Survey of Patients With Idiopathic Orbital Inflammation Syndrome (IOIS): Clinical, Morphological and Pathological Features and Treatment Outcomes
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 87
- 试验地点
- 1
- 主要终点
- Percentage of remission, relapse or resistance among patients with IOIS during the 24 month follow-up
研究概览
简要总结
The purpose of this study is to characterise the clinical features, histopathology and the treatment outcomes of patients with idiopathic orbital inflammation syndrome.
详细描述
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.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Other
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Patients with biopsy proven IOIS or presumed IOIS
- •Patients with chronic IOS
- •Patient with inaugural IOIS or being treated for IOIS
排除标准
- •Patients who do not fulfill the inclusion criteria
- •Patients with systemic disease-associated IOIS
- •Incomplete follow-up of patients treated for IOIS
结局指标
主要结局
Percentage of remission, relapse or resistance among patients with IOIS during the 24 month follow-up
时间窗: The remission, relapse, or the resistance at inclusion (for those previously diagnosed as IOIS), and at 6, 12, 18 and 24 months
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.
次要结局
- Histopathological classification of IOIS patients(at diagnostic)
- MRI features (muscle enlargement, irregular borders, extension to the orbital fat, enhancement around globe) of patients with IOIS(at diagnosis and in case of remission, or relapse, or resistance.)
- Incidence of orbital lymphomas(at 6, 12, 18, 24 months.)
- Ophthalmologic features (laterality, pain, visual acuity, eye movement and eyelid)(at diagnosis and in case of remission, or relapse, or resistance)
- Immunologic features of IOIS patients(at inclusion)
- Cumulated dose of prednisone(at remission, or relapse, or resistance)