Classical Wegener granulomatosis is a disease of unknown cause, characterized by necrotizing granulomatous involvement of the upper and lower parts of the respiratory tract, focal necrotizing glomerulitis, and necrotizing vasculitis involving both arteries and veins. The disease may involve multiple organ systems. First described in 19311 and 19362 and considered a rare disease, it is now recognized with increasing frequency. In 1954, Godman and Churg3 could find only 29 cases in the world literature. By 1967, there were at least 200 published cases, and several large series have been reported recently.4-6
In 1966, Carrington and Liebow7 described a limited form of Wegener granulomatosis that appeared to have a better prognosis. They described 16 patients with characteristic pulmonary lesions but without the focal glomerulitis. Drachman,8 in 1963, reported that the frequency of neurological involvement in Wegener granulomatosis approximated 50% (Table). However, in his series of
Sahn EE, Sahn SA. Wegener Granulomatosis With Aphasia. Arch Intern Med. 1976;136(1):87–89. doi:10.1001/archinte.1976.03630010069013
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