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.