Systemic Artery Aneurysms and Kawasaki Disease

Abstract

BACKGROUND: Coronary artery aneurysms (CAAs) are a well-known complication of Kawasaki disease (KD), but there are no data on incidence or outcomes of systemic artery aneurysms (SAAs) in the current era. METHODS: From April 1, 2016, to March 31, 2019, we screened for SAAs in 162 patients with KD at risk for SAAs with magnetic resonance angiography or peripheral angiography and analyzed incidence and early outcomes of SAAs. RESULTS: Twenty-three patients had SAAs, demonstrating an incidence of 14.2% (23 of 162) in patients who were screened at 1 month after onset. The proportion of patients with SAAs was estimated to be 2% (23 of 1148) of all patients with KD. The median age at onset of KD with SAAs was 5 months. All patients with SAAs had CAAs, with z scores .8. Of patients with giant CAAs, 38.6% (17 of 44) had SAAs. A total of 129 SAAs occurred in 17 different named arteries. The most common sites for SAAs were the axillary (18.6%), common iliac (12.4%), and brachial (11.6%) arteries. During a median follow-up time of 6 months, 92.9% (79 of 85) of SAAs had some degree of regression, with 80% (68 of 85) of SAAs returning to normal. The overall regression rate was higher for medium to large SAAs than for medium to giant CAAs. CONCLUSIONS: Although the incidence of SAAs may not be as dramatically reduced as we expected compared with previous data, SAAs have a high regression rate during short-term follow-up. WHAT'S KNOWN ON THIS SUBJECT: Studies from the 1980s revealed that systemic artery aneurysms (SAAs) may occur in 0.8% to 2% of patients with Kawasaki disease at 3 months after onset. It is speculated that SAAs are now much less common than before. WHAT THIS STUDY ADDS: SAAs occur in 14.2% of patients at risk for SAAs at 1 month after onset, giving an estimated proportion of patients with SAAs as 2% of all patients with Kawasaki disease. SAAs have a high regression rate during short-term follow-up.

Neonatal Kawasaki disease with multiple arterial aneurysms: a case report

“…A previous study by our team demonstrated that the incidence of SAAs in KD is not as low as we thought. Longer duration of fever, larger CAAs, and younger age may be risk factors for SAAs, with the regression rate of SAAs was better than that of CAAs over time [11]. The clinical features and prognosis of this newly identified patient are consistent with our previous findings, and again suggest that clinicians should be alert to the possibility of SAAs in KD, as large SAAs may progress to obstructive lesions or cause ischemic symptoms.…”

“…The internal diameter of the LAD and RCA were 3.5 mm (z score = 6.7) and 2.9 mm (z score = 5.8), respectively. Full-body magnetic resonance angiography (MRA), performed routinely in patients with medium to giant CAAs in our institution [11], also revealed bilateral axillary artery aneurysms that could not be palpated on physical examination ( Fig. 1).…”

“…When misdiagnosed as other infectious diseases, affected children are at risk for delayed diagnosis and coronary artery aneurysms (CAAs) [10]. KD-related systemic artery aneurysms (SAAs) are currently thought to be not uncommon [11] but have never been reported in neonates. Here we report a case of delayed diagnosis of neonatal KD with both coronary artery and axillary artery aneurysms.…”

Cardiovascular Involvement in Kawasaki Disease Is Much More Than Mere Coronary Arteritis

“…Since than there have been reports of SAAs involving large arteries (e.g., iliac, femoral, subclavian, axillary). Recently Zhao et al have reported full-body magnetic resonance angiography (MRA) or peripheral angiography in patients with KD for identification of SAAs (108). MRA (n = 110) was performed in patients with KD who had presumed risk factors for SAAs (e.g., patients having giant coronary aneurysm, increasing size aneurysm during acute phase or IVIg resistant KD).…”

“…Most commonly affected arteries were axillary and common iliac. Risk factors for development of SAAs were young age and pronged fever ( 108 ). It appears that KD may also have a component of systemic vasculitis but this needs more detailed evaluation ( 107 – 109 ).…”