Cardiovascular Medicine Book

http://www.fpnotebook.com/

Kawasaki's DiseaseAka: Mucocutaneous Lymph Node Syndrome, Infantile Polyarteritis

Advertisement

  1. Epidemiology: Incidence
    1. Japanese descent: 150 per 100,000 children <5 years
    2. Overall U.S.: 10-15 per 100,000 children <5 years
  2. Etiology
    1. Retrovirus
    2. Rickettsia
  3. Differential Diagnosis
    1. Toxic shock Syndrome
    2. Scarlet Fever
    3. Serum Sickness
    4. Steven's Johnson
    5. Parvovirus B19
  4. Diagnosis: Requires fever and 4 other criteria
    1. Fever prolonged more than 5 days (100%)
      1. Fever >102.2 F (often >104 F)
      2. Duration: Averages 11 days untreated
    2. Polymorphous skin eruption on trunk (80%)
      1. Starts within first five days
      2. Groin rash accentuated
      3. No bullae or vessicles
    3. Conjunctivitis (84%)
      1. Bilateral Conjunctival injection (bulbar)
      2. No Ciliary Flush (no Limbus injection)
      3. No photophobia
      4. No Eye Pain
      5. Non-purulent
    4. Acute Cervical Lymphadenopathy >1.5 cm (73%)
    5. Changes in hands and feet
      1. Edema of hands and feet - may be painful (65%)
      2. Red palms and soles (69%)
        1. Sharp demarcation at ankles and wrists
      3. Desquamation of fingertips (80%)
        1. Late sign occurs at 1 to 2 weeks
        2. Starts at periungual area
    6. Mucus mebrane changes (92%)
      1. Dry, red, fissured, or vertically cracked lips
      2. Strawberry Tongue
      3. Diffuse reddening of the oropharynx
      4. No focal lesions, exudates or ulcers
  5. Other Associated signs and symptoms
    1. Urethritis with sterile pyuria (75%)
    2. Polyarthralgia
    3. Abdominal Pain and Diarrhea (25%)
    4. Aseptic Meningitis (25%)
    5. Cardiac Disease (20%)
      1. Pericardial Effusion
      2. Congestive Heart Failure
      3. Arrhythmias
    6. Obstructive Jaundice with acute gallbladder hydrops
  6. Differential Diagnosis
    1. Hypersensitivity Reaction (medication, Mercury)
    2. Juvenile Rheumatoid Arthritis
    3. Leptospirosis
    4. Rocky Mountain Spotted Fever
    5. Scarlet Fever
    6. Staphylococcal scalded skin syndrome
    7. Toxic shock syndrome
    8. Viral infection (Mononucleosis, Measles, Adenovirus)
  7. Labs
    1. Complete Blood Count
      1. Differential cell count
      2. Platelet Count
    2. Erythrocyte Sedimentation Rate >40 mm/hr (may be >100)
    3. C-Reactive Protein (CRP) >3 mg/dl
      1. Preferred (unlike ESR, not altered like IVIG)
    4. Blood Culture
    5. Throat Culture
    6. Antistreptolysin-O Titer (ASO Titer)
    7. Urinalysis
    8. Consider Lumbar Puncture if Meningitis suspected
  8. Radiology: Echocardiogram coronary artery evaluation
    1. Initial echocardiogram at presentation
    2. Follow-up echocardiogram at 2 weeks and 6-8 weeks
    3. Indications for echocardiogram at 6-12 months
      1. Abnormalities on prior echocardiograms
      2. AHA guidelines currently recommend in all patients
      3. However no benefit if prior echocardiograms normal
      4. Tuohy (2001) Am J Cardiol 88:328
    4. Findings
      1. Coronary artery changes
        1. Acute: Tapering, perivascular brightness, ectasia
        2. Late: Coronary artery aneurysm
      2. Other variable changes
        1. Decreased ventricular function
        2. Pericardial Effusion
  9. Management
    1. Admission
    2. Hydration
    3. Obtain Rheumatology and Cardiology consultation
    4. Observe for and treat Congestive Heart Failure
    5. Start treatment as soon as possible
      1. Especially within 10 days of onset
    6. Aspirin
      1. Initial: 60-110 mg/kg/day divided every 6 hours
      2. Later: 5-10 mg/kg/day for 6-8 weeks
        1. Start after fever has resolved at least 2 days or
        2. 14 days passes since onset of illness
    7. Intravenous Gamma Globulin (IVIG)
      1. Dose: 2 g/kg over 8-12 hours
      2. Must be given early in disease to be efficacious
    8. Other agents to consider
      1. Abciximab (Remicade)
  10. Complications: Coronary artery aneurysm
    1. Incidence
      1. Occurs in 20% of untreated Kawasaki's Disease
      2. Occurs in 2% of treated Kawasaki's Disease
    2. Mechanism: Coronary artery Vasculitis
    3. Occurs 1-4 weeks after onset of illness
    4. Most aneurysms regress in 1-2 year
      1. Spontaneously resolve in 33-66% of cases
    5. Associated Risks
      1. Subsequent stenosis
      2. Thrombosis leading to Myocardial Infarction
      3. Sudden Death
    6. Risk Stratification
      1. Risk Level 1: Normal coronary arteries on imaging
        1. May stop Aspirin at 8 weeks following onset
        2. Life-long counseling on Cardiac Risk Factors
          1. Long-term endothelial dysfunction risk
      2. Risk Level 2: Transient coronary ectasia, dilation
        1. Same approach as Risk Level 1
      3. Risk Level 3: 3-6 mm coronary aneurysms (Z-Score 3-7)
        1. Aspirin for >8 weeks and until aneurysm regresses
        2. Stress Test every 2 years before sports if age >10
        3. Annual echocardiogram and Electrocardiogram
      4. Risk Level 4: >6 mm coronary aneurysms or multiple
        1. Long-term Aspirin
        2. Warfarin for giant aneurysms
        3. Annual cardiac stress test
        4. Echocardiogram and Electrocardiogram every 6 months
        5. Cardiac angiogram 6-12 months after illness
        6. Avoid collision sports
      5. Risk Level 5: Obstructed coronary artery aneurysms
        1. Includes recommendations for risk level 4
        2. Beta Blocker
  11. References
    1. Dummer (2004) Progress Pediatr Cardiol 19:129
    2. Freeman (2006) Am Fam Physician 74:1141
    3. Newburger (2004) Pediatrics 114:1708

Mucocutaneous Lymph Node Syndrome (C0026691)

Definition (MSH)An acute, febrile, mucocutaneous condition accompanied by swelling of cervical lymph nodes in infants and young children. The principal symptoms are fever, congestion of the ocular conjunctivae, reddening of the lips and oral cavity, protuberance of tongue papillae, and edema or erythema of the extremities.
Definition (CSP)systemic disease primarily of infants and young children, characterized by skin rash, swelling of hands and feet, enlarged cervical lymph nodes, "strawberry tongue", dry and cracked lips, high fevers, and coronary artery disease.
ConceptsDisease or Syndrome (T047)
ICD9446.1
EnglishAcute febrile MCLS, Acute febrile mucocutaneous lymph node syndrome, INFANTILE POLYARTERITIS, INFANTILE POLYARTERITIS NODOSA, KAWASAKI DIS, Kawasaki Disease, Kawasaki syndrome, Kawasaki's disease, Kawasaki's syndrome, KD, MCLS, MLNS, MUCOCUTAN LYMPH NODE SYN, Mucocutaneous Lymph Node Syndrome
Spanishenfermedad de Kawasaki, síndrome ganglionar mucocutáneo, síndrome ganglionar mucocutáneo febril agudo, sindrome ganglionar mucocutaneo, sindrome ganglionar mucocutaneo febril agudo
CreditsDerived from the NIH UMLS (Unified Medical Language System)



Navigation Tree