Document Detail

Three linked vasculopathic processes characterize Kawasaki disease: a light and transmission electron microscopic study.
MedLine Citation:
PMID:  22723916     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Kawasaki disease is recognized as the most common cause of acquired heart disease in children in the developed world. Clinical, epidemiologic, and pathologic evidence supports an infectious agent, likely entering through the lung. Pathologic studies proposing an acute coronary arteritis followed by healing fail to account for the complex vasculopathy and clinical course.
METHODOLOGY/PRINCIPAL FINDINGS: Specimens from 32 autopsies, 8 cardiac transplants, and an excised coronary aneurysm were studied by light (n=41) and transmission electron microscopy (n=7). Three characteristic vasculopathic processes were identified in coronary (CA) and non-coronary arteries: acute self-limited necrotizing arteritis (NA), subacute/chronic (SA/C) vasculitis, and luminal myofibroblastic proliferation (LMP). NA is a synchronous neutrophilic process of the endothelium, beginning and ending within the first two weeks of fever onset, and progressively destroying the wall into the adventitia causing saccular aneurysms, which can thrombose or rupture. SA/C vasculitis is an asynchronous process that can commence within the first two weeks onward, starting in the adventitia/perivascular tissue and variably inflaming/damaging the wall during progression to the lumen. Besides fusiform and saccular aneurysms that can thrombose, SA/C vasculitis likely causes the transition of medial and adventitial smooth muscle cells (SMC) into classic myofibroblasts, which combined with their matrix products and inflammation create progressive stenosing luminal lesions (SA/C-LMP). Remote LMP apparently results from circulating factors. Veins, pulmonary arteries, and aorta can develop subclinical SA/C vasculitis and SA/C-LMP, but not NA. The earliest death (day 10) had both CA SA/C vasculitis and SA/C-LMP, and an "eosinophilic-type" myocarditis.
CONCLUSIONS/SIGNIFICANCE: NA is the only self-limiting process of the three, is responsible for the earliest morbidity/mortality, and is consistent with acute viral infection. SA/C vasculitis can begin as early as NA, but can occur/persist for months to years; LMP causes progressive arterial stenosis and thrombosis and is composed of unique SMC-derived pathologic myofibroblasts.
Jan Marc Orenstein; Stanford T Shulman; Linda M Fox; Susan C Baker; Masato Takahashi; Tricia R Bhatti; Pierre A Russo; Gary W Mierau; Jean Pierre de Chadarévian; Elizabeth J Perlman; Cynthia Trevenen; Alexandre T Rotta; Mitra B Kalelkar; Anne H Rowley
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2012-06-18
Journal Detail:
Title:  PloS one     Volume:  7     ISSN:  1932-6203     ISO Abbreviation:  PLoS ONE     Publication Date:  2012  
Date Detail:
Created Date:  2012-06-22     Completed Date:  2012-12-12     Revised Date:  2014-06-03    
Medline Journal Info:
Nlm Unique ID:  101285081     Medline TA:  PLoS One     Country:  United States    
Other Details:
Languages:  eng     Pagination:  e38998     Citation Subset:  IM    
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MeSH Terms
Aneurysm / etiology
Aneurysm, Ruptured / etiology
Cell Proliferation
Child, Preschool
Lymphocytes / pathology
Mucocutaneous Lymph Node Syndrome / complications,  etiology*,  pathology*
Myocarditis / etiology,  metabolism,  pathology
Myocytes, Smooth Muscle / metabolism,  pathology,  ultrastructure
Myofibroblasts / metabolism,  pathology,  ultrastructure
Neutrophils / pathology
Thrombosis / etiology
Grant Support

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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