Wegeners Granulomatosis

Fig. 3.7. Wegener's granulomatosis of gingiva showing intense inflammation, haemorrhage and scattered, small multinucleated giant cells

Wegener's granulomatosis (WG) is an uncommon but distinctive form of vasculitis characterised in its classical form by necrotising granulomatous inflammation of the upper and lower respiratory tracts and segmental necrotising glomerulonephritis [76]. It is now recognised that a wide variety of other organs and tissues may be involved. Rarely, a proliferative rather than a destructive response produces tumefactions [65]. Variants of WG include a limited form, which has few extra-pulmonary manifestations, and a protracted superficial form, which is characterised by lesions restricted to the upper respiratory tract, mucosa and skin for a prolonged period, although it may eventually progress to renal involvement [58]. Wegener's granulomatosis limited to the respiratory system may respond to antibiotics such as co-trimoxazole and Staphylococcus aureus has been implicated as a triggering agent for this disease, but the evidence remains equivocal [141].

Head and neck manifestations, particularly in the si-nonasal complex, are common and can affect as many as 90% of patients at presentation [50] (see Chap. 2). They include severe rhinorrhoea, sinusitis, otitis media and destruction of the nasal septum and cartilage to produce a saddle-nose deformity. By contrast, oral lesions are less common and affect only about 5% of patients [69]. They include oral ulceration, delayed healing of extraction wounds, tooth mobility and loss of teeth. Perforation of the palate is usually as a direct extension of si-nonasal disease. Extraorally, head and neck manifestations include swelling and desquamation of the lips, parotid gland enlargement, and cranial nerve palsies.

Fig. 3.7. Wegener's granulomatosis of gingiva showing intense inflammation, haemorrhage and scattered, small multinucleated giant cells

A rare, but particularly characteristic oral feature is so-called strawberry gums, which is considered to be virtually pathognomonic of Wegener's granulomatosis [111, 124]. There is a localised or generalised prolifera-tive gingivitis with a mottled, purplish-red granular surface, which resembles an over-ripe strawberry. Disease localised to the gingiva tends to be fairly low-grade. Involvement of the underlying bone, however, may cause the related teeth to loosen or exfoliate.

Microscopy of the gingiva shows irregular epithelial hyperplasia with downgrowths of the rete processes into the underlying corium. The connective tissue shows vascular lakes of extravasated blood and haemosiderin-con-taining macrophages and there are neutrophil and eo-sinophil microabscesses, together with a more diffuse mixed inflammatory infiltration (Fig. 3.7). Small multi-nucleated giant cells are unevenly distributed in the lesion, and although considered to be characteristic, may be absent in many levels. Vasculitis is not usually seen, possibly because vessels of sufficient size to show this feature are rarely present in gingivectomy specimens. By contrast, biopsies of other oral lesions rarely show micro-abscesses or necrosis. Also, the granulomatous reaction characteristic of many other sites is uncommon [42].

Investigations of patients with suspected oral WG should include sinus and chest radiographs, full blood picture, erythrocyte sedimentation rate, C-reactive protein, autoantibody profile (including rheumatoid factor) and renal function tests. An important investigation is the titre of antineutrophil cytoplasmic antibody (ANCA), particularly cytoplasmic or cANCA. cANCA is a useful marker of WG and is found in up to 100% of patients with widespread, active disease [27], but only 6070% of patients with limited forms of the disease [63]. Although cANCA has a very high specificity for WG, it is rarely found in other types of vasculitis [27]. Other ANCA-associated vasculitides include microscopic polyangiitis and Churg-Strauss syndrome [38], but these do not affect the mouth. The titre of cANCA may be related to the severity of the disease and therefore can be a useful index of prognosis and efficacy of treatment. However, in patients with limited or protracted superficial forms of the disease, the ANCA may be negative for months or even years so that other clinicopathologi-cal criteria should not be ignored when making the diagnosis.

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