Diagnosis
Rheumatic fever
An abnormal auto-immune response to group A Streptococcus infections (usually pharyngitis) that can result in arthritis, carditis and erythema marginatum, subcutaneous nodules, and chorea.
Etiology
Cause [qis]
Arises 2-3 weeks after streptococcal infections with inadequate antibiotic therapy:
Pharyngitis
Impetigo
Toxic shock syndrome
Necrotizing fasciitis
Affects 0.5-3% of the patients with streptococcal pharyngitis [lqf]
Pathophysiology [n7f][yow]
Immunologic response triggered by molecular mimicry between Group A Streptococcus antigens and self-antigens in various target organs in the body
Clinical manifestations:
Cardiac: Carditis, valvulitis (mitral/aortic valve regurgitation/stenosis)
Neurologic: Sydenham chorea
Musculoskeletal: Migratory polyarthritis
Dermatological: Subcutaneous nodules, erythema marginatum
Risk factors [yow]
Certain streptococcal M protein serotypes
Stronger host immune response (genetic tendencies)
Complications [yow]
Rheumatic heart disease (occurs 10 to 20 years after the original illness)
Valvular damage --> heart failure, pulmonary hypertension, dysrhythmias, embolic strokes, and sudden cardiac death
Jaccuod arthropathy
Sydenham chorea
Epidemiology
Incidence per 100.000 [9aa][ohh][9np][qis][7ta][yfn][lqf][ogz][n7f][td3][st9][dgk]
Symptoms & findings
Symptoms
Arthralgia, Arthritis, Chorea, Cough, Dyspnea, Edema, Fever, Heart murmur, Orthopnea, Polyarthritis, Systolic murmur
Clinical findings
Cardiomegaly, Elevated CRP, Elevated Sedimentation Rate, Pericardial effusion, Peritonitis, Prolonged PR interval
Anamneses
None listed.
Localized findings
Approach
Blood test: streptococcal antibody titer, ESR and CRP
Blood gas
Blood culture
Throat culture (the gold standard): rapid streptococcal antigen test, Nucleic acid amplification test (PCR) [yow]
Serelogy: Antistreptolysin O titer, antideoxyribonuclease B antibody titer
ECG
Echocardiography: carditis, mitral valve stenosis, aortic regurgitation
Chest x-ray
Artrocentesis: exclude septic arthritis
Biopsy: subcutaneous nodules, synovial biopsy, endomyocardial biopsy
Jones Diagnostic criteria: 2 major, or 1 major and 2 minor [qis][lqf][yow]
Major criteria:
Carditis (valvulitis)
Arthritis (migratory asymmetrical polyarthritis in the large joints: knees, ankles, elbows, or shoulders)
Chorea
Subcutaneous nodules
Erythema marginatum
Minor criteria
Arthralgia
First degree heart block
Fever ≥ 38.5 °C
Elevated erythrocyte sedimentation rate ≥60 or CRP ≥3.0 mg/dL
Evidence of prior infection with group A streptococcus
Other clinical features: Abdominal pain, nephritis, anemia, pneumonia, and epistaxis
Treatment
The acute inflammatory phase: supportive and symptomatic relief [qis][v3c][lqf]
Arthritis: Aspirin, NSAIDs (naproxen 10-20 mg/kg/day)
Carditis: Bed rest if symptoms, aspirin, corticosteroids, diuretics, vasodilators, aortic/mitral valve surgery is delayed as much as possible if long-term valvular cardiac sequela is present
Chorea: quiet nonstimulatory environment, valproate and carbamazepine for reducing involuntary movements, intravenous immunoglobulin, steroids, and plasmapheresis
Treatment of group A streptococcus infection: [qis][yow]
Phenoxymethylpenicillin 250 mg x 2 (children) or 500 mg x 2 (adults) for 10 days
Or one dose of IM benzylpenicillin 600,000 IU (<27 kg) or 1.2 million IU (>27 kg)
If penicillin allergy --> Cephalosporin (Cefuroxime) or Macrolide (Azithromycin)
Prophylaxis: Intramuscular benzylpenicillin every 3-4 weeks for 10 years or until age 21 (lifelong if carditis with residual damage)
Differential diagnoses
Aortic regurgitation, Aortic stenosis, Atrial myxoma, Cardiomyopathy, Dermatomyositis, Drug side effects, Endocarditis, Erythema multiforme, Glomerulonephritis, Gonorrhea, Gout, Heart failure, Henoch-Schönlein purpura, Influenza, Juvenile rheumatoid arthritis, Kawasaki disease, Leukemia, Lyme disease, Lymphoma, Mitral stenosis, Mixed connective tissue disease, Myocarditis, Osteomyelitis, Pericarditis, Pseudogout, Reactive arthritis, Rheumatoid arthritis, Sarcoidosis, Scarlet fever, Sepsis, Septic arthritis, Serum sickness, Sickle cell disease, Systemic lupus erythematosus, Takayasu arteritis, Tuberculosis, Urticaria, Wilson's disease
References
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[2] http://emedicine.medscape.com/article/333103 (2014-01-29); [Medscape]
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[14] https://emedicine.medscape.com/article/236582