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Type: Journal article
Title: Idiopathic sclerosing inflammation of the orbit: A case of steroid-responsive disease in a patient with autoimmune hemolytic anemia
Author: Billing, K.
Hsuan, J.
Selva-Nayagam, D.
Citation: European Journal of Ophthalmology, 2005; 15(2):263-266
Publisher: Wichtig Editore
Issue Date: 2005
ISSN: 1120-6721
Statement of
K.J. Billing, J. Hsuan and D. Selva
Abstract: Purpose: Idiopathic sclerosing orbital inflammation (ISOI) has been categorized by some authors as a unique clinicopathologic entity, separate from the heterogenous group of disorders known collectively as nonspecific orbital inflammation. Histologic similarity and clinical association with other fibrosclerosing conditions has been shown. The authors present a case of ISOI in conjunction with autoimmune hemolytic anemia. Methods: A 59-year-old woman with a history of hemolytic anemia had left upper lid swelling, periocular pain, proptosis, and restriction of ocular motility. Magnetic resonance imaging (MRI) showed a homogenously enhancing lateral orbital mass. Biopsy revealed dense fibrous connective tissue with a paucicellular infiltrate, consistent with ISOI, and treatment with prednisolone 60 mg/day was instituted. Results: At 4-week review, the proptosis had settled and the patient regained full range of extraocular movements. At 14 months, the response was sustained and repeat MRI showed a 70% reduction in size of the mass. Conclusions. The first known case of ISOI and hemolytic anemia is presented. Despite dense fibrosis histologically, steroid responsiveness can be encountered in cases of ISOI. An early, aggressive approach to management is recommended, and corticosteroids should be considered as a treatment option.
Keywords: Orbit; Humans; Exophthalmos; Anemia, Hemolytic, Autoimmune; Sclerosis; Prednisolone; Glucocorticoids; Magnetic Resonance Imaging; Middle Aged; Female; Orbital Pseudotumor
RMID: 0020092106
DOI: 10.1177/112067210501500214
Appears in Collections:Surgery publications

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