Survey of Patients With Idiopathic Orbital Inflammation Syndrome (SIOI)

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

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

Study Overview

• Status: Completed
• Conditions: Orbital Pseudotumor; Orbital Ischemic 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 Type: Observational
• Enrollment (Actual): 87

Contacts and Locations

Study Locations

• France
  • Bobigny, France, 93000
    • Service de Médecine interne - Hôpital Avicenne

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (ADULT, OLDER_ADULT)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Patients followed for IOIS

Description

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 Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	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

Secondary Outcome Measures

Outcome Measure	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
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.

Collaborators and Investigators

• Sponsor: Assistance Publique - Hôpitaux de Paris
• Collaborators: Ministry of Health, France
• Investigators: Principal Investigator: Sébastien ABAD, MD, Assistance Publique - Hôpitaux de Paris

Study record dates

Study Major Dates

• Study Start (ACTUAL): March 3, 2012
• Primary Completion (ACTUAL): June 3, 2017
• Study Completion (ACTUAL): June 3, 2017

Study Registration Dates

• First Submitted: June 15, 2011
• First Submitted That Met QC Criteria: September 28, 2011
• First Posted (ESTIMATE): September 29, 2011

Study Record Updates

• Last Update Posted (ACTUAL): November 22, 2021
• Last Update Submitted That Met QC Criteria: November 19, 2021
• Last Verified: November 1, 2021

More Information

Terms related to this study

• Keywords: Treatment; Outcome; Observational study; IOIS; Orbital Pseudotumor
• Additional Relevant MeSH Terms: Pathologic Processes; Eye Diseases; Disease; Orbital Diseases; Syndrome; Inflammation; Orbital Pseudotumor
• Other Study ID Numbers: NI08014; 2010-A00512-37 (OTHER: IDRCB)