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and glomerulonephritis. Limited forms of WG also occur, often with involvement of the respiratory tract, but without kidney involvement. The vast majority of patients have antineutrophil cytoplasmic antibodies (ANCA) in the serum with a characteristic cytoplasmic pattern (C-ANCA) [172].

The upper respiratory tract is the commonest presenting site of WG, mainly affecting the paranasal sinuses, followed by the nose, nasopharynx and larynx [82]. Local clinical symptoms and signs, such as rhi-norrhoea, pain, mucosal ulcerations and hoarseness, are non-specific. They may be accompanied by systemic symptoms and signs, such as fever, weakness and weight loss [339].

Histological features include inflammation, necro-tising granulomas, and vasculitis. Necrosis in WG has a patchy distribution, with serpiginous borders, and is usually basophilic, with a finely granular appearance. Granulomas tend to be loose, not closely packed as in sarcoidosis or tuberculosis [82]. Vasculitis typically involves small to medium-sized arteries and veins, with any of the following features: fibrinoid necrosis, fragmentation of the elastic lamina, acute and chronic inflammatory cells and granulomas. The lesions may undergo organisation and fibrosis.

The diagnosis of WG is based on clinical features, biopsy of the related lesions, and the cytoplasmic pattern of anti-neutrophil cytoplasmic antibodies (C-ANCA) in the serum [102]. A positive biopsy of the upper respiratory tract has a high predictive value, up to 100%, indicating few or even no false-positive results [173]. However, vasculitis is only rarely seen on biopsy. Histology often reveals non-specific features - inflammation and necrosis, with or without granuloma formation [397].

Wegener's granulomatosis should be differentiated from other forms of vasculitis, other granulomatous diseases, cocaine abuse, and from neoplasms, particularly NK/T lymphoma of the nasal type [339]. The presence of C-ANCA proves extremely helpful in differentiation from almost all the diseases mentioned [173].

Wegener's granulomatosis was almost universally fatal in the past, usually within a few months of the onset of clinically apparent renal disease. However, with modern immunosuppressive therapy, the prognosis of WG is excellent. A marked improvement is seen in 90% of patients and complete remission is achieved in 75% of patients [160]. Early detection of WG is essential to prevent fully developed disease. For WG at other sites in the head and neck, see also Chaps. 2 and 3.

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