Background: The clinical picture of IgG4-related sclerosing disease (IgG4-RSD) may mimic lymphoma, and should be in the differential diagnosis of patients with this clinical picture.
Case summary: A 32-year-old female had recurrent swelling of both eyelids for more than 15 years. Examination revealed elastic, firm, swollen lacrimal glands about 2-3 cm in diameter that was not painful. Head and orbits magnetic resonance imaging (MRI) showed mass lesions over the bilateral lacrimal glands, submandibular glands, and left foramen of ovale. The differential diagnosis included lymphoid tissue, inflammatory masses, and lymphoma. Gallium single-photon emission computed tomography/computed tomography (SPECT/CT) showed uptake in the bilateral lacrimal glands, right parotid and bilateral submandibular glands, bilateral perirenal region, mediastinal, prevertebral, paraaortic, lumbar, bilateral pelvic (including internal iliac chain) lymph nodes, anterior aspect of right 3rd rib, and lateral aspect of left 6th rib. CT showed multiple enlarged lymph nodes in the mediastinum, right pulmonary hilum, prevertebral space of the thoracolumbar spine, retroperitoneal paraaortic area, bilateral parailiac areas, and bilateral perirenal spaces. Antinuclear and anti-SSA/SSB antibodies were negative, and the serum IgG4 level was 740 mg/dL (normal, 8-140 mg/dL). Right parotid gland biopsy showed abundant IgG4-positive plasma cells. Mikulicz disease (IgG4-related sclerosing disease) was diagnosed and she received glucocorticoid treatment. Follow-up CT and MRI showed with resolved eyelid swelling and perirenal mass lesions. Follow-up gallium scan was normal.
Conclusion: Gallium SPECT/CT can be a useful tool for initial and follow-up evaluation of IgG4-RSD.