Kawasaki syndrome M30.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

acute febrile mucocutaneous lymphadenopathy syndrome; Kawasaki disease; Kawasaki fever; Kawasaki Fever; Lymphadenopathy syndrome acute febrile mucocutaneous; Mucocutaneous lymph node syndrome

History
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Kawasaki 1967

Definition
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Probably immunologically mediated diffuse vasculitis, occurring predominantly in childhood, clinically characterized by high fever, enlarged cervical lymph nodes, skin and mucous membrane infestation and complicated in 15-25% of cases by the onset of myocarditis and coronariitis with consecutive thrombus and aneurysm formation in the coronary vessels.

Occurrence/Epidemiology
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Rare disease, highest incidence in Japan (4-6,000 diseases/year or 140/100,000/year). In Southeast Asia the incidence in children <5 years is 112/100,000 per year, in Europe 8.1/100,000 per year and in Germany approximately 8/100,000 per year.

Etiopathogenesis
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Unexplained, pathogen-induced triggering is probable (e.g. SARS-Cov-19) There is also a genetic predisposition. Change in an activity of a C-kinase that intervenes in the activation of T-lymphocytes (1,4,5-triphosphatase-3-kinase C). Several susceptibility genes are also known to play a role in the pathogenesis of the disease: ITPKC, CASP3, CD40 and ORAI(Onouchi Y 2018).


Manifestation
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Mostly occurring in infants aged 1-5 years, rarely in young adults. Slight emphasis on the male sex.

Clinical features
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Accommodation/General: High fever resistant to antibiotics, persisting for more than 5 days. Conjunctivitis (>75%). Painful, cervical lymph node swelling (25-75%), pharyngitis (90%)

Integument: Bright red, cracked lips, diffuse redness of the mucous membranes of the mouth and throat, possibly raspberry tongue (90%). Palmoplantar erythema (50-85%) with oedematous transformation (50-85%); after 2-3 weeks of desquamation, which begins crescent-shaped at the fingertips. Later Beau-Reilsche transverse furrows of the fingernails.

Polymorphic exanthema: morbilli- or scarlatiniform, erythema exsudativum multiforme-like(70-90%).

Other accompanying symptoms: gastroenteritis, urethritis, abacterial meningitis, arthralgia, gallbladder hydrops.

Complicative are cardiac symptoms which can occur 12-28 days after infection. They manifest themselves as coronaritis and can lead to (giant) aneurysms, thrombosis, arrhythmias, stenosis, myocardial infarction, heart failure, PAD.

Laboratory
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Leukocyturia and proteinuria, pronounced leukocytosis with left-shift, CRP and alpha-2-globulins increased Thrombocytosis from the 2nd week of illness.

Differential diagnosis
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Scarlet fever: clinical, pathogen detection, antistreptolysin titre (AST) increase (at the earliest 8-14 days after fresh infection, baseline and control values), Dick test, eradication phenomenon.

Measles: clinical picture with typical exanthema and enanthema (palate, tonsils, uvula). Titre increase by 2 levels in the haemagglutination inhibition test

Infectious mononucleosis: generalized lymphadenopathy, splenomegaly, morbilliform exxanthema, + serology

Brucellosis; M. Weil: rare

Acute rheumatic fever: carditis, polyarthritis (migratory), minor chorea (Sydenham), erythema marginatum rheumaticum (formerly also called eythema anulare rheumaticum), and subcutaneous nodules (rheumatic nodules). Secondary criteria: fever, arthralgias.

Hand-foot-mouth disease: fever, generally no severe feeling of illness, blisters painful

Breastfeeding syndrome: long-term course with recurrent fever attacks

Erythema exsudativum multiforme: typical clinical morphology of exanthema with cockades

Complication(s)
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In 20% of cases, vasculitis leads to aneurysm formation, mainly in the area of the coronary arteries. After months mostly regression.

Therapy
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See Table 1.

Progression/forecast
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Early diagnosis and therapy is important.

Untreated, the disease can heal completely within 12-28 days.

The frequent (dreaded) cardiac complications occur 12-28 days after the onset of the disease.

The lethality rate is about 1-2%, mainly due to heart attacks.

Decisive for the course and prognosis is the extent of vascular involvement.

Tables
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Therapy recommendations for Kawasaki syndrome (modified according to Cremer)

Medication

Example preparation

Indication

Dosage

Duration

Gamma globulin and acetylsalicylic acid

Intratect

Standard therapy

Gamma globulin preparation with intact Fc segment 2 g/kg bw as short infusion in 5% glucose solution

Single dose over 12 hours i.v.

In combination with:

ASS

Acetylsalicylic acid in a dose of at least 100 mg/kg bw (in smaller children often up to 150 mg/kg bw, in older children possibly less). Frequent ASA level determinations (therapeutic level between 1530 mg/dl or 1.12.2 mmol/l). From the third week of illness or one week after defibrillation, reduce ASA to 3 mg/kg bw.

Total duration 3 months. Longer therapy (at least 2 years) for patients with proven changes in the coronary arteries.

Gamma globulin and acetylsalicylic acid

Intratect

Standard therapy, if therapy can be started in the first week of illness, especially for children at risk (< 2 years).

Gamma globulin preparation with intact Fc segment, 400 mg/kg bw as short infusion in 5% glucose solution.

Over 5 days.

In combination with:

ASS

Acetylsalicylic acid 30-50 mg/kg bw until defibrillation, then 5 mg/kg bw

Prednisolone and acetylsalicylic acid

Decortin H Tbl.

Alternative therapy, if gamma globulin could not be started in the 1st phase of the disease or if defibrillation did not occur under this therapy.

Prednisolone 2 mg/kg bw/day (1st week) in 3-4 ED.

Over 3 weeks, then weaning out within 1 week. 4 weeks beyond discontinuation of prednisolone therapy. Do not stop therapy until echocardiography is unremarkable and platelets and BSG normalize.

In combination with:

ASS

Acetylsalicylic acid 30-50 mg/kg bw/day until defibrillation, then 5 mg/kg bw/day

Monotherapy with acetylsalicylic acid

ASS

Only for very slight gradients!

60-100 (-130) mg/kg bw/day in 4 ED. Target acetylsalicylic acid level 20-25 mg/dl. In case of defever, dose reduction to 50 mg/kg bw/day in 2 weeks. From 3rd week (thrombocytosis!) 3-5 mg/kg bw/day once/day for thrombocyte inhibition.

Therapy discontinuation after 6 weeks if echocardiography is unremarkable and platelets and BKS normalized.

Literature
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  1. Cheung YF et al (2004) Novel and traditional cardiovascular risk factors in children after Kawasaki disease: implications for premature atherosclerosis. J Am Coll Cardiol 43: 120-124
  2. Cremer H (1990) The Kawasaki syndrome (mucocutaneous lymph node syndrome). Dt Ärztebl 87: 1526-1531
  3. Dajani AS, Taubert KA, Gerber MA et al (1993) Diagnosis and therapy of Kawasaki disease in children. Circulation 87: 1776-1780
  4. Kawasaki T (1967) Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the finger and toes in children. Jap J Allerg 16: 178.
  5. Lee CS, Lim HW (2003) Cutaneous diseases in Asians. Dermatol Clin 21: 669-677
  6. Machet L et al (1990) Kawasaki disease in a young adult with perineal rash. Br J Dermatol 123: 413
  7. Needle S, Levin M (1993) Kawasaki disease. Curr Opin Pediatr 5: 29-34
  8. Onouchi Y (2018) The genetics of Kawasaki disease. Int J Rheum Dis 21:26-30.

  9. Shaukat N, Ashraf S, Mebewu A et al (1993) Myocardial infarction in a young adult due to Kawasaki disease. A case report and review of the late cardiological sequelae of Kawasaki disease. Int J Cardiol 39: 222-226

  10. Teraki Y et al (1994) Dermatoses described in Japan. Dermatologist 45: 125-131
  11. Wortman DW (1992) Kawasaki syndrome. Semin Dermatol 11: 37-47

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 29.10.2020