode of ARF: two major criteria or one major plus two minor criteria
    • Recurrent ARF: either the same as for an initial episode of ARF or the presence of three minor criteria
Revised Jones criteria for the diagnosis of ARF

Low-risk populations

Moderate- to high-risk populations
Major criteria
Minor criteria

Routine laboratory studies [10]

Initial laboratory findings typically show nonspecific signs of infection.

Confirmation of GAS infection [10]

Assessment for cardiac involvement [10][13][14]

Cardiac workup for ARF
Modality Characteristic findings [12][13][14]
ECG
Echocardiography
Chest x-ray

Assessment for neurological involvement [16][17]

  • The diagnosis of Sydenham chorea is based on clinical and laboratory findings.
  • Neuroimaging (MRI or CT brain)
    • Not routinely indicated but may be performed to exclude other forms of chorea
    • Findings are nonspecific and variable.
  • Lumbar puncture: not routinely indicated; may be performed to exclude other disorders
  • Echocardiography should be obtained in all patients with Sydenham chorea because concurrent cardiac involvement is common. [13]

Treatment of ARF involves pain relief for arthritis, antibiotics to both treat acute GAS infection and prevent recurrence, and medical management of associated complications.

GAS eradication [13]

Symptomatic treatment of arthritis and fever

Cardiac involvement

Management of cardiac complications in ARF [13][18]
Cardiac complication Acute management Follow-up (cardiology evaluation and echocardiography)
Mild to moderate
  • Mild: every 1–3 years
  • Moderate: annually
Severe
  • At least every 6 months
  • Consider valve repair/replacement once acute disease has resolved.

Sydenham chorea [13][18]

In the majority of cases, Sydenham chorea is self-limiting, with most patients seeing an improvement within a few weeks and nearly all patients fully recovered by six months.

  • Supportive therapy: indicated for all patients; may be the only treatment required in mild disease
    • Rest in a calm environment
    • Avoidance of overstimulation
    • Patient and carer education about the condition
  • Pharmacotherapy

Primary prevention [20]

Secondary prevention

  • Patients with a history of ARF are at high risk for recurrence, which may worsen existing rheumatic heart disease.
  • All patients require antibiotic prophylaxis.
    • Start immediately after completion of antibiotics for ARF.
    • Drug of choice: IM penicillin G benzathine every 4 weeks
    • Alternatively: oral penicillin V [13]
    • Patients with penicillin allergy: sulfadiazine
    • Antibiotic prophylaxis should be decided according to whichever of the following results in the longest treatment duration: [20]
      • Possible ARF: 12 months [13]
      • Rheumatic fever without carditis: 5 years or until the patient reaches 21 years of age
      • Rheumatic fever with carditis (with no residual heart disease): 10 years or until the patient reaches 21 years of age
      • Rheumatic fever with carditis and permanent valvular heart defects: 10 years or until the patient reaches 40 years of age
  • Educate patients on early recognition of symptoms and the importance of adhering to prophylaxis.
  • Emphasize the importance of dental care and regular dental checkups.
  • Cardiac involvement is the most important prognostic factor.
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