Selective IgA deficiency complicated by Kawasaki syndrome

Treatment of a patient with Kawasaki disease associated with selective IgA deficiency by continuous infusion of cyclosporine A without intravenous immunoglobulin

“…Nishikawa et al 8 reported a case of a KD patient associated with sIgAD, who was treated with steroid pulse therapy (intravenous methylprednisolone 30 mg/kg/day for 3 days) and improved without CAL. However, it is well known that steroid therapy for KD without IVIG is a contraindication and increased the incidence of CAL on acute phase of KD 9 .…”

Selective immunoglobulin A deficiency complicated by Kawasaki syndrome: Reply

“…We are grateful for the interest that Rowley et al. showed in our paper 1 . We reported an extremely rare case of selective immunoglobulin (Ig) A deficiency in a patient who developed Kawasaki syndrome (KS).…”

“…We are grateful for the interest that Rowley et al showed in our paper. 1 We reported an extremely rare case of selective immunoglobulin (Ig) A deficiency in a patient who developed Kawasaki syndrome (KS). Rowley et al reported that IgA plasma cells were predominantly detected in KS arterial tissues, 2 that the vascular IgA response in the acute KS patient is oligoclonal based on sequencing of the CDR3 regions of a genes from KS vascular tissues, 3 and that synthetic KS antibodies from IgA sequences target cytoplasmic inclusion bodies in acute KS ciliated bronchial epithelium.…”

Immunoglobulin A deficiency and Kawasaki disease

“…Nishikawa et al. 1 reported a case of Kawasaki disease with selective immunoglobulin (Ig) A deficiency, and stated that this case provides evidence against the theory of the pathogenesis of Kawasaki disease (KD) proposed by our research group 2 …”