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Kawasaki Disease - Ten Fascinating Facts 

Dr Sampurna Roy MD   

 

                  

                  

Necrotizing Arteritis (NA) and severe subacute/chronic  (SA/C) pan-arteritis.

A. Portion of a coronary artery (CA) undergoing NA. The friable fragmenting wall is a mixture of neutrophils and debris. The adventitia is virtually obscured by inflammation and RBCs. 

B. Higher magnification of an area of CA NA predominantly of neutrophils.  

C. The necrotizing process has reached into the adventitia, which is only mildly inflamed.

D. An area of coronary artery aneurysms (CAA) consistent with having undergone severe SA/C pan-arteritis, leaving only adventitia rich in SA/C inflammatory cells, almost exclusively small lymphocytes. Note the longitudinally sectioned vessel and fibrin/RBC lining the luminal surface.

 

Source: Orenstein JM, Shulman ST, Fox LM, et al. Three Linked Vasculopathic Processes Characterize Kawasaki Disease: A Light and Transmission Electron Microscopic Study. Moretti C, ed. PLoS ONE. 2012;7(6):e38998. 

 

(1) Kawasaki disease was first reported as an acute febrile mucocutaneous lymph-node syndrome by Tomisaku Kawasaki in 1967.

 

(2) It is referred to as a "self-limited acute vasculitis", which predominantly strikes children three months to five years of age.

However, some cases have been reported in adults.

 

(3) Kawasaki disease is most prevalent in Japan, while Korea holds the second place as to the number of patients.

Its incidence in Japanese and Korean children living in the USA and following a Western lifestyle is higher than in Caucasian children.

In Japan 1% of children develop the disease by age five.   

 

(4) With control of rheumatic heart disease, Kawasaki disease is recognized as the most common acquired childhood heart disease in developed nations.

Severely affected patients show coronary artery lesions such as aneurysms and ectasias, which develop in approximately one quarter of untreated children.

 

(5) Clinical manifestations of Kawasaki disease include prolonged fever (1-2 weeks, mean 10-11 days), conjunctival injection, oral lesions, polymorphous skin rashes, extremity changes, and cervical lymphadenopathy.

In addition, arthritis, aseptic meningitis, anterior uveitis, gall bladder hydrops, urethritis and lung involvement can be seen.

 

(6) Exact etiology of Kawasaki disease remains unknown. Possible causes considered are infectious and genetic.

 

(7) The most striking pathologic finding at autopsy is necrotizing panarteritis of coronary arteries with multiple aneurysms.

The inflammation may be acute or chronic depending on the duration of the illness.

Fibrous arterial scars with luminal stenosis are seen in later stages.

 

(8) Necrotizing panarteritis is often found in iliac, renal, mesenteric, hepatic and pancreatic arteries, occasionally with aneurysms.

 

(9) In Kawasaki disease, many inflammatory indices change throughout the disease process. 

Elevated levels of C-reactive protein , erythrocyte sedimentation rate , leukocyte count with neutrophilia (lymphopenia), platelet count, alanine aminotransferase , aspartate aminotransferase and other inflammation associated enzymes, as well as decreased levels of lymphocytes, albumin, hemoglobin, sodium, potassium and total cholesterol including high density lipoprotein cholesterol (HDL-cholesterol) have been detected.

 

(10) Diseases to be differentiated from Kawasaki are viral and bacterial infections, drug hypersensitivity syndromes, toxic shock syndrome and autoimmune diseases with idiopathic juvenile arthritis.

 

Source

 

Orenstein JM, Shulman ST, Fox LM, et al. Three Linked Vasculopathic Processes Characterize Kawasaki Disease: A Light and Transmission Electron Microscopic Study. Moretti C, ed. PLoS ONE. 2012;7(6):e38998. 

 

Lee K-Y, Rhim J-W, Kang J-H. Kawasaki Disease: Laboratory Findings and an Immunopathogenesis on the Premise of a “Protein Homeostasis System.” Yonsei Medical Journal. 2012;53(2):262-275. doi:10.3349/ymj.2012.53.2.262.

 

 

 

 

 

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