Sclerosing orbital inflammation and systemic disease.

PURPOSE: To determine the clinical differences between sclerosing orbital inflammation (SOI) isolated to the orbit and SOI manifesting as part of systemic inflammatory disease.

METHODS

For the case series, the authors identified patients with SOI from their patient database and tabulated their clinical data. For the literature review, a search for case reports of SOI in the English literature was performed. The clinical data of each were tabulated and analyzed.

RESULTS

For the case series, 13 cases of SOI were identified, 2 with multifocal fibrosclerosis (MFF) and 4 with other systemic inflammatory diseases. Of the 5 bilateral cases, 4 (80%) had some form of systemic inflammatory disease including 2 with MFF. Only 2 (25%) of the 8 unilateral cases had systemic inflammatory disease. The average ESR was 7 mm/hour for those without systemic disease and 41 mm/hour with evidence of systemic disease (p = 0.038). For the literature review, the authors identified 68 case reports of SOI not previously reported in large case series, 34 associated with MFF, 5 associated with other systemic disease, and 29 not associated with any systemic disease. Sixty-nine percent of cases with systemic disease had bilateral orbital involvement, whereas only 3.6% of those without systemic disease were bilateral (p = 1.15 × 10(-8)). Twenty of the cases with MFF noted ESR (83 mm/hour, mean), 95% of which were above normal. ESR was noted in only one non-MFF, nonsystemic disease case and was normal.

CONCLUSIONS

SOI in the setting of MFF or other systemic inflammatory disease is often bilateral and associated with an elevated ESR.