Wegeners Granulomatosis

Wegener's granulomatosis is an immunologically mediated inflammatory disease characterised by granulomatous vasculitis of the upper and lower respiratory tracts together with glomerulonephritis. Variable degrees of disseminated vasculitis involving both small arteries and veins may also occur. The lesions in the upper respiratory tract are ulcerative and destructive and occur mainly in the nasal cavity and paranasal sinuses. The hallmarks of Wegener's granulomatosis are the presence of geographic necrosis surrounded by palisaded histiocytes, granulomas and scattered giant cells, vasculitis with fibrinoid necrosis or infiltration of vessel walls by inflammatory cells, neutrophilic microabscesses and a mixed inflammatory infiltrate with variable fibrosis [57, 165]. Stains for acid fast bacilli and fungi are negative. There is no cytological atypia. The classic histological features of Wegener granulomatosis are not present in many biopsy specimens. Repeat biopsies and clinical correlations are often essential for early diagnosis. The disease may be restricted to the upper respiratory tract in the early stages. A high percentage of patients develop c-ANCA. More details are to be found in Chap. 3.

Lepromatous leprosy is the most frequent form of this type of disease involving the nasal cavity [101]. It is characterised by nodular masses of foamy macrophages (lepra cells) in which large numbers of acid fast bacilli (Mycobacterium leprae) are demonstrable by the modified Ziehl-Neelsen method. Tuberculoid leprosy is characterised by non-caseating granulomas and the indeterminate variant by a non-specific chronic inflammatory reaction; acid fast bacilli are seldom demonstrable in these types.

Tuberculosis of head and neck occurs infrequently and involvement of the nose is rare, representing in most cases a secondary event to pulmonary involvement [231]. In most cases there is a polyp of the nasal septum or an ulcerated granular lesion. Presence of intracranial extension may lead to a clinical diagnosis of malignancy [19]. Microscopically, there are caseating giant cell granulomas in which acid-fast bacilli may occasionally be identified. The definitive diagnosis is made by iso-

lating Mycobacterium tuberculosis from tissue removed during biopsy.

2.8.4 Sarcoidosis

Sarcoidosis is a chronic multisystem granulomatous disorder that has a predilection for pulmonary and upper respiratory tract mucosa. The sinonasal mucosa is rarely involved, and most patients have generalised disease [143]. Discrete non-caseating granulomata composed predominantly of epithelioid histiocytes with multinucleated giant cells and a peripheral rim of lymphocytes are present in the mucosa. Stains for acid-fast bacilli are negative. The differential diagnosis includes other granulomatous disorders, like tuberculosis, leprosy, Wegener's granulomatosis and cholesterol granuloma [57].

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