Large Vessel Pulmonary Arteritis

Abstract

Takayasu’s arteritis (TA) and giant cell arteritis (GCA) are the most well known large vessel vasculitides. Although similar in histologic appearance, they have been divided into two distinct clinical entities on the basis of their age of onset, clinical presentation, and distribution of vascular involvement. Although both may involve large and mediumsized arteries, including the aorta and its branches, the vast majority of large vessel pulmonary arteritis cases reported in the literature are attributed to TA. TA will therefore be the primary focus of this chapter. TA is named after Mikito Takayasu, a Japanese ophthalmologist who first reported a case of a 21-year-old woman with an interesting fundoscopic finding of coronary anastomosis of the retinal central vessels. Takayasu did not report on any other physical findings in the patient during his presentation at the Ophthalmology Society in Japan in 1905. However, at this same meeting two other ophthalmologists, Onishi and Kagoshima, presented two additional cases with the same retinal abnormalities associated with absent radial pulses. TA is now recognized to be a vasculitis whose characteristic ophthalmic findings are a result of retinal artery ischemia. TA is an acute and chronic form of vasculitis of the elastic arteries resulting in stenosis, occlusion, and occasionally aneurysmal changes of the aorta, its main branches, pulmonary arteries, and coronary arteries. It is a disease that predominantly affects young women, with symptoms determined by the distribution of the vessels involved, the type of vascular lesion, and the presence of active inflammation. Treatment strategies are focused on suppressing inflammation with corticosteroids or other immunosuppressive agents, but revascularization procedures may be required in some patients.