Idiopathic sclerosing orbital inflammation.
OBJECTIVE
To perform a multicenter review of the clinical features and treatment of 31 patients with idiopathic sclerosing orbital inflammation.


METHODS
We included all patients with histologically confirmed idiopathic sclerosing orbital inflammation from 5 regional orbital centers. We reviewed the case notes to determine the clinical presentation, diagnostic features, and response to treatment. The main outcome measures were duration and nature of symptoms, anatomical location of disease, histopathological findings, treatment modalities, treatment efficacy and adverse effects, and final clinical status.


RESULTS
We included 13 male and 18 female patients ranging in age from 7 to 83 years. The average duration of symptoms at presentation was 13.4 months. There was a predilection for the lateral and superior quadrants. Thirteen patients had apical disease, and 4 had extraorbital involvement. Histopathological findings invariably showed sclerosis associated with a sparse mixed cellular infiltrate. Twenty-seven patients were treated with oral prednisolone, response to which was good in 9 patients, partial in 11, and poor in 7. Six patients were treated with a second-line immunosuppressive agent, and 6 received radiotherapy. The response to radiotherapy was generally poor.


CONCLUSIONS
Idiopathic sclerosing orbital inflammation is a rare condition that can be difficult to diagnose and manage. Early intervention with immunosuppression in the form of corticosteroids combined with second-line agents can result in control and even regression of the disease.
Hsuan
James D
JD
Department of Ophthalmology, Walton Hospital, Rice Lane, Liverpool L9 1AE, England.
Selva
Dinesh
D
McNab
Alan A
AA
Sullivan
Timothy J
TJ
Saeed
Peerooz
P
O'Donnell
Brett A
BA
D000284:Administration, Oral; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D003941:Diagnostic Techniques, Ophthalmological; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009915:Orbit / Q000187:drug effects / Q000473:pathology* / Q000528:radiation effects; D016727:Orbital Pseudotumor / Q000175:diagnosis* / Q000188:drug therapy / Q000532:radiotherapy; D011239:Prednisolone / Q000627:therapeutic use; D012598:Sclerosis; D014057:Tomography, X-Ray Computed
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D011239:Prednisolone