Orbital inflammation with IgG4-positive plasma cells: Manifestation of IgG4 systemic disease

Objective: To describe clinical, radiographic, and morphologic findings in patients with IgG4-positive cells present on orbital biopsy specimens. Design: Retrospective review (from January 1, 1993, through December 31, 2006) of patients with orbital biopsy specimens that excluded lymphoma; comparison of patients with and without IgG4-positive cells on immunostaining. Results: Of 21 patients, 11 had increased IgG4-positive cells (defined as >10 cells on biopsy). Symptoms included eyelid or periocular swelling (8 patients) or proptosis (3 patients), with bilateral involvement in 6 patients. Computed tomographic imaging displayed lacrimal gland mass in 10 patients; 6 patients had lesions in other organs. Two patients had increased serum IgG4 levels. In 10 patients without IgG4-positive cells (≤10 cells on biopsy), 6 had proptosis, 1 had eyelid swelling, 2 had eyelid mass, and 1 had diplopia, all unilateral. None had systemic symptoms. Patients with IgG4-positive cells had longer symptom duration, and their biopsy specimens showed more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils. Conclusions: The clinical appearance, high incidence of bilateral disease, association with lesions in other organs, and increased IgG4 serum levels in some patients-with an increased number of IgG4-positive cells in the biopsy specimen, which shows more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils-indicate that these patients have an orbital manifestation of IgG4-associated systemic disease.