rheumatic%20fever%20-%20acute
RHEUMATIC FEVER - ACUTE
Acute rheumatic fever is an autoimmune response to a previous group A beta-hemolytic streptococcal (GAS) infection causing acute generalized anti-inflammatory response primarily affecting the heart.
It often presents in patients 5-14 years of age and uncommon before 3 years and after 21 years of age.
Patients presenting with acute rheumatic fever are severely unwell, in extreme pain and requires hospitalization.

Diagnosis

  • Diagnosis of acute rheumatic fever (ARF) is primarily based on clinical assessment

Classification

Definite acute rheumatic fever (ARF)
  • Initial episode of ARF w/ 2 major or 1 major & 2 minor manifestations w/ evidence of previous Group A streptococcus (GAS) infection
Probable acute rheumatic fever (ARF)
  • Initial episode of ARF w/ 1 major & 2 minor manifestations w/ evidence of previous beta-hemolytic streptococcal (GAS) infection as 1 of the minor manifestations
Possible acute rheumatic fever (ARF)
  • Strong clinical suspicion of ARF but w/ insufficient signs & symptoms

Evaluation

Diagnosis of initial attack of rheumatic fever (RF) based on revised Jones criteria
  • Generally, 2 major or 1 major & 2 minor criteria leads to the diagnosis of RF along w/ history of Group A streptococcus (GAS) infection
Major manifestations
  • Arthritis
    • Most common manifestation of acute rheumatic fever (ARF)
    • Usually affects >1 joint, large joints (eg ankles, wrists, knees, elbows, or shoulders)
    • Presents w/ swelling, heat, redness, severe pain, tenderness & limited movement
    • Highly responsive to nonsteroidal anti-inflammatory drugs (NSAIDs)
    • Presents as polyarthritis (asymmetrical & migratory) in low-risk populations
    • Presents as either polyarthritis or monoarthritis in moderate- & high-risk populations
      • Polyarthralgia may be considered only after other causes of arthralgia ( viral, autoimmune) has been ruled out
  • Carditis (inflammation of the heart)
    • Sometimes accompanied by weakness & shortness of breath or chest pain
    • May be clinical &/or subclinical
    • Tachycardia, valvulitis, heart murmur of mitral &/or aortic regurgitation
    • Pericarditis
      • Friction rub, pericardial effusion, chest pain & electrocardiogram (ECG) changes
    • Cardiomegaly on chest X-ray
    • Signs of chronic HF: Gallop rhythm, distal heart sounds, cardiomegaly
  • Sydenham’s chorea
    • Neuropsychiatric disorder primarily involving jerky or uncoordinated movement of the limbs & face or more subtle movement difficulties (eg difficulty w/ handwriting)
    • May also present w/ psychiatric signs (eg emotional lability, hyperactivity, separation anxiety, obsessions & compulsions)
    • Often affects prepubertal girls 8-12 years old
  • Subcutaneous nodules
    • Hard, painless, nonpruritic, freely movable, small bumps under the skin
    • Usually 0.5-2 cm in diameter & number from 1 to dozens w/ a mean of 3-4
    • Nodules appear over the extensor surfaces of the elbows, knees, ankles, knuckles, scalp & spinous processes of the lumbar & thoracic vertebrae
    • Tend to appear 2-4 weeks after onset of other symptoms & usually lasts 1-2 weeks
  • Erythema marginatum
    • Begins as 1-3 cm diameter, pink to red nonpruritic macules or papules that spread outward to form a serpiginous ring w/ erythematous raised margins & central clearing
    • Located on the trunk & proximal limbs but never on the face
Minor manifestations
  • Fever
    • Common manifestation of ARF
    • Usually >38.5OC (101.3OF) in low-risk populations, >38OC (100.4OF) in moderate- to high-risk populations & responds to antipyretics
  • Arthralgia
    • Pain in ≥1 joint w/o inflammation
      • Polyarthralgia in low-risk populations; monoarthralgia in moderate- to high-risk populations
    • May not be used as a criteria if arthritis is a major manifestation
  • Blood test indicating inflammation
    • Low-risk populations: elevated C-reactive protein (CRP) of ≥3.0 mg/dL &/or erythrocyte sedimentation rate (ESR) of ≥60 mm in the 1st hour
    • Moderate- to high-risk populations: CRP of ≥3.0 mg/dL &/or ESR of ≥30 mm/hour
  • Prolonged P-R interval on ECG
    • Should be done in all patients suspected to have ARF except if carditis is a major manifestation
    • Upper limit of normal should be adjusted according to age
Exceptions to the Jones criteria that are diagnostic of acute rheumatic fever (ARF)

Careful diagnosis is warranted, there are ARF patients w/ insufficient history, clinical or laboratory findings

  • Only has chorea as manifestation
  • Patients who consult after month of RF & only has indolent carditis as manifestation

History

Previous Group A streptococcus (GAS) infection
  • History of an antecedent sore throat 1-5 week prior to onset is present in most patients
  • Evidenced by streptococcal antibody tests
    • Plasma Antistreptolysin O (ASO) titer
      • 333 Todd units is considered elevated
    • Antideoxyribonuclease B (anti-DNAse B) titer
      • >240 Todd units is considered elevated
    • Positive throat culture for GAS

Laboratory Tests

  • Complete blood count (CBC)
  • Chest radiograph (cardiomegaly, pulmonary congestion & other findings consistent w/ heart failure)
  • Echocardiography w/ Doppler
    • Recommended for patients w/ suspected or definite acute rheumatic fever (ARF) including chorea
    • More sensitive & specific for diagnosing acute rheumatic conditions as compared to auscultation
    • Recommended as confirmatory tool for carditis in the absence of murmurs (subclinical carditis)
  • Electrocardiogram (ECG)
  • Rapid antigen detection test (RADT)
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