Idiopathic Sclerosing Orbital Inflammation

Pemberton, John deJ.; Fay, Aaron

doi:10.1097/iop.0b013e318238ecf7

Cited by 49 publications

(20 citation statements)

References 26 publications

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“…Idiopathic orbital inflammation is usually unilateral, but bilateral disease, either simultaneously or sequentially, occurs with an incidence of 8% to 20% in nonmyositic IOI and 14% in myositic IOI. [10][11][12]14,22 For patients with bilateral IOI, the possibility of contributing systemic disease should be considered, which involves new evaluation (eg, biopsy) of the contralateral orbit if affected at a later date. 23 Age Between 10 and 75 Years Although patient age has no absolute limit with regards to IOI, younger (<10 years) and older (>75 years for nonmyositic and >60 years for myositic IOI) patients are more likely to be classified as having another disease.…”

Section: Unilateralitymentioning

confidence: 99%

Consensus on Diagnostic Criteria of Idiopathic Orbital Inflammation Using a Modified Delphi Approach

et al. 2017

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IMPORTANCE Current practice to diagnose idiopathic orbital inflammation (IOI) is inconsistent, leading to frequent misdiagnosis of other orbital entities, including cancer. By specifying criteria, diagnosis of orbital inflammation will be improved.OBJECTIVE To define a set of criteria specific for the diagnosis of IOI. DESIGN, SETTING, AND PARTICIPANTSA 3-round modified Delphi process with an expert panel was conducted from June 8, 2015, to January 25, 2016. Fifty-three orbital scientist experts, identified through membership in the Orbital Society, were invited to participate in on online survey and they scored, using 5-point Likert scales, items that are eligible as diagnostic criteria from the literature and from personal experience. The items were clustered around the anatomic subtypes of IOI: idiopathic dacryoadenitis and idiopathic orbital fat inflammation (2 nonmyositic IOIs), and idiopathic orbital myositis (myositic IOI). Items with dissensus were rescored in the second round, and all items with consensus (median, Ն4; interquartile range, Յ1) were ranked by importance in the third round. MAIN OUTCOMES AND MEASURESConsensus on items to be included in the criteria. RESULTSOf the 53 experts invited to participate, a multinational panel of 35 (66%) individuals with a mean (SD) years of experience of 31 (11) years were included. Consensus was achieved on 7 of 14 clinical and radiologic items and 5 of 7 pathologic items related to diagnosis of nonmyositic IOI, and 11 of 14 clinical and radiologic items and 1 of 5 pathologic items for myositic IOI. There was agreement among panelists to focus on surgical tissue biopsy results in the diagnosis of nonmyositic IOI and on a trial with systemic corticosteroids in myositic IOI. Panelists agreed that a maximum number of 30 IgG4-positive plasma cells per high-power field in the orbital tissue is compatible with the diagnosis of IOI.CONCLUSIONS AND RELEVANCE An international panel of experts endorsed consensus diagnostic criteria of IOI. These criteria define a level of exclusion suggested for diagnosis and include tissue biopsy for lesions not confined to the extraocular muscles. This consensus is a step toward developing guidelines for the management of IOI, which needs to be followed by validation studies of the criteria.

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Section: Unilateralitymentioning

confidence: 99%

Consensus on Diagnostic Criteria of Idiopathic Orbital Inflammation Using a Modified Delphi Approach

et al. 2017

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“…Idiopathic sclerosing orbital inflammation (ISOI) is a rare disease [45, 46]. Previously thought to be the endstage of IOI, it is now considered a distinct pathologic entity with marked fibrosis present early in the disease [45–47].…”

Section: Characteristics Of Orbital Inflammation Due To Different Infmentioning

confidence: 99%

“…First-line treatment, although still controversial, encompasses corticosteroids and azathioprine, with poor results as fibrosis increases and inflammation subsides [45]. Some patients may show an elevated seric and tissue IgG4 [46]. It is difficult to know whether IgG4-related orbital disease is a distinct entity or an additional defining characteristic of idiopathic sclerosing orbital inflammation [46].…”

Section: Characteristics Of Orbital Inflammation Due To Different Infmentioning

confidence: 99%

“…Some patients may show an elevated seric and tissue IgG4 [46]. It is difficult to know whether IgG4-related orbital disease is a distinct entity or an additional defining characteristic of idiopathic sclerosing orbital inflammation [46]. Testing IgG4 is important as rituximab seems effective in IgG4-related disease.…”

Section: Characteristics Of Orbital Inflammation Due To Different Infmentioning

confidence: 99%

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CT and MR imaging of orbital inflammation

et al. 2018

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PurposeOrbital inflammation can be idiopathic or in the context of a specific disease and it can involve different anatomical orbital structures. On imaging, inflammatory disease is frequently mistaken for infection and malignant tumors, and its underlying cause is often not determined. Through this article we aim to improve orbital inflammation diagnosis and underlying inflammatory diseases recognition.MethodsThe imaging protocols and characteristics of orbital inflammation were reviewed.ResultsA decision tree for the evaluation of these patients is provided. First, a combination of clinical and radiological clues is used to recognize inflammation, in particular to differentiate it both from orbital infection and tumor. Subsequently, different radiological patterns are recognized, often allowing the differentiation of the several orbital inflammatory diseases.ConclusionThe use of adequate imaging protocols and subsequent evaluation allow the recognition of an orbital lesion as inflammatory and the diagnosis of the underlying inflammatory disease. All in all, a proper treatment can be established, and at times, a biopsy can be avoided.

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“…Only 10% to 20% of patients with IOI experience decreased vision, and complete vision loss is exceptionally rare. 1,5 The mainstay of treatment involves systemic steroids, which typically results in remission. Although the efficacy of treatment can vary, absolute failure to improve after steroid administration is uncommon.…”

mentioning

confidence: 99%

Rapid and complete vision loss due to idiopathic orbital inflammation with partial recovery following orbital decompression

Vrcek¹,

Wotipka

Somogyi³

et al. 2018

Baylor University Medical Center Proceedings

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We present a case of a 36-year-old man who developed rapid onset of proptosis and orbital compartment syndrome resulting in no light perception vision. He was treated with surgery and subsequent rituximab therapy with some recovery of vision and motility. Though the range of presentations of idiopathic orbital inflammation is broad, such rapid evolution of proptosis with complete vision loss is very uncommon.

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Idiopathic Sclerosing Orbital Inflammation

Cited by 49 publications

References 26 publications

Consensus on Diagnostic Criteria of Idiopathic Orbital Inflammation Using a Modified Delphi Approach

Consensus on Diagnostic Criteria of Idiopathic Orbital Inflammation Using a Modified Delphi Approach

CT and MR imaging of orbital inflammation

Rapid and complete vision loss due to idiopathic orbital inflammation with partial recovery following orbital decompression

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