Kawasaki disease

Kawasaki disease

Introduction

Kawasaki disease is a type of vasculitis where the endothelial cells in the coronary arteries become damaged, potentially leading to complications like myocardial infarction and aneurysms.
  • Alson known as mucocutaneous lymph node syndrome
  • Medium-vessel vasculitis
  • Usually seen in asian children < 4 years old
figure-2-distribution-of-vessel-involvement-by-large-vessel-vasculitis-medium-vessel
Distribution of vessel involvement by large vessel vasculitis, medium vessel vasculitis, and small vessel vasculitis. Note that there is substantial overlap with respect to arterial involvement, and an important concept is that all 3 major categories of vasculitis can affect any size artery. Large vessel vasculitis affects large arteries more often than other vasculitides. Medium vessel vasculitis predominantly affects medium arteries. Small vessel vasculitis predominantly affects small vessels, but medium arteries and veins may be affected, although immune complex small vessel vasculitis rarely affects arteries. Not shown is variable vessel vasculitis, which can affect any type of vessel, from aorta to veins. The diagram depicts (from left to right) aorta, large artery, medium artery, small artery/arteriole, capillary, venule, and vein. Anti-GBM anti–glomerular basement membrane; ANCA antineutrophil cytoplasmic antibody. | Revised international chapel hill consensus conference nomenclature of vasculitides – Scientific Figure on ResearchGate. Available from: https://www.researchgate.net/264457553_fig1_Figure-1-Types-of-vessels-that-are-defined-as-large-vessels-A-medium-vessels-B-and [accessed 7 Sep, 2017]

History

The disease was first reported by Tomisaku Kawasaki in a four-year-old child with a rash and fever at the Red Cross Hospital in Tokyo in January 1961, and he later published a report on 50 similar cases. Later, Kawasaki and colleagues were persuaded of definite cardiac involvement when they studied and reported 23 cases, of which 11 (48%) patients had abnormalities detected by an electrocardiogram. In 1974, the first description of this disorder was published in the English-language literature. In 1976, Melish et al. described the same illness in 16 children in Hawaii. Melish and Kawasaki had independently developed the same diagnostic criteria for the disorder, which are still used today to make the diagnosis of classic Kawasaki disease.

drkawasaki
Dr. Tomisaku Kawasaki (centre right) at the 8th International Kawasaki Disease Symposium, 2005

Pathophysiology

Complication: Coronary artery aneurysms

Thrombosis/rupture can cause death
  • Can present as arrhythmias, myocardial infarction (MI), heart failure
  • Risk factors:
    • Age < 1 year or > 9 years, males sex
    • Fever ≥ 14 days
    • Hematocrit < 35%
    • Serum Na < 135 mEq/L
    • WBC count > 12,000/mm3
Coronary arteries are demonstrated in red. The panel on the left shows a heart with normal coronary arteries, and the panel on the right shows a heart with large aneurysms of 2 types: fusiform (cigar shaped) and saccular (bubble shaped).

Clinical features

Classic symptoms (CRASH & burn):

  • Conjunctivitis (sparing limbus)
  • Rash
    • Affects whole body
    • Progression: Polymorphous → Desquamation
  • Adenopathy (cervical)
  • Strawberry tongue (oral mucositis)
  • Hand-foot changes (oedema, erythema)
  • High-fever
kawasaki
(A) Bilateral, non-exudative conjunctival injection with perilimbal sparing. (B) Strawberry tongue and bright red, swollen lips with vertical cracking and bleeding. (C) Erythematous rash involving perineum. (D) Erythema of the palms, which is often accompanied by painful, brawny edema of the dorsa of the hands. (E) Erythema of the soles, and swelling dorsa of the feet. (F) Desquamation of the fingers. (G) Erythema and induration at the site of a previous vaccination with Bacille Calmette-Gurin (BCG). (H) Perianal erythematous desquamation. | By Dong Soo Kim – Kawasaki disease., CC BY-SA 4.0, https://commons.wikimedia.org/w/index.php?curid=9962875
kawasakidiseasemanifestations
Signs and symptoms and time course of Kawasaki disease | By Maen K Househ – Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=17543116

Diagnosis

Diagnostic criteria:

4 out of 5 CRASH symptoms + fever for ≥ 5 days
  • DEFINITIVE DIAGNOSIS: Vasculitis in coronary arteries

Blood panel:

  • Anaemia
  • ↑ WBC (left shift, i.e. more immature WBCs)
  • ↑ C-reactive protein (CRP)
  • ↑ ESR
  • ↑ Liver enzymes

Urinalysis:

  • Mononuclear WBCs in urine (without bacteria)
  • ↑ Platelets

Echocardiogram (ECG):

Coronary artery & heart muscle involvement

Management

  • IV Ig
  • Aspirin
    • Inhibits COX → Prevents platelet aggregation
    • Contraindicated in children due to Reye’s syndrome

Summary

Kawasaki Syndrome.jpg

Further reading

  • Kim DS. Kawasaki disease. Yonsei Med J [Internet]. 2006 Dec 31 [cited 2017 Jan 12];47(6):759–72. Available from: http://www.ncbi.nlm.nih.gov/pubmed/17191303
  • Fimbres AM, Shulman ST. Kawasaki Disease. Pediatr Rev. 2008;29(9)
  • Diagnostic Guidelines for Kawasaki Disease. Circulation. 2001;103(2)
  • Baker AL, Newburger JW. Kawasaki Disease. Circulation. 2008;118(7)


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