Wegener’s granulomatosis of the major salivary glands

Abstract

Wegener's granulomatosis is one of the anti-cytoplasmic autoantibodies (ANCA)-associated vasculitides. Although it typically affects the lungs and kidneys, the head and neck are also involved in most cases but the site usually affected is the upper airway. However, there are 35 cases with well-documented clinicopathological data in which Wegener's granulomatosis manifested in the major salivary glands, most commonly the parotid. Twenty-four patients presented with salivary signs and symptoms, in eight of whom there was no other presenting manifestation. These signs and symptoms may mimic infection or neoplasia and laboratory investigations, including ANCA serology and histopathology, may be non-specific; thus, in 21 of the 35 patients (60%) there was a delay in diagnosis. Amongst the 21 were 11 of the 14 (78.6%) patients who presented with unilateral parotid disease and three of the five who died. Three other patients suffered permanent pulmonary, two renal and five facial nerve damage. This article reviews the literature on major salivary gland involvement in Wegener's granulomatosis, which should be considered in the differential diagnosis of major salivary gland disease particularly if commoner causes have been excluded. A detailed medical history, and persistently inconclusive laboratory tests, could provide the clues that enable prompt diagnosis.