Files and links (1)
Published, Version of Record (VoR)CC BY V4.0, Open
Journal article
Three Linked Vasculopathic Processes Characterize Kawasaki Disease: A Light and Transmission Electron Microscopic Study
PloS one, v 7(6), 38998
18 Jun 2012
PMID: 22723916
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
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.
Metrics
8 Record Views
Details
- Title
- Three Linked Vasculopathic Processes Characterize Kawasaki Disease: A Light and Transmission Electron Microscopic Study
- Creators
- Jan Marc Orenstein - George Washington UniversityStanford T. Shulman - Northwestern UniversityLinda M. Fox - Loyola University ChicagoSusan C. Baker - Loyola University ChicagoMasato Takahashi - Univ So Calif, Dept Pediat, Childrens Hosp Los Angeles, Sch Med, Los Angeles, CA 90089 USATricia R. Bhatti - Children's Hospital of PhiladelphiaPierre A. Russo - Children's Hospital of PhiladelphiaGary W. Mierau - Children's Hospital ColoradoJean Pierre de Chadarevian - Drexel UniversityElizabeth J. Perlman - Lurie Children's HospitalCynthia Trevenen - Alberta Children's HospitalAlexandre T. Rotta - Lurie Children's HospitalMitra B. Kalelkar - Office of the Medical Examiner of Cook CountyAnne H. Rowley - Northwestern Univ, Childrens Mem Hosp, Dept Microbiol Immunol, Feinberg Sch Med, Chicago, IL 60614 USA
- Publication Details
- PloS one, v 7(6), 38998
- Publisher
- Public Library Science
- Number of pages
- 25
- Grant note
- Children's Memorial Hospital R01HL63771; R21HL109955; R21HL089526 / National Institutes of Health; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA Max Goldenberg Foundation R21HL089526 / NATIONAL HEART, LUNG, AND BLOOD INSTITUTE; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Heart Lung & Blood Institute (NHLBI)
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Pathology (and Laboratory Medicine)
- Web of Science ID
- WOS:000305583300078
- Scopus ID
- 2-s2.0-84862497099
- Other Identifier
- 991021893404904721
UN Sustainable Development Goals (SDGs)
This publication has contributed to the advancement of the following goals:
InCites Highlights
Data related to this publication, from InCites Benchmarking & Analytics tool:
- Collaboration types
- Domestic collaboration
- International collaboration
- Web of Science research areas
- Cardiac & Cardiovascular Systems