Clinical Notes : Infection and Sepsis

70. Acute Rheumatic Fever

Definition

Epidemiology

Presentation

Diagnosis

Treatment

Recurrence and Prophylaxis

- References -

- CPD Quiz -

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

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Rheumatic Fever

Parrillo, Steven J.  

eMedicine.

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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

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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

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CPD Quiz and Certificate

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