Hypertensive Pulmonary Arteritis

NECROTIZING arteritis of the pulmonary arterial vasculature may be a manifestation of a variety of conditions including the allergic angiitides and various cardiac and pulmonary diseases associated with pulmonary hypertension. There are 16 recorded instances^1-14 of idiopathic necrotizing lesions of the pulmonary arterial system where the only associated condition was pulmonary hypertension or cor pulmonale. This condition was reported only in children and young adult women. Diagnoses of "primary pulmonary arteritis"^2,8 or "pulmonary polyarteritis nodosa"^3,6,7,15 were made, the latter term implying a hyperimmune or allergic etiology. There is now adequate evidence, however, to support the view that many instances of arteritis limited to the pulmonary vasculature are neither primary nor a form of the connective tissue disease polyarteritis nodosa. The purpose of this communication is to present two cases of necrotizing pulmonary arteritis in children, emphasizing the relation of the vasculitis to pulmonary hypertension.

Report of Cases