Idiopathic orbital inflammation: a report of 18 cases

Purpose Idiopathic orbital inflammation is a rare clinical entity which has protean clinical manifestations. It’s a diagnosis of exclusion which imposes a biopsy, and which can be made only after a screening to rule out a systemic etiology of inflammation. Methods Retrospective series of 18 histologically-proven orbital inflammation cases seen in our service between 2006 and 2011. Results The study encompassed 7 men and 11 women, with a mean age of 47 years old (4-83). Patients complained of a pain (44%), a diplopia (33%), a decreased visual acuity (17%). A swollen eyelid or a palpable mass were presents in 78% of the cases. Less often, we noted proptosis, or a diminished ocular motility. The radiologic analysis (CT-scan, NMR, doppler ultrasound examination) found an inflammation localized to the orbital fat, to the lacrymal gland, and to one or several oculomotor muscle(s) in respectively 89%, 67%, and 39% of the cases. Excisional biopsy was curative for 33% of the cases. 61% of the patients received a corticotherapy, with a relapse or a recurrence in 60% of the cases, making necessary to have recourse to immunosuppressive agents (methotrexate), with a good control rate on the inflammation (75%). Finally, 76% of the patients obtained a complete resolution of their symptoms at the end of the follow-up (mean : 20 months). Conclusion Treatment lies on surgical exeresis if it is safely. Corticotherapy is frequently not sufficient. Methotrexate seems to be an interesting alternative. Recent works concentrate on Systemic IgG4 Disease, who could be a frequent cause of orbital inflammation, calling the term idiopathic into question, and letting us think about targeting treatments.