Orbital inflammation: a rare association of common variable immunodeficiency

Ocular associations with common variable immunodeficiency (CVID) are very rare which include granulomatous uveitis, mulitfocal choroiditis and retinal vasculitis. However there have been no reports of orbital involvement, either inflammatory or neoplastic in association with CVID. We describe a unique case of a patient with CVID who developed orbital inflammation.

Clinical, histological and radiological findings and the management of this case is presented.

A 58 year old male with known CVID treated with regular intravenous immunoglobulin infusion, presented with left peri-orbital redness, pain, proptosis and restriction of ocular movements. CT scan showed an ill-defined mass in the left lateral orbit, adjacent to the lacrimal gland. An orbital biopsy revealed a monomorphic lymphocytic infiltrate forming loose lymphoid aggregates in the lacrimal gland tissue. Immunocytochemistry confirmed this to be a reactive inflammatory infiltrate. The patient was successfully treated with a course of oral prednisolone which resulted in improvement of orbital inflammation, ocular movements and proptosis.

To our knowledge this is the first reported case of non-granulomatous orbital inflammation in association with CVID. This case serves to remind ophthalmologists encountering a patient with CVID that they may have associated orbital inflammatory disease which is responsive to steroid therapy similar to sterile inflammatory masses described in other organs.