Survey of Patients With Idiopathic Orbital Inflammation Syndrome

Orbital Pseudotumor Clinical Trial

— SIOI  

Official title:

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

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT01443000
• Other study ID #: NI08014
• Secondary ID: 2010-A00512-37
• Status: Completed
• Start date: March 3, 2012
• Est. completion date: June 3, 2017

Study information

• Verified date: November 2021
• Source: Assistance Publique - Hôpitaux de Paris
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial Summary

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

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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 87
• Est. completion date: June 3, 2017
• Est. primary completion date: June 3, 2017
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

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

Related Conditions & MeSH terms

• Inflammation
• Orbital Ischemic Syndrome
• Orbital Pseudotumor
• Syndrome

Locations

Country	Name	City	State
France	Service de Médecine interne - Hôpital Avicenne	Bobigny

Sponsors (2)

Lead Sponsor	Collaborator
Assistance Publique - Hôpitaux de Paris	Ministry of Health, France

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	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	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
Secondary	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
Secondary	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.
Secondary	Immunologic features of IOIS patients	the IgG4 level and ANA in sera will be assessed	at inclusion
Secondary	Cumulated dose of prednisone		at remission, or relapse, or resistance
Secondary	Incidence of orbital lymphomas		at 6, 12, 18, 24 months.