A review of sclerosing idiopathic orbital inflammation.

PURPOSE OF REVIEW: Sclerosing idiopathic orbital inflammation is a rare, distinct subset of orbital inflammation. No consistent therapeutic regimen has been described and the majority of reports on this disease are case reports or small case series. This review looks at historical and current treatment modalities in an attempt to define the most current, effective management strategy for this disease.

RECENT FINDINGS: Recently, the largest review to date of these patients was published, detailing clinical findings and responses to the various interventions. Two other case reports of patients who responded completely to therapy appeared in the literature.

SUMMARY: Sclerosing idiopathic orbital inflammation is a rare disease with an unknown pathogenesis and poor prognosis. Current treatments are disappointing and often have little beneficial effect. There are anecdotal reports of success using combinations of immunosuppression, radiotherapy, and surgery, but a consistent, effective treatment course is lacking. Younger patients and those with a shorter duration of disease seem to have the best prognosis. Further research into the immunologic basis of the disease and a large, controlled study comparing the various sclerosing idiopathic orbital inflammation treatments are needed to determine the most effective treatment regimen.