Fatal Granulomatosis of the Respiratory Tract (Lethal Midline Granuloma-Wegener's Granulomatosis)

Fatal granulomatosis of the respiratory tract is a disease with a noteworthy natural history, recognizable clinical manifestations, and identifying pathologic characteristics. Men, women, and children of all ages are susceptible, many of whom have no previous stigmata of allergic or vascular disease. The earliest reference to this disease appeared in 1897, when McBride1 1 reported bizarre ulcerative granulomatous lesions in "a case of rapid destruction of the nose and face." Similar cases have been reported by Fordyce, 2 Woods, 3 Falchi, 6 Chatellier, 7 Kraus, 8 Stewart, 13 and others. In 1936 and 1939, Wegener 26,27 published three cases characterized by necrotizing granulomatosis of the nose, nasal accessory sinuses, larynx, and lungs associated with a "peculiar granulomatous nephritis." Wegener believed that the nose was the primary site of the disease and applied the term "rhinogenous granulomatosis" to the disease process. A review of the available literature from 1897 to 1957