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.

Rheumatic%20fever%20-%20acute Diagnosis

Diagnosis

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

Classification

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

Evaluation

Diagnosis of Initial Attack of Rheumatic Fever (RF) Based on Revised Jones Criteria
  • Generally, 2 major or 1 major and 2 minor criteria leads to the diagnosis of RF along with 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 with swelling, heat, redness, severe pain, tenderness and limited movement
    • Highly responsive to nonsteroidal anti-inflammatory drugs (NSAIDs)
    • Presents as polyarthritis (asymmetrical and migratory) in low-risk populations
    • Presents as either polyarthritis or monoarthritis in moderate- and 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 and shortness of breath or chest pain
    • May be clinical and/or subclinical
    • Tachycardia, valvulitis, heart murmur of mitral and/or aortic regurgitation
    • Pericarditis
      • Friction rub, pericardial effusion, chest pain and 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 and face or more subtle movement difficulties (eg difficulty with handwriting)
    • May also present with psychiatric signs (eg emotional lability, hyperactivity, separation anxiety, obsessions and 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 and number from 1 to dozens with a mean of 3-4
    • Nodules appear over the extensor surfaces of the elbows, knees, ankles, knuckles, scalp and spinous processes of the lumbar and thoracic vertebrae
    • Tend to appear 2-4 weeks after onset of other symptoms and 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 with erythematous raised margins and central clearing
    • Located on the trunk and proximal limbs but never on the face
Minor Manifestations
  • Fever
    • Common manifestation of ARF
    • Usually >38.5°C (101.3°F) in low-risk populations, >38°C (100.4°F) in moderate- to high-risk populations and responds to antipyretics
  • Arthralgia
    • Pain in ≥1 joint without 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 and/or erythrocyte sedimentation rate (ESR) of ≥60 mm in the 1st hour
    • Moderate- to high-risk populations: CRP of ≥3.0 mg/dL and/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 ARF

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

  • Only has chorea as manifestation
  • Patients who consult after month of RF and 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 and other findings consistent with heart failure)
  • Echocardiography with Doppler
    • Recommended for patients with suspected or definite acute rheumatic fever (ARF) including chorea
    • More sensitive and 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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