Wegener's granulomatosis, lymphomatoid granulomatosis, and benign lymphocytic angiitis and granulomatosis of lung. Recognition and treatment.
Observations on 35 patients with pulmonary angiitis and granulomatosis show the value of separating pulmonary lesions of this type into three categories. Fifteen patients had Wegener's granulomatosis characterized by necrotizing granulomatous inflammation of lung, frequent upper airway and renal involvement, and responsiveness to cyclophosphamide. Nine had lymphomatoid granulomatosis characterized by necrotic atypical lymphoreticular infiltrates and frequent cutaneous and neurologic involvement, usually fatal despite intensive cytotoxic drug therapy. Eleven had benign lymphocytic angiitis and granulomatosis, in the past included in the above categories and characterized by nodular collections of mature lymphocytes and plasma cells, with predominantly pulmonary involvement and consistent responsiveness to chlorambucil. Wegener's granulomatosis and benign lymphocytic angiitis and granulomatosis were frequently associated with serum immunoglobulin elevations, with intact cell-mediated responses. Clinical and immunologic assessment was useful indistinguishing benign lymphocytic angiitis and granulomatosis from lymphomatoid granulomatosis.