Clinical Images: Dacryoadenitis in eosinophilic granulomatosis with polyangiitis mimicking immunoglobulin G4-related disease.

Abstract

The patient, a 26-year-old woman, presented with a 2-week history of acute swelling in both lacrimal glands ( A ). She had recently developed uncontrolled asthma and nasal polyps. The laboratory test results were notable for an absolute eosinophil count of 5300 cells per μ l (reference range 100-400 μ l), exceeding 50% of circulating leukocytes. The antineutrophil cytoplasmic antibody test result was negative. Magnetic resonance imaging of the head con fi rmed swelling of the bilateral lacrimal glands ( B , yellow arrowheads) and rhinosi-nusitis. A biopsy of her lacrimal gland revealed eosinophil-rich necrotizing granulomatous in fl ammation with vasculitis ( C ). Her serum immunoglobulin G4 (IgG4) level was slightly elevated (265 mg/dl, normal upper limit is 121 mg/dl), but IgG4 immunostaining of the biopsy specimen did not show a signi fi cant fi nding. A diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) was made. Treatment with 30 mg of prednisone was initiated, which was gradually tapered without glucocorticoid-sparing agents over 12 months. The swelling of her lacrimal glands resolved, and she was free of symptoms, including asthma and lacrimal gland swelling, at the 12-month follow-up. In summary, EGPA can present with lacrimal gland swelling and mimic IgG4-related Mikulicz disease (1,2). A histopathological examination of the swollen lacrimal gland is the key to differentiating EGPA from IgG4-related Mikulicz disease in such cases. Author disclosures