IgG4-Positive orbitopathy

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To give an overview of IgG4-positive orbitopathy. A newly described disease of the orbit.


Critical review of the present literature demonstrated by a patient case.


The clinical appearance, high incidence of bilateral disease, association with lesions in other organs, and increased IgG 4 serum levels along with the histological findings of IgG4–positive cells, more background fibrosis, lymphoid hyperplasia, plasma cells and eosinophils are the characteristics of this disease.


Patients with IgG4–positive cells in an orbital biopsy may have an orbital manifestation of IgG4-associated systemic disease

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