Wegener's granulomatosis presenting as major salivary gland enlargement.

Salivary gland involvement is rare in Wegener's granulomatosis. We report the first case of widespread major salivary gland enlargement as part of the presentation of this disease. A review of the few reported cases in the literature suggests that salivary gland involvement may be associated with a limited form of the disease and an improved prognosis. The anti-neutrophil cytoplasmic antibody (c-ANCA) assay remains the gold standard of diagnosis but care should be exercised in the interpretation of results. This patient responded well to current immunosuppressive therapy.