Eighteen young Beagle dogs (eight males and 10 females), ages 6-40 mon
ths, with canine juvenile polyarteritis syndrome (CJPS), a naturally o
ccurring vasculitis and perivasculitis of unknown etiology, were necro
psied, and their tissues were examined by histopathologic and histoche
mical methods. The condition is characterized by recurring episodes of
an acute onset of fever (>40 C) and neck pain that persist for 3-7 da
ys. The major histopathologic alterations were a systemic vasculitis a
nd perivasculitis. During the febrile, painful period of CJPS, the vas
cular lesions ranged from a histiocytic-lymphocytic periarterial infil
tration to transmural arterial inflammation with concomitant fibrinoid
necrosis and vascular thrombosis. Massive periarterial accumulations
of inflammatory cells were common and often extended into adjacent tis
sues. The small- to medium-sized muscular arteries of the heart, crani
al mediastinum, and cervical spinal meninges were consistently involve
d. Vasculitis occasionally occurred in other organ systems. The vascul
ar lesions in dogs examined during clinically normal periods consisted
of intimal and medial fibrosis, ruptured elastic laminae, and mild pe
rivasculitis; these lesions were probably related to previous episodes
of vasculitis. Eight dogs that had experienced repeated acute episode
s also developed splenic, hepatic, and renal amyloidosis. The clinical
signs, laboratory abnormalities, and the vascular lesions suggest tha
t the condition may be immune-system mediated. CJPS may serve as a nat
urally occurring animal model of human immune-system-mediated vasculit
ides such as polyarteritis nodosa, infantile polyarteritis, and Kawasa
ki disease.