Pulmonary Vasculitis



Pulmonary vasculitis may occur as a part of one of the aforementioned connective tissue disorders or in the course of a systemic granulomatous or hypersensitivity vasculitis.

The granulomatous vasculitides include classic Wegener’s granulomatosis, limited Wegener’s, and lymphomatoid granulomatosis. Classic We­gener’s is a necrotizing vasculitis initially de­scribed as involving three organ systems: the lung, the upper respiratory tract, and the kidneys. How­ever, many other organs in the body may be af­fected. Lung involvement usually takes the form of single or multiple nodular lesions that have a propensity to cavitate. The limited form of We­gener’s granulomatosis refers to patients with sim­ilar pathology but free of renal disease. Both dis­eases respond well to cyclophosphamide. Lymphomatoid granulomatosis resembles Wege­ner’s but differs in three important features: there is frequent central nervous system involvement, more than 15 per cent develop malignant lym­phoma, and although cyclophosphamide may achieve remission, relapses are very common.
In hypersensitivity vasculitis, pulmonary in­volvement is a less prominent part of a systemic disease. The disorders in which this is most com­monly seen are anaphylactoid purpura, essential mixed cryoglobulinemia, and the vasculitis as­sociated with malignancy, infection, or drugs.