Orbital Pseudotumor: Uncommon Initial Presentation of IgG4-Related Disease

الملخص EN

IgG4-related disease (IgG4-RD) encompasses a group of fibroinflammatory conditions recognized in recent times.

The main clinical features include variable degrees of tissue fibrosis, tumorlike expansions, perivascular lymphocytic infiltration rich in IgG4-positive plasma cells, and elevated serum IgG4.

A case has been reported of an elderly patient with an unexplained unilateral exophthalmia; biopsy was performed and revealed lymphocytic infiltration, suggesting IgG4-RD.

High serum levels of IgG4, in association with a good response to steroid therapy and to the exclusion of other diagnoses, confirmed the hypothesis of orbital pseudotumor by IgG4-RD.