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This condition was described by Wegener as 'a variant of polyarteritis nodosa
in which nasal or paranasal granulomata were prominent features'. It is
a rare form of necrotising vasculitis characterised by: - pulmonary
disease
- upper and lower respiratory tract lesions - affecting nose, sinuses,
and lung
- focal or diffuse glomerulonephritis
Other summary
features include: - disease may run a course that might vary from indolence
to one of rapid progression leading to life-threatening multiorgan failure
-
disease generally develops over a period of time with the mean period from onset
of symptoms to diagnosis ranging from 4.7 to 15 months
- without treatment
it is invariably fatal
Reference: - Ponniah
I et al. Wegener's granulomatosis: The current understanding. Oral Surg Oral Med
Oral Pathol Oral Radiol Endod. 2005 Sep;100(3):265-70.
- Savage COS.
Systemic vasculitides.Medicine International 1994; 22(2):53-7.
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