Survey of Patients With Idiopathic Orbital Inflammation Syndrome

Assistance Publique - Hôpitaux de Paris87 enrolled

Overview

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.

Study Type

OBSERVATIONAL

Enrollment

Conditions

Orbital Ischemic Syndrome Orbital Pseudotumor

Eligibility

Sex: ALLMin age: 18 Years

Medical Language ↔ Plain English

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

Locations (1)

Service de Médecine interne - Hôpital Avicenne

Bobigny, France

Outcomes

Primary Outcomes

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.

Time frame: The remission, relapse, or the resistance at inclusion (for those previously diagnosed as IOIS), and at 6, 12, 18 and 24 months

Secondary Outcomes

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.

Time frame: 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.

Time frame: 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

Time frame: 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

Data from ClinicalTrials.gov

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