Idiopathic Sclerosing Orbital Inflammation

Abstract

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. Thir-teen 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 secondline agents can result in control and even regression of the disease.

Idiopathic sclerosing orbital inflammation mimicking a malignant spindle cell tumor in a dog

“…ISOI patients exhibit fewer inflammatory signs, a more chronic onset, and more aggressive disease course than other IOI types, reminiscent of that seen in the dog 30. Histopathologically, ISOI comprises a dense fibrosis with a paucicellular infiltrate of lymphocytes, histiocytes, eosinophils, and plasma cells 30, 31. This damages orbital structures via cicatricial entrapment with mass effect and is associated with a poor response to conventional therapy (corticosteroids ± radiation) 11, 17, 25, 30, 31.…”

“…ISOI patients exhibit fewer inflammatory signs, a more chronic onset, and more aggressive disease course than other IOI types, reminiscent of that seen in the dog 30. Histopathologically, ISOI comprises a dense fibrosis with a paucicellular infiltrate of lymphocytes, histiocytes, eosinophils, and plasma cells 30, 31.…”

“…Idiopathic sclerosing orbital inflammation accounts for 6–8% of human inflammatory orbital lesions and is otherwise described as “sclerosing orbital pseudotumor” 11, 16, 30. ISOI patients exhibit fewer inflammatory signs, a more chronic onset, and more aggressive disease course than other IOI types, reminiscent of that seen in the dog 30.…”

“…Histopathologically, ISOI comprises a dense fibrosis with a paucicellular infiltrate of lymphocytes, histiocytes, eosinophils, and plasma cells 30, 31. This damages orbital structures via cicatricial entrapment with mass effect and is associated with a poor response to conventional therapy (corticosteroids ± radiation) 11, 17, 25, 30, 31. While several of these features may be seen with aggressive orbital neoplasms, we are not aware of any neoplastic disorders that would present independently (and almost identically) in both orbits.…”

Inflamação esclerosante idiopática da órbita: estudo clinico-patológico

“…I nflamação esclerosante idiopática da órbita é considerada subgrupo da inflamação idiopática da órbita, caracterizada por processo inflamatório insidioso, causando lesões de estruturas orbitárias através da formação exuberante de fibrose e colagenização (1,2) . Ocorre raramente, sendo a maior série de casos publicada representada por trinta e um pacientes, em estudo que envolveu cinco centros de referência em doenças da órbita 2 seguida pela série de dezesseis casos estudada por Rootman et al (3) .…”