Wegener's Granulomatosis

Case 9-1999: Wegener's Granulomatosis

“…The number of cases collected from the literature by Fahey et al (1954) and (1953) or in the first case of , and the renal lesions described by Klinger (1931) are not characteristic.…”

“…the lesions in the nasal septum, lungs, spleen, and kidneys. Wegener (1937Wegener ( , 1939 Fahey, Leonard, Churg, and Godman (1954) and by . In two excellent papers they review the literature, describe seven new cases and clarify the diagnostic criteria of this disease.…”

Clinical Aspects of Systemic Vasculitis in Adults

“…. Before the development of steroids and Immunosuppressive drugs the mortality of most systemic vasculitides are high: 80% one-year mort~lity in Wegener's granulomatosis 26 and 85% five-year mortality in polyarteritis nodosa. 27 ,28 The prognosis of systemic vasculitis has improved dramatically over the last 20 years, especially with the use of potent immunosuppressive drugs.…”

Vasculitis and granulomatosis of the respiratory tract.

“…[8][9][10][11][12][13][14][15][16][17][18][19] It is characterised by a diagnostic triad consisting of granulomatous vasculitis of the upper and lower respiratory tract, a generalised small vessel vasculitis, and focal glomerulonephritis. 9 The sexes are affected equally, with a peak incidence in the fifth decade.…”

“…Untreated patients die in about five months, usually of renal failure. [8][9][10] The initial lesions almost always occur in the respiratory tract.16 20 Upper respiratory tract manifestations start as a persistent "cold", with rhinorrhoea, epistaxis, and sinusitis.19 Later there is obstruction and ulceration of the nose and nasopharynx, perforation of the nasal septum, saddle nose deformity, and eventually horrific destruction of mid-facial structures.…”