Survey of Patients With Idiopathic Orbital Inflammation Syndrome

Completed

• Conditions: Orbital Ischemic Syndrome; Orbital Pseudotumor

• Registration Number: NCT01443000
• Lead Sponsor: Assistance Publique - Hôpitaux de Paris

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.

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 Timeline

• Study First Submitted: 2011-06-15
• Study First Submitted QC: 2011-09-28
• Study First Posted: 2011-09-29
• Study Start: 2012-03-03
• Primary Completion: 2017-06-03
• Study Completion: 2017-06-03
• Status Verified: 2021-11
• Last Update Submitted: 2021-11-19
• Last Update Posted: 2021-11-22

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 87

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

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
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

Name	Time	Method
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.
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
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	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.
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

Trial Locations

Locations (1)

Service de Médecine interne - Hôpital Avicenne; 🇫🇷 Bobigny, France