Platelet immune complex interaction in pathogenesis of Kawasaki disease and childhood polyarteritis.
The role of platelets in the pathogenesis of vasculitis and the formation of coronary artery aneurysms was studied in 19 children with Kawasaki disease and five with polyarteritis. All patients with Kawasaki disease developed thrombocytosis in the third week of illness. The peak platelet count was significantly correlated (p less than 0.005) with the subsequent development of coronary artery aneurysms. The rise in platelet count was associated with the appearance in the circulation of a factor that induced aggregation and serotonin release in normal platelets. This factor was shown to be of high molecular weight, and its activity was lost at low pH--features suggestive of an immune complex. Immune complexes, detected by precipitation with polyethylene glycol, also appeared in the circulation as the platelet count increased. These complexes induced platelet aggregation, and there was a significant correlation (p less than 0.001) between the concentrations of IgG and IgA in the polyethylene glycol precipitated material and the platelet aggregating activity. Similar platelet aggregating activity was also detected in patients with polyarteritis but followed a different time course, persisting in the circulation for several months in association with continued disease activity. These findings imply that different mechanisms have a role in distinct phases of Kawasaki disease. The initial feverish phase (probably infective) is probably followed by an immune complex vasculitis that occurs when antibodies to the initiating agent appear in the circulation. The immune complexes aggregate platelets and induce release of serotonin. Platelet derived vasoactive mediators may increase vascular permeability and facilitate further deposition of complexes in the tissues.
D000783:Aneurysm / Q000209:etiology; D000936:Antigen-Antibody Complex / Q000032:analysis / Q000502:physiology*; D001167:Arteritis / Q000097:blood / Q000209:etiology*; D001792:Blood Platelets / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D003327:Coronary Disease / Q000209:etiology; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000097:blood / Q000209:etiology*; D010974:Platelet Aggregation; D010976:Platelet Count; D012701:Serotonin / Q000097:blood; D013922:Thrombocytosis / Q000150:complications; D013997:Time Factors
D000936:Antigen-Antibody Complex; D012701:Serotonin