Limited forms of angiitis and granulomatosis of Wegener's type

Abstract

Sixteen patients with angiitis and granulomatosis are described, all with massive pulmonary lesions characteristic of Wegener's granulomatosis and seven with extrapulmonary lesions. In none thus far has glomerulitis developed. This experience suggests that limited forms of Wegener's granulomatosis exist in which the prognosis is better than in those with the classic triad. Some patients have survived for long intervals. Clinically and roentgenographically the lesions are usually multiple and bilateral, often simulate primary or metastatic neoplasms of the lung, but may suggest infectious granulomatous disease. A tendency to involve the lower portions of the lung is useful in differentiating between these lesions and those of tuberculosis. The presence of bizarre reticuloendothelial cells may suggest Hodgkin's disease, but angiitis is a distinguishing feature. The pulmonary lesions often resemble "white infarcts" grossly. This appearance is so characteristic as to suggest the nature of the lesion on gross examination. Cavitation occurs in approximately half the cases. The presence of a granulomatous angiitis affecting both arteries and veins is the critical clue to the diagnosis and should be sought in any example of "granuloma of unknown etiology" as diligently as are the common infectious agents. To be considered in differential diagnosis are rheumatoid nodules and plasma cell granulomas. The relationship to "lethal midline granuloma" and other forms of granulomatosis and angiitis is not as yet clear. It is noteworthy that some of these patients also had sarcoid-like granulomas in the lungs, which are common in many forms of hypersensitivity reactions involving these organs. The relation of the limited or classic forms of Wegener granulomatosis to antigen-antibody reactions in general has not been established however. Adrenal cortical steroid treatment may be of benefit in some of these patients, although others seem to do equally well without such therapy. In still others, such therapy is ineffective in altering the progressive course of the illness. © 1966.