Open AccessPathogenetic determinants in Kawasaki disease: the haematological point of view
Author(s) -
Del Principe Domenico,
Pietraforte Donatella,
Gambardella Lucrezia,
Marchesi Alessandra,
Tarissi de Jacobis Isabella,
Villani Alberto,
Malorni Walter,
Straface Elisabetta
Publication year - 2017
Publication title -
journal of cellular and molecular medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.44
H-Index - 130
eISSN - 1582-4934
pISSN - 1582-1838
DOI - 10.1111/jcmm.12992
Subject(s) - kawasaki disease , medicine , vasculitis , disease , rash , vascular disease , coronary artery disease , genetic predisposition , pathology , artery , dermatology , immunology , cardiology
Kawasaki disease is a multisystemic vasculitis that can result in coronary artery lesions. It predominantly affects young children and is characterized by prolonged fever, diffuse mucosal inflammation, indurative oedema of the hands and feet, a polymorphous skin rash and non‐suppurative lymphadenopathy. Coronary artery involvement is the most important complication of Kawasaki disease and may cause significant coronary stenosis resulting in ischemic heart disease. The introduction of intravenous immunoglobulin decreases the incidence of coronary artery lesions to less than 5%. The etiopathogenesis of this disease remains unclear. Several lines of evidence suggest that an interplay between a microbial infection and a genetic predisposition could take place in the development of the disease. In this review, we summarize the state of the art of pathogenetic mechanisms of Kawasaki disease underscoring the relevance of haematological features as a novel field of investigation.