Rheumatic
fever
Eslam Elsayed
CARDIO LECTURER
Introduction
• Acute rheumatic fever (ARF) is a systemic illness that
occurs 2-4 weeks after pharyngitis in some people,
due to cross-reactivity to group A β-
hemolytic streptococcus (GAS), also
called Streptococcus pyogenes.
Epidemiology
• 4 million children affected worldwide
• 94% of cases are in developing countries
• Most common in tropical countries with no seasonal
variation
• More common in females
Pathophysiology
• Streptococcus pyogenes is a gram-positive cocci and
it produces two cytolytic toxins: streptolysin O and S.
• Rheumatogenic strains of GAS contain M proteins in
their cell wall and are immunogenic. B cells are
stimulated to produce anti-M
protein antibodies against the infection which also
cross react with other tissues e.g. that of
the heart (causing rheumatic heart
disease), brain, joints and skin leading to a
constellation of multiorgan signs and symptoms. This is
also exacerbated by production of activated cross
reactive T cells.
Risk Factors
• Children and young people
• Poverty
• Overcrowded and poor hygiene places
• Family history of rheumatic fever
• D8/17 B cell antigen positivity
• The Revised Jones Diagnostic Criteria (below)
describes the key clinical features that may be present.
In addition to this, you may elicit from the history that
they had a recent sore throat or scarlet fever. Also,
in severe acute rheumatic failure, a
heart murmur might be heard on examination and it is
most commonly the mitral valve, which is affected.
Diagnostic Requirements
• Positive throat culture for Group A β-haemolytic
streptococcus or elevated anti-streptolysin O (ASO)
or anti-deoxyribonuclease B (anti-DNASE B) titre.
• AND
• 2 major criteria OR 1 major and 2 minor criteria present
for initial ARF. (Same criteria for recurrent ARF plus can
also be just 3 minor criteria)
Major Criteria (SPECS)
• Sydenham’s chorea
• Polyarthritis
• Erythema marginatum
• Carditis
• Subcutaneous nodules
Minor Criteria (CAPE)
• CRP or ESR – Raised acute phase reactant
• Arthralgia
• Pyrexia/Fever
• ECG – Prolonged PR interval
• *Joint (arthritis or arthralgia) and cardiac (carditis or
prolonged PR interval) manifestations can only be
counted once, not twice, as either a major or a minor
criterion.
• *Slight variation of criteria for high risk population
Differential diagnosis
•·Usually only one joint involved
Septic Arthritis •·Positive gram strain, culture and elevated
WBC of aspirated synovial fluid
Reactive arthropathy •Commonly males and often associated
with urethritis and conjunctivitis
•Positive blood culture
•Echocardiogram shows vegetations on the
Infective endocarditis valves
•Signs: Janeway lesions, Osler nodes,
splinter haemorrhages
•Troponin and creatinine kinase elevated
Myocarditis •ECG: saddle ST segments or T wave
changes
Investigations
• Bloods: ESR, CRP, FBC (WBC),
• Blood cultures to exclude sepsis
• Rapid Antigen Detection Test
• Throat culture: may be negative by the time rheumatic fever
symptoms occur
• Anti-streptococcal serology: ASO and anti-DNASE B titres
• ECG: prolonged PR interval
• CXR if carditis is suspected: congestive heart failure may be
seen in ARF due to valvular damage
• Echocardiography
Management
• Initial management in confirmed rheumatic fever
1.Antibiotics e.g. benzathine benzylpenicillin (1st choice due to
its long acting property, serving the purpose of GAS eradication
and secondary prophylaxis), phenoxymethylpenicillin , amoxicillin.
In confirmed penicillin allergy, alternatives include cephalosporins
(avoid in IgE mediated penicillin allergy and anaphylaxis),
macrolides and clindamycin (4)
2.Aspirin or NSAIDs e.g. naproxen or ibuprofen
3.Assess for emergency valve replacement
4.In severe carditis (e.g. congestive cardiac failure or 3rd degree
heart block) glucocorticoids and diuretics may be required
• Definitive and Long‐term management
• Secondary prophylaxis with intramuscular Benzathine
benzylpenicillin every 3-4 weeks, oral
Phenoxymethylpenicillin twice daily, oral sulfadiazine
daily, or oral azithromycin (in penicillin allergy) (4)
• Complications and Prognosis
• 2% of the population can get permanent damage to
heart valves and chronic rheumatic heart disease
• With treatment ARF should resolve within 2
weeks but cardiac inflammation may take months to
resolve fully and thus, it is common for patients to
relapse within this time
References
References
[
1 [Online]. Available: http://bestpractice.bmj.com
]
[
2 [Online]. Available: emedicine.medscape.com
]
American Heart Association Committee on Rheumatic Fever,
[ Endocarditis, and Kawasaki Disease of the Council on
3 Cardiovascular
for the
Disease in the Young. Revision of the Jones Criteria
diagnosis of acute rheumatic fever in the era of Doppler
] echocardiography: a scientific statement from the American Heart
Association. Circulation. 2015 May 19;131(20):1806-18.
Prevention of rheumatic fever and diagnosis and treatment of
acuteStreptococcal pharyngitis: a scientific statement from the
American Heart Association Rheumatic Fever, Endocarditis, and
[ Kawasaki Disease Committee of the Council on Cardiovascular
4 Disease in the Young, the Interdisciplinary Council on Functional
] Genomics and Translational Biology, and the Interdisciplinary
Council on Quality of Care and Outcomes Research: endorsed by
the American Academy of Pediatrics. Circulation. 2009 Mar
24;119(11):1541-51.
