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Pediatr Dev Pathol. 2015 Jul-Aug;18(4):310-7. doi: 10.2350/14-12-1588-OA.1. Epub 2015 Apr 9.

Role of TGF-β Signaling in Remodeling of Noncoronary Artery Aneurysms in Kawasaki Disease.

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1 Kawasaki Disease Research Center, Department of Pediatrics, University of California San Diego and Rady Children's Hospital, La Jolla, CA, USA.
2 Toho University, Ohashi Medical Center, Tokyo, Japan.
3 Department of Medicine, University of California San Diego, La Jolla, CA, USA.
4 Department of Cardiothoracic Surgery, Sharp Memorial Hospital, San Diego, CA, USA.
5 San Diego Cardiac Center and Sharp Memorial Hospital, San Diego, CA, USA.


Coronary artery aneurysms (CAA) remain an important complication of Kawasaki disease (KD), the most common form of pediatric acquired heart disease in developed countries. Potentially life-threatening CAA develop in 25% of untreated children and 5% of children treated with high-dose intravenous immunoglobulin during the acute phase of the self-limited vasculitis. Noncoronary artery aneurysms (NCAA) in extraparenchymal, muscular arteries occur in a minority of patients with KD who also have CAA, yet little is understood about their formation and remodeling. We postulated that activation of the transforming growth factor-β (TGF-β) pathway in KD may influence formation and remodeling of aneurysms in iliac, femoral, and axillary arteries, the most common sites for NCAA. We studied a resected axillary artery from one adult and endarterectomy tissue from the femoral artery from a second adult, both with a history of CAA and NCAA following KD in infancy. Histology of the axillary artery aneurysm revealed destruction of the internal elastic lamina and recanalization of organized thrombus, while the endarterectomy specimen showed dense calcification and luminal myofibroblastic proliferation. Immunohistochemistry for molecules in the TGF-β signaling pathway revealed increased expression of TGF-β2, TGF-β receptor 2, and phosphorylated SMAD3. These findings suggest ongoing tissue remodeling of the aneurysms decades after the acute injury and demonstrate the importance of the TGF-β signaling pathway in this process.


Kawasaki disease; aneurysms; axillary artery; endarterectomy; femoral artery; iliac artery

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