Survey of Patients With Idiopathic Orbital Inflammation Syndrome

Study Overview
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Brief Summary
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The purpose of this study is to characterise the clinical features, histopathology and the treatment outcomes of patients with idiopathic orbital inflammation syndrome.

Detailed Description
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Idiopathic orbital inflammatory syndrome (IOIS) is a heterogeneous group of disorders characterised by orbital inflammation without any identifiable local or systemic causes. It is a rare clinical entity and a diagnosis of exclusion. Lymphomas, thyroid eye diseases or systemic diseases can have similar presentation and so, a histopathological diagnosis is considered important. IOIS is a difficult condition to treat. Compilation of reported small series of patients with IOIS suggested that they require multiple systemic immunosuppressant drugs and radiotherapy. Recently, a large monocentric study including patients with biopsy proven IOIS showed that up to 40% of them can relapse. Their clinical and pathological features did not correlate with treatments outcomes. The investigators decide therefore to conduct a multicentric retro/ prospective study to determine clinical features, histopathology and treatment outcomes of French patients with IOIS.

Official Title
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Survey of Patients With Idiopathic Orbital Inflammation Syndrome (IOIS): Clinical, Morphological and Pathological Features and Treatment Outcomes

Conditions
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Orbital Ischemic Syndrome, Orbital Pseudotumor

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients with biopsy proven IOIS or presumed IOIS Patients with chronic IOS Patient with inaugural IOIS or being treated for IOIS Exclusion Criteria: Patients who do not fulfill the inclusion criteria Patients with systemic disease-associated IOIS Incomplete follow-up of patients treated for IOIS

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Percentage of remission, relapse or resistance among patients with IOIS during the 24 month follow-up | Remission: absence of steroids, their withdrawal or their pursuit at a dose ≤ 10 mg/d in the absence of immunosuppressor treatment. Relapse: re initiation of steroids, or their ascension in patients for whom they have been reduced to less than 20 mg/d. Resistance: inability to reduce steroids at an effective dose ≤ 20 mg/d. | The remission, relapse, or the resistance at inclusion (for those previously diagnosed as IOIS), and at 6, 12, 18 and 24 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Histopathological classification of IOIS patients | -Histopathological forms of the IOIS patients at diagnosis, according to the classification described by Mombaerts, namely: classical orbital pseudotumor, sclerosing orbital pseudotumor, granulomatous orbital pseudotumor and vasculitic pseudotumor. | at diagnostic | 
| Ophthalmologic features (laterality, pain, visual acuity, eye movement and eyelid) | The clinical manifestations of systemic diseases mentioned below, will be evaluated in case of relapse or resistance: Grave's disease or auto immune thyroiditis, sarcoidosis, Wegener's granulomatosis, polyarteritis nodosa, Churg- Strauss syndrome, systemic lupus erythematosus and Gougerot-Sjögren syndrome. | at diagnosis and in case of remission, or relapse, or resistance | 
| MRI features (muscle enlargement, irregular borders, extension to the orbital fat, enhancement around globe) of patients with IOIS | the lesional topography and the T1/T2 weighted sequences will be studied | at diagnosis and in case of remission, or relapse, or resistance. | 
| Immunologic features of IOIS patients | the IgG4 level and ANA in sera will be assessed | at inclusion | 
| Cumulated dose of prednisone |  | at remission, or relapse, or resistance | 
| Incidence of orbital lymphomas |  | at 6, 12, 18, 24 months. | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Observational study, IOIS, Orbital Pseudotumor, Outcome, Treatment