Immunologic abnormalities in canine juvenile polyarteritis syndrome: a naturally occurring animal model of Kawasaki disease.

This study describes the immunologic abnormalities during the acute phase of juvenile polyarteritis syndrome (JPS), a multisystem necrotizing vasculitis of young dogs with a predilection for the coronary arteries. JPS has striking clinical, laboratory, and pathologic similarities to Kawasaki disease (KD), the most common cause of acquired heart disease in children in the United States. The immunologic abnormalities include an increase in serum IgA, an increase in the percentage of peripheral B cells and a decrease in the percentage of total peripheral T cells, a marked suppression of the blastogenic response to mitogenic stimulation, an inability to generate immunoglobulin-secreting plasma cells following polyclonal activation, the presence of antineutrophil cytoplasmic antibodies, and evidence of monocyte/macrophage activation. These immunoregulatory abnormalities are similar to those observed in children during the acute phase of KD. This unique, naturally occurring animal model of necrotizing vasculitis may prove useful for investigating novel therapeutic interventions in the treatment of necrotizing vasculitis and may yield insight into the immunopathology and etiology of KD.