The involvement of Fc gamma receptor gene polymorphisms in Kawasaki disease

Kawasaki disease is an acute febrile syndrome in infancy, characterized by vasculitis of medium-sized arteries. Without treatment the disease can lead to coronary artery lesions (CAL) in approximately 25% of the children. Therapy consists of intravenous immunoglobulins (IVIG), leading to a decrease of complications to 5-16%. Little is known about the working mechanisms of IVIG. In this study we evaluated the involvement of Fc gamma receptors (Fc gamma Rs) in Kawasaki disease by the determination of the frequency of known single nucleotide polymorphisms (SNPs) in the genes coding for the Fc gamma Rs and compared this with frequencies in a cohort of healthy controls. There was no difference in the distribution of the functionally relevant genotypes for Fc gamma RIIa-131H/R, Fc gamma RIIb-2321/T, Fc gamma RIIIa-158 V/F and Fc gamma RIIIb-NA1/NA2 between the patient group and the healthy controls. Furthermore, there were no polymorphisms linked to the disease severity as indicated by the absence or development of CAL during the disease. Altered transcription or expression of Fc gamma R on specific cell types of the immune system may still play a role in susceptibility and treatment success, but at a level different from the functional SNPs in Fc gamma R genes tested in this study.