Clinical images: Lacrimal gland and renal involvement in IgG4-related sclerosing disease



Illustration 1.

The patient, a 32-year-old woman, presented to our hospital with a 10-year history of recurrent painless swelling of both eyelids. Ophthalmologic examination showed a 3 × 2–cm fusiform, nontender, firm swelling in the lacrimal gland (A and B). Magnetic resonance imaging of the head and brain revealed enlargement of the lacrimal, parotid, and submandibular glands bilaterally. Computed tomography of the abdomen showed multiple enlarged lymph nodes and mass infiltration over the perirenal spaces bilaterally (D). No proteinuria or renal insufficiency was found. Test results for antinuclear and anti-SSA/SSB antibodies were negative; the serum IgG4 level was 740 mg/dl (normal 8–140). Biopsy of the right parotid gland revealed abundant IgG4-positive plasma cells (C). The patient was diagnosed as having IgG4-related sclerosing disease. She received treatment with glucocorticoids, and the eyelid swelling and renal mass infiltration resolved. The wide spectrum of clinical manifestations in IgG4-related sclerosing disease should be carefully considered in order to avoid unnecessary laparotomy.