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.

Wegener's granulomatosis: a review of clinical features and an update in diagnosis and treatment

“…Involvement of the major salivary glands is uncommon and generally occurs in association with other features of upper and lower airway disease . Salivary gland disease of WG may manifest as unilateral or bilateral enlargement of one or more of the major glands, night sweats and arthralgia.…”

“…Magnetic resonance imaging or ultrasonographic examination of the salivary gland can demonstrate diffuse heterogeneous enlargement of the gland with small areas of central necrosis . The histopathological features of WG of the salivary glands comprise sialadenitis with an infiltrate of leucocytes, macrophages and multinucleated giant cells, with no definitive necrosis .…”

Untypical Manifestations of Granulomatosis with Polyangiitis—A Review of the Literature

“…Vessels of the upper or lower airways or whole respiratory tract and kidneys are involved with a prevalence of 90% [3] and 78% [4], respectively. The biopsy findings include neutrophilic vasculitis with necrosis and multinucleated giant cells and mononuclear infiltrate in walls of vessels affected with fibrinoid necrosis [5,6].…”

Biomarkers in Autoimmune Salivary Gland Disorders: A Review

“…ENT symptoms can occur both in GPA (with an estimated frequency of 90%) and in MPA (35%), involving nasal crusting, sinusitis, otitis media, otorrhea, persistent rhinorrhea, purulent or bloody nasal discharge, oral or nasal ulcers, and polychondritis [36][37][38] . Additionally, the parotid glands can be affected, typically during the early phase of the disease, presenting as massive enlargement with signs of infection or neoplasia [39] . A nonsevere, limited form of AAV occurs in approximately 25% of cases and usually involves no organ-threatening manifestations but usually chronic and recurring disease of the upper respiratory tract [39] .…”

“…Additionally, the parotid glands can be affected, typically during the early phase of the disease, presenting as massive enlargement with signs of infection or neoplasia [39] . A nonsevere, limited form of AAV occurs in approximately 25% of cases and usually involves no organ-threatening manifestations but usually chronic and recurring disease of the upper respiratory tract [39] .…”