Noninfectious Necrotizing Granulomatosis: The Pulmonary Roentgen Signs

Rencently we have been stimulated to peruse the literature concerning Wegener's granulomatosis and periarteritis nodosa, the stimulus being provided by the circumstance of seeing examples in the brief span of six months. The cases with roentgen findings which have been reported number approximately 25. From these, a pattern of pulmonary roentgen shadows consisting of nodular and infiltrative densities seems to emerge. It is the purpose of this article to add to the knowledge concerning these radiologic signs. Descriptive Profile Felson and Braunstein (5) coined the term “noninfectious necrotizing granulomatosis” to embrace several entities which appear to be related. Among these are Wegener's granulomatosis, Churg and Strauss' granulomatosis, midline lethal granuloma, allergic or hypersensitivity angiitis, and pulmonary granulomatosis. The relationship of periarteritis nodosa to this group is a matter of controversy. Histologically the necrotizing angiitis of periarteritis nodosa is quite similar to the angiitis of necrotizing granulomatosis. There is evidence, therefore, for the assumption that periarteritis nodosa is merely a manifestation of the vascular component of necrotizing granulomatosis without the formation of aseptic granulomas. It is proper that the pathologists continue to strive to resolve the problems of proper classification, etiology, and pathogenesis. For the purpose of the radiologist, who is attempting to recognize the pulmonary roentgen signs, it is well to consider all of the entities as manifestations of a single disease process. To elaborate further, these disorders have in common a histologic response characterized by aseptic granulomas and necrotizing inflammation of the arteries and veins. The changes are compatible with a hypersensitivity reaction such as has been produced experimentally in animals. Evidence has accumulated to support speculation that the etiologic factor in man is an allergy (1, 2). The granulomas vary in size from a few millimeters to more than 5 cm. in diameter. They are surrounded by inflammatory cells and have friable and necrotic centers. Eosinophilia may be a prominent feature. The vascular alterations consist in localized fibrinoid necrosis followed by inflammatory reaction. The organ system primarily affected gives rise to the clinical manifestations. Most prominent are necrotizing granulomas of the respiratory tract, generalized angiitis, and necrotizing glomerulitis (2, 4). A disease pattern begins to evolve. The diagnosis depends on evaluation of an allergic history, destructive granulomas of facial and respiratory structures, granulomas of the skin, eosinophilia, renal disease, and roentgen signs. With the exception of those cases which show only the hypersensitivity angiitis, noninfectious necrotizing granulomatosis has invariably terminated fatally after months or several years.