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B. J. Winn, J. Rootman; Sclerosing Orbital Inflammation and Systemic Disease. Invest. Ophthalmol. Vis. Sci. 2010;51(13):3530.
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To determine the clinical differences between sclerosing orbital inflammation (SOI) isolated to the orbit and SOI manifesting as multifocal fibrosclerosis (MFF) when associated with retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, and/or Riedel’s thyroiditis. While large studies of isolated SOI have found that it is essentially a unilateral disease with no sex predilection, most often affecting people in their 30s to 50s, no large studies of MFF-related SOI have been performed due to its rarity.
SOI in the setting of MFF is often bilateral and associated with an elevated ESR. As orbital disease may precede or coincide with extra-orbital involvement, investigations including chest and abdominal imaging, liver and kidney function tests, ESR and thyroid stimulating hormone should be obtained in cases of SOI to identify potentially life-threatening systemic involvement.
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