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
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arthritis
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carditis
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chorea
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erythema marginatum
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subcutaneous nodules
Epidemiology
Most major outbreaks occur under conditions of impoverished overcrowding where access to antibiotics is limited
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more prevalent in Middle East, the Indian subcontinent, and some areas of Africa and South America
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as many as 20 million new cases occur each year.
Presentation
Inflammatory response to Group A streptococcus infection
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manifests 2-3 weeks after throat infection
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Approximately 70% of older children and young adults recollect the pharyngitis.
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However, only approximately 20% of young children recollect pharyngitis.
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Therefore, younger children who present with signs or symptoms consistent with ARF merit a higher index of suspicion.
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highest incidence in age 5-15
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painful joints and carditis
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persists and resolves over weeks - months (90% of episodes are clinically contained within 3 months)
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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)
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Major
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Arthritis (asymmetrical migratory polyarthritis affecting large joints)
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Carditis ((in 80% of cases, affects mitral and aortic valves causing regurgitation)
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Palpitations in 95%
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Fatigue in 90%
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Dyspnea in 75%
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Chest pain in 75%
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Erythema marginatum (torso, arms, legs)
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Subcutaneous nodules (extensor surfaces of elbow, wrist, knee, ankles, Achilles)
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Chorea (Sydenham's chorea) - rare
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- Minor
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Arthralgia
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Fever (single measurement of >38 °C)
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ESR ≥30 mm/h and/or CRP ≥3.0 mg/dL
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First degree heart block
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Prolonged PR interval, after accounting for age variability (unless carditis is a major criterion)
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Erythema marginatum
(clear centre)
typically in Rheumatic Fever

Erythema migrans
(bull's eye centre)
typically in Lyme Disease

Subcutaneous nodules
in rheumatic fever
Treatment
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Refer to secondary care
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Supportive treatment during active inflammation
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10 days of penicillin V
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Recurrence and prophylaxis
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The risk of rheumatic fever recurrence is greatest during the first 3-5 years following the attack.
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Secondary antibiotic prophylaxis
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IM benzyl penicillin every 3-4 weeks
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Continue for 5-10 years after the first attack
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Continue indefinitely in patients with established heart disease
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Continue indefinitely in patients who are frequently exposed to streptococci and are difficult to monitor
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Video : Sydenham's chorea

Rheumatic heart disease
Marijon, E; Mirabel, M; Celermajer, DS; Jouven, X
Lancet. 379 (9819): 953–64
10 March 2012
Rheumatic Fever
Parrillo, Steven J.
eMedicine.
Rheumatic fever & rheumatic heart disease: The last 50 years
Kumar, RK; Tandon, R
The Indian Journal of Medical Research. 137 (4): 643–658.
2013
Revised Jones Criteria for the Diagnosis of Acute Rheumatic Fever
Michael H et al
American Heart Association
2015
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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