Pulmonary angiitis and granulomatosis: a review

Vasculitis is a clinical-pathological process characterized by inflammation and necrosis of blood vessels. It has been effectively classified by Fauci. Granulomatosis in the lung may be angiocentric or bronchocentric in distribution. The angiocentric forms of granulomatosis and vasculitis include Wegener's granulomatosis, allergic angiitis and granulomatosis of Churg and Strauss, lymphomatoid granulomatosis, and necrotizing sarcoid granulomatosis. Wegener's granulomatosis is a well-defined syndrome characterized by necrotizing granulomatous vasculitis of the upper and lower respiratory tracts, segmental necrotizing glomerulonephritis, and systemic small vessel vasculitis. Allergic angiitis and granulomatosis is a less common multisystem vasculitis with many features similar to polyarteritis nodosa. Lymphomatoid granulomatosis is an angiocentric and angiodestructive lymphoreticular proliferative and granulomatous disease involving predominantly the lungs and resembling lymphoma. Necrotizing sarcoid granulomatosis is probably a variant of sarcoidosis in which an angiitis is a prominent feature. Because the radiology of these diseases can be similar, their important differences are highlighted. The appearance of multiple nodules, often with cavities, and pleural-based consolidations resembling pulmonary infarcts should suggest pulmonary angiitis and granulomatosis, especially if improvement occurs in one area while disease is progressing elsewhere. Bronchocentric granulomatosis is not a primary vasculitis but is discussed because of its similarity to the other diseases.