Not provided
Not provided
Not provided
| ID | Type | Description | Link |
|---|---|---|---|
| 2010-A00512-37 | Other Identifier | IDRCB |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Class |
|---|---|
| Ministry of Health, France | OTHER_GOV |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
The purpose of this study is to characterise the clinical features, histopathology and the treatment outcomes of patients with idiopathic orbital inflammation syndrome.
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.
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| 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 |
| 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 |
Not provided
Inclusion Criteria:
Exclusion Criteria:
Not provided
Not provided
Not provided
Patients followed for IOIS
Not provided
| Name | Affiliation | Role |
|---|---|---|
| Sébastien ABAD, MD | Assistance Publique - Hôpitaux de Paris | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Service de Médecine interne - Hôpital Avicenne | Bobigny | 93000 | France |
Not provided
| ID | Term |
|---|---|
| D016727 | Orbital Pseudotumor |
| ID | Term |
|---|---|
| D009916 | Orbital Diseases |
| D005128 | Eye Diseases |
Not provided
Not provided
Not provided
Not provided
Not provided
Blood samples will be frozen to form a serum storage and a DNAthèque. These samples will achieve further analysis based on the evolution of knowledge on mechanisms contributing to the orbital inflammation.
| 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. |