I. Epidemiology: Incidence

  1. Japanese descent: 150 per 100,000 children <5 years
  2. Overall U.S.: 10-15 per 100,000 children <5 years
  3. Age of onset
    1. Most cases occur by age 5 years old (but can occur in older children)
    2. Peak age of onset: 1 to 2 years old
    3. Rare under 4 months of age
  4. Gender: Boys more often affected
  5. More common in winter and spring

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II. Pathophysiology

  1. Idiopathic Vasculitis
  2. Postulated associations
    1. Retrovirus
    2. Rickettsia

III. Symptoms

  1. Fever for 5 days or more
  2. Irritability

IV. Diagnosis: Requires fever and 4 other criteria

  1. Fever prolonged more than 5 days (100%)
    1. Fever >102.2 F (often >104 F) with abrupt onset
    2. Duration: Averages 11 days untreated
  2. Polymorphous skin eruption on trunk (80%)
    1. Starts within first five days with a 7 day duration
    2. Groin rash accentuated
    3. May appear morbiliform or Urticarial
    4. 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
    6. May be associated with painless anterior Uveitis
  4. Acute Lymphadenopathy
    1. Typically a single, unilateral, minimally tender node >1.5 cm (73%)
    2. Cervical Lymphadenopathy is most common
  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, Vesicles or ulcers

V. Signs: Other Associated

  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

VI. Differential Diagnosis

VII. Labs

  1. Complete Blood Count with differential cell count
    1. Leukocytosis
    2. Thrombocytosis
  2. Erythrocyte Sedimentation Rate >40 mm/hr (may be >100)
  3. C-Reactive Protein (CRP) >3 mg/dl
    1. Preferred (unlike ESR, CRP is not altered with IVIG administration)
  4. Liver Function Tests
  5. Blood Culture
  6. Throat Culture
  7. Antistreptolysin-O Titer (ASO Titer)
  8. Urinalysis
    1. Sterile pyuria
  9. Consider Lumbar Puncture if Meningitis suspected

VIII. Diagnostics

  1. Electrocardiogram (EKG)
    1. May demonstrate strain pattern (ST and T Wave changes)
  2. Chest XRay
    1. Observe for cardiomegaly

IX. Imaging: 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 88:328-30
  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

X. Evaluation

  1. Diagnostic criteria met (five days of fever and 4 of the 5 other findings present)
    1. Treat as Kawasaki Disease
    2. May also treat at 4 days if criteria otherwise met
  2. Diagnostic criteria not met (five days of fever with only 2-3 other findings)
    1. CRP <3 mg/dl and ESR <40 mm/h
      1. Discharge home with follow-up
      2. Repeat labs and other testing if fever persists
    2. CRP >3 mg/dl or ESR >40 mm/h
      1. Obtain additional criteria (requires 3 or more)
        1. Serum Albumin <3 g/dl
        2. Anemia
        3. Serum ALT increased
        4. White Blood Cell count >12,000
        5. Platelet Count >450,000
        6. Sterile pyuria
      2. Three or more additional criteria present
        1. Treat as Kawasaki Disease
      3. Less than three additional criteria
        1. Obtain Echocardiogram

XI. Management

  1. Admission
  2. Hydration
  3. Obtain Rheumatology and Cardiology Consultation
  4. Observe for and treat Congestive Heart Failure
  5. Cardiac monitoring in all patients
  6. Start treatment as soon as possible
    1. Especially within 10 days of onset
  7. 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
  8. Intravenous Gamma Globulin (IVIG)
    1. Dose: 2 mg/kg over 8-12 hours
    2. Must be given early in disease to be efficacious
    3. Risk of Hypotension and Seizures
  9. Other agents to consider
    1. Abciximab (Remicade)

XII. Complications: General

  1. Late complications (>1-2 weeks)
    1. Coronary Artery aneurysm (see below)
  2. Early complications
    1. Myocarditis
    2. Pericarditis
    3. Valvular insufficiency
    4. Arrhythmia

XIII. 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 ArteryVasculitis
  3. Occurs 2-4 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

Images: Related links to external sites (from Google)

Ontology: Mucocutaneous Lymph Node Syndrome (C0026691)

Definition (NCI) A vasculitis characterized by inflammation of the arteries, particularly the coronary arteries. The vasculitis may lead to aneurysm formation and possibly, heart attacks. It affects young children who usually present with persistent high fever, redness of the mucous membranes of the mouth, redness of the palms and soles, skin rashes, lymphadenitis, and joint pain and swelling.
Definition (MEDLINEPLUS)

Kawasaki disease is a rare childhood disease. It makes the walls of the blood vessels throughout the body become inflamed. It can affect any type of blood vessel, including the arteries, veins and capillaries.No one knows what causes Kawasaki disease. Symptoms include

  • High fever
  • Swollen lymph nodes in the neck
  • A rash on the mid-section and genital area
  • Red, dry, cracked lips and a red, swollen tongue
  • Red, swollen palms of the hands and soles of the feet
  • Redness of the eyes

Kawasaki disease can't be passed from one child to another. It is mainly treated with medicines. Rarely, medical procedures and surgery also may be used for children whose coronary arteries are affected.

Kawasaki disease can't be prevented. However, most children who develop the disease fully recover -- usually within weeks of getting signs and symptoms. Further problems are rare.

NIH: National Heart, Lung, and Blood Institute

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.
Concepts Disease or Syndrome (T047)
MSH D009080
ICD9 446.1
ICD10 M30.3
SnomedCT 155444003, 195348009, 195349001, 75053002
English Kawasaki Disease, Lymph Node Syndrome, Mucocutaneous, Acute febrile mucocutaneous lymph node syndrome (MCLS), INFANTILE POLYARTERITIS NODOSA, Act febr muccut lymph node syn, Acute febrile MCLS NOS, Acute febrile mucocutaneous lymph node syndrome NOS, Mucocutaneous lymph node syndr, Mucocutaneous lymph node syndrome [Kawasaki], mucocutaneous lymph node syndrome, Mucocutaneous Lymph Node Syndrome, KAWASAKI DIS, Kawasaki disease (diagnosis), Kawasaki's disease (disorder), KAWASAKI DISEASE, KD, MUCOCUTANEOUS LYMPH NODE SYNDROME, Mucocutan lymph node syn, Kawasaki Syndrome, Acute febrile mucocutaneous lymph node syndrome NOS (disorder), Mucocutaneous Lymph Node Syndrome [Disease/Finding], kawasaki's syndrome, kawasakis syndrome, kawasaki's disease, kawasaki disease, kawasaki syndrome, Acute febrile mucocutaneous lymph node syndrome, Kawasaki's disease, Kawasaki syndrome, Kawasaki disease, MCLS, MLNS, Mucocutaneous lymph node syndrome, Acute febrile mucocutaneous lymph node syndrome (disorder), Kawasaki's syndrome, Kawasaki, mucocutaneous lymph node; syndrome, syndrome; mucocutaneous lymph node, Acute febrile mucocutaneous lymph node syndrome [MCLS], Acute febrile MCLS, Disease;Kawasaki
Dutch mucocutaan lymfeknoopsyndroom, acuut febriel mucocutaan lymfeknoopsyndroom (MCLS), mucocutane lymfeklier; syndroom, syndroom; mucocutane lymfeklier, Mucocutaan lymfekliersyndroom [Kawasaki], ziekte van Kawasaki, Kawasaki-ziekte, Lymfkliersyndroom, mucocutaan, Mucocutaan lymkliersyndroom, Syndroom, mucocutaan lymfklier-
French Syndrome adéno-cutanéo-muqueux de Kawasaki aigu fébrile, Syndrome de ganglion lymphatique mucocutané, Maladie de Kawasaki, Périartérite noueuse infantile du Japon, Syndrome adéno-cutanéo-muqueux, Syndrome adénocutanéomuqueux
German akutes febriles mukokutanes Lymphknotensyndrom (MLCS), mukokutanes Lymphknotensyndrom, Mukokutanes Lymphknotensyndrom [Kawasaki-Krankheit], Kawasaki Syndrom, Kawasaki-Krankheit, Lymphknotensyndrom, mukokutanes, MCLS, Mukokutanes Lymphknotensyndrom
Italian Sindrome muco-cutanea linfonododale, Sindrome mucocutanea linfonodale febbrile acuta [MCLS], MCLS, Malattia di Kawasaki, Sindrome di Kawasaki, Sindrome mucocutanea linfonodale
Portuguese Síndrome de gânglios linfáticos mucocutâneos, Síndrome febril aguda dos gânglios linfáticos mucocutâneos, Síndrome do Linfonodo Mucocutâneo, Síndrome do Linfonodo Mucocutâneo (MLNS), Síndrome do Linfonodo Mucocutâneo (MCLS), Síndrome de Linfonodos Mucocutâneos, Doença de Kawasaki
Spanish Síndrome mucocutáneo con adenopatías, Síndrome del ganglio linfático mucocutáneo febril agudo (SLMC), Kawasaki's disease, Síndrome Mucocutáneo Linfonodular (MLNS), Síndrome Mucocutáneo Linfonodular (SMCL), Sindrome Mucocutaneo Linfonodular (MCLS), Sindrome Mucocutaneo Linfonodular (MLNS), Sindrome Mucocutaneo Linfonodular (SMCL), Síndrome Mucocutáneo Linfonodular (MCLS), enfermedad de Kawasaki (trastorno), enfermedad de Kawasaki, síndrome ganglionar mucocutáneo febril agudo, SAI (trastorno), síndrome ganglionar mucocutáneo febril agudo, SAI, síndrome ganglionar mucocutáneo febril agudo, síndrome ganglionar mucocutáneo, Enfermedad de Kawasaki, Síndrome del Nódulo Linfático Mucocutáneo, Síndrome Mucocutáneo Linfonodular, Sindrome Mucocutaneo Linfonodular, Sindrome del Nodulo Linfatico Mucocutaneo
Japanese 急性熱性粘膜皮膚リンパ節症候群, ヒフネンマクリンパセツショウコウグン, キュウセイネッセイネンマクヒフリンパセツショウコウグン, カワサキビョウ, キュウセイネツセイネンマクヒフリンパセツショウコウグン, 川崎病, 急性熱性皮膚粘膜リンパ節症候群, 皮膚粘膜リンパ節症候群, リンパ節症候群-皮膚粘膜
Swedish Mukokutant lymfkörtelsyndrom
Czech Kawasakiho nemoc, Kawasakiho syndrom, mukokutánní lymfatický syndrom, Akutní horečnatý mukokutánní uzlinový syndrom, Mukokutánní uzlinový syndrom
Finnish Kawasakin tauti
Russian LIMFOUZLOVOI SINDROM SLIZISTO-KOZHNYI, SLIZISTO-KOZHNYI LIMFOUZLOVOI SINDROM, KAVASAKI BOLEZN', КАВАСАКИ БОЛЕЗНЬ, ЛИМФОУЗЛОВОЙ СИНДРОМ СЛИЗИСТО-КОЖНЫЙ, СЛИЗИСТО-КОЖНЫЙ ЛИМФОУЗЛОВОЙ СИНДРОМ
Korean 점막피부성 림프절 증후군[가와사키]
Polish Zespół Kawasaki, Choroba Kawasaki, Skórno-śluzówkowy zespół węzłów chłonnych
Hungarian Kawasaki-betegség, Acut lázas mucocutan nyirokcsomó syndroma (MCLS), Mucocutan nyirokcsomó syndroma

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