Wegener's granulomatosis presenting as unilateral parotid enlargement

Major salivary gland enlargement is a rare presenting symptom of Wegener's granulomatosis. The unrecognized occurrence of this entity can delay diagnosis, leading to increased morbidity from disease progression. This report discusses the clinical features and diagnostic testing of salivary gland enlargement secondary to Wegener's granulomatosis to differentiate it from other, more common, salivary gland diseases.

A case report of a single subject with unilateral parotid gland enlargement secondary to Wegener's granulomatosis.

A review of the clinical course, diagnostic studies, and histopathology related to the presenting disease.

A 54-year-old male was evaluated for a 6-week history of progressive right parotid enlargement and pain unresponsive to antimicrobial therapy. Computed tomography scans showed diffuse, unilateral parotid swelling without enhancement and without a mass lesion or sialolith. Multiple open biopsies from the gland were necessary to demonstrate the presence of necrotizing granulomatous inflammation with vasculitis. Elevated antineutrophil cytoplasmic autoantibody, cytoplasmic pattern (c-ANCA) titers confirmed Wegener's granulomatosis. Hemoptysis and acute renal failure requiring hemodialysis developed shortly after diagnosis but eventually resolved after the initiation of corticosteroids and cyclophosphamide.

Unilateral parotid enlargement is a rare presentation of Wegener's granulomatosis. A high level of clinical suspicion should prompt biopsy and testing of c-ANCA when initial studies or empirical treatment fails to lead to a proper diagnosis. Early treatment may prevent the development of other serious systemic complications such as renal failure.