SIOI: Survey of Patients With Idiopathic Orbital Inflammation Syndrome

Sponsor

Assistance Publique - Hôpitaux de Paris (Other)

Overall Status

Completed

CT.gov ID

NCT01443000

Collaborator

Ministry of Health, France (Other)

Enrollment

Location

Actual Duration (Months)

1.4

Patients Per Site Per Month

Study Details

Study Description

Brief Summary

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

Condition or Disease	Intervention/Treatment	Phase
Orbital Ischemic Syndrome Orbital Pseudotumor

Detailed Description

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.

Study Design

Study Type:

Observational

Actual Enrollment :

87 participants

Observational Model:

Cohort

Time Perspective:

Other

Official Title:

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

Actual Study Start Date :

Mar 3, 2012

Actual Primary Completion Date :

Jun 3, 2017

Actual Study Completion Date :

Jun 3, 2017

Outcome Measures

Primary Outcome Measures

Percentage of remission, relapse or resistance among patients with IOIS during the 24 month follow-up [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.

Secondary Outcome Measures

Histopathological classification of IOIS patients [at diagnostic]
-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.
Ophthalmologic features (laterality, pain, visual acuity, eye movement and eyelid) [at diagnosis and in case of remission, or relapse, or resistance]
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.
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.]
the lesional topography and the T1/T2 weighted sequences will be studied
Immunologic features of IOIS patients [at inclusion]
the IgG4 level and ANA in sera will be assessed
Cumulated dose of prednisone [at remission, or relapse, or resistance]
Incidence of orbital lymphomas [at 6, 12, 18, 24 months.]