SYSTEMIC ARTERY ANEURYSMS IN KAWASAKI DISEASE — A COMPREHENSIVE REVIEW
Introduction: Kawasaki Disease (KD) is a self-limited, unknown-cause febrile vasculitis that predominantly addresses medium-sized arteries and most patients are under 5-years-old. In addition to life-threatening coronary artery involvement, aneurysms may develop in almost any medium-sized vessel, however systemic artery aneurysms (SAA) are described in only 2% of cases. The objective of this study was to identify cases of KD associated SAA and to analyze their frequency, anatomical distribution, treatment and outcomes.
Methods: systematic literature review, performed using MEDLINE database.
Results: Literature review retrieved a total of 9 reports corresponding to 38 patients and a total of 134 KD-SAA. The most affected arteries were the brachial artery (29.8%), the internal iliac artery (18,6%), the common iliac artery (16,4%) and the subclavian artery (11,2%) with bilateral involvement being a common feature. History of concomitant coronary involvement was present in almost every patients. Only 5 SAA (3,7%) required intervention. Medical therapy was associated with SAA regression in approximately half of the patients, with better results when initiated promptly after diagnosis. Worst outcomes were found in younger patients, showing higher rates of morbimortality. The underlying coronary sequelae dictated the prognosis.
Conclusions: Considering their rarity, only multicenter collaboration and literature research can provide more insight about KD-SAA treatment and outcomes, defining the potential role of KD as a primary cause of late vascular lesion. Late presentation KD-SAA may present some diagnostic and therapeutic challenges in view of a more likely need for intervention once the benefit of medical treatment has been outweighed.
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