Wegener's granulomatosis. Review of the literature and description of a case
Frezza, M.; Rinaldi, C.; Pozzato, G.; Terpin, M.M.; Marcuzzi, P.; Melato, M.
Giornale di Clinica Medica 68(11): 575-579
1987
ISSN/ISBN: 0017-0275 PMID: 3329132 Document Number: 299657
Wegener's granulomatosis is a relatively rare disease, identified pathologically by necrotizing granulomatous lesions in the respiratory tract, generalized vasculitis and focal glomerulonephritis. Other organs and systems involved are: joints, eyes, skin, nervous system and heart. No laboratory findings are pathognomonic for the disease. The mean survival of untreated disease was five months. Actually, cyclophosphamide (alone or associated with prednisone) is clearly the drug of choice. This treatment has allowed a high rate of long-term remissions, without life-threatening complications. A case of Wegener's granulomatosis with primitive ocular and pulmonar involvement is reported. During the course of the disease, other pathognomonic symptoms appeared as: skin nodules, otitis media, suppurative rhinorrhea and renal involvement. A complete remission of the form has been induced, after four months, with the use of cyclophosphamide plus prednisone therapy.