Clinical Notes : Infection and Sepsis

70. Acute Rheumatic Fever

Definition

Acute rheumatic fever (ARF) is a nonsuppurative sequela that occurs 2 - 3  weeks following group A Streptococcus (GAS) pharyngitis and may consist of

  • arthritis

  • carditis

  • chorea

  • erythema marginatum

  • subcutaneous nodules

 

Epidemiology

Most major outbreaks occur under conditions of impoverished overcrowding where access to antibiotics is limited

  • more prevalent in Middle East, the Indian subcontinent, and some areas of Africa and South America

  • as many as 20 million new cases occur each year.

 

Presentation

Inflammatory response to Group A streptococcus infection

  • manifests 2-3 weeks after throat infection

    • Approximately 70% of older children and young adults recollect the pharyngitis.

    • However, only approximately 20% of young children recollect pharyngitis.

    • Therefore, younger children who present with signs or symptoms consistent with ARF merit a higher index of suspicion.

  • highest incidence in age 5-15

  • painful joints and carditis

  • persists and resolves over weeks - months (90% of episodes are clinically contained within 3 months)

  • about half of individuals are left with chronic rheumatic heart disease.

 

Diagnosis

There is no diagnostic laboratory test for rheumatic fever

Diagnosis is clinical using the Jones criteria

2 major OR 1 major and 2 minor criteria AND laboratory evidence of streptococcal throat infection (usually throat swab)

  • Major

    • Arthritis (asymmetrical migratory polyarthritis affecting large joints)

    • Carditis ((in 80% of cases, affects mitral and aortic valves causing regurgitation)

      • Palpitations in 95%

      • Fatigue in 90%

      • Dyspnea in 75%

      • Chest pain in 75%

    • Erythema marginatum (torso, arms, legs)

    • Subcutaneous nodules (extensor surfaces of elbow, wrist, knee, ankles, Achilles)

    • Chorea (Sydenham's chorea) - rare

  • Minor
    • Arthralgia

    • Fever (single measurement of >38 °C)

    • ESR ≥30 mm/h and/or CRP ≥3.0 mg/dL

    • First degree heart block

    • Prolonged PR interval, after accounting for age variability (unless carditis is a major criterion)

 
 
 
 

Erythema marginatum

(clear centre)

typically in Rheumatic Fever

Erythema migrans

(bull's eye centre)

typically in Lyme Disease

Subcutaneous nodules

in rheumatic fever

Treatment

  • Refer to secondary care

    • Supportive treatment during active inflammation

    • 10 days of penicillin V

 

Recurrence and prophylaxis

  • The risk of rheumatic fever recurrence is greatest during the first 3-5 years following the attack.

  • Secondary antibiotic prophylaxis

    • IM benzyl penicillin every 3-4 weeks

    • Continue for 5-10 years after the first attack

    • Continue indefinitely in patients with established heart disease

    • Continue indefinitely in patients who are frequently exposed to streptococci and are difficult to monitor

 
 

Video : Sydenham's chorea

 
 

Rheumatic heart disease

Marijon, E; Mirabel, M; Celermajer, DS; Jouven, X

Lancet. 379 (9819): 953–64

10 March 2012

Access

Rheumatic Fever

Parrillo, Steven J.  

eMedicine.

Access

Rheumatic fever & rheumatic heart disease: The last 50 years

Kumar, RK; Tandon, R

The Indian Journal of Medical Research. 137 (4): 643–658.

2013

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Revised Jones Criteria for the Diagnosis of Acute Rheumatic Fever

Michael H et al

American Heart Association

2015

Access

Guidelines for rheumatic fever: diagnosis, management and secondary prevention of acute rheumatic fever and rheumatic heart disease

Kumar, RK; Tandon, R

Heart Foundation of New Zealand and Cardiac Society of Australia and New Zealand

2014

Access

 

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