Multiple pulmonary-artery aneurysms due to mucoid vasculopathy--angiographic and histological observations

Multiple peripheral pulmonary artery aneurysms were diagnosed by computed tomography and confirmed angiographically in a 7-year-old boy. On histological examination unusual mucoid vasculopathic changes were noted in the pulmonary artery aneurysms and pulmonary vasculature as a part of systemic vascular involvement. This case demonstrates a new association between mucoid vasculopathic changes and pulmonary aneurysms. Mucoid vasculopathy could represent one more disorder having a causative role in the etiology of intrapulmonary arterial aneurysms.