Rheumatic fever is a multisystem inflammatory disease that occurs in the genetically susceptible host 2 to 4 wk after a pharyngeal infection with group A streptococci.
PHYSICAL FINDINGS & CLINICAL PRESENTATION
- Acute streptococcal pharyngitis, which may be subclinical and not reported by the patient
- After latent period of 2 to 4 wk (average, 19 days), acute rheumatic attack
- Patient is febrile, with a migratory polyarthritis of knees, ankles, wrists, elbows; typically severe for 1 wk, remits by 3 to 4 wk
- New heart murmur
- Mitral regurgitation
- Aortic insufficiency
- Diastolic mitral murmur
- Pericardial friction rub or effusion
- Rarely, pancarditis is severe and fatal
- Subcutaneous nodules can be palpated over extensor tendon surfaces or bony prominences, such as the skull
- Chorea (Sydenham’s chorea) is characterized by rapid involuntary movements affecting all muscles:
- Muscular weakness
- Emotional lability
- Rarely seen after adolescence and almost never in adult males
- Erythema marginatum:
- Evanescent, pink, well-demarcated spreading to trunk and proximal extremities
- Not specific
- Arthralgias (joint pain without swelling)
- Abdominal pain
- Group A streptococci not recovered from tissue lesions.
- It does not occur in the absence of a streptococcal antibody response.
- Immunologic cross-reactivity between certain streptococcal antigens and human tissue antigens suggests an autoimmune cause.
- Both initial attacks and recurrences can be completely prevented by prompt treatment of streptococcal pharyngitis with penicillin.
- Rheumatoid arthritis
- Juvenile rheumatoid arthritis (Still’s disease)
- Bacterial endocarditis
- Systemic lupus
- Viral infections
- Poststreptococcal reactive arthritis: severe arthritis but no carditis
- “Jones Criteria (revised) for Guidance in the Diagnosis of Rheumatic Fever”
- One major and two minor criteria if supported by evidence of an antecedent group A streptococcal infection
- Major criteria:
- Carditis and valvulitis
- Migratory arthritis (predominantly involving large joints)
- Central nervous system involvement (e.g., chorea)
- Erythema marginatum
- Subcutaneous nodules
- Minor criteria:
- Increased acute-phase reactants
- C-reactive protein
- Prolonged P-R interval
- Throat cultures are usually negative at presentation of clinical manifestations of rheumatic fever.
- Streptococcal antibody tests are more useful in establishing the diagnosis.
- Peak at the beginning of the attack
- Can document a recent streptococcal infection
- ASO (antistreptolysin O) titers peak:
- 4 to 5 wk after the actual streptococcal throat infection
- Which coincides during the second or third wk of illness of rheumatic fever
- Anti-DNase B (Streptozyme) can be detected for 6 to 9 mo following infection and can be tested for if ASO titer negative.
- Other antistreptococcal antibody tests include: streptokinase and antihyaluronidase.
- High-titer streptococcal antibodies:
- Are supportive of diagnosis, but not proof
- Should be interpreted in the context of clinical criteria
- Chest x-ray to assess heart size
- To evaluate murmurs
- To rule out pericardial effusion
ACUTE GENERAL Rx
- Course of penicillin to eradicate throat carriage of group A streptococci
- Arthralgia or arthritis without carditis: aspirin 90 to 100 mg/kg/day for 2 wk, followed by 60 to 70 mg/kg/day for the subsequent 4 to 6 wk if needed
- Carditis and heart failure:
- Prednisone 40 to 60 mg/day
- IV corticosteroids, such as methylprednisolone, 10 to 40 mg/day for severe carditis prophylaxis