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Completed

NCT Number: NCT01443000

Survey of Patients With Idiopathic Orbital Inflammation Syndrome

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

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Key information

Conditions

Orbital Ischemic Syndrome Eye Diseases Orbital Diseases Orbital Pseudotumor

Age range

18 year and older

Sex eligibility

All sexes

Study type

Observational

Primary location

Service de Médecine interne - Hôpital Avicenne

Bobigny, 93000, France

About this study

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.

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

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

Treatment and study plan

Primary outcomes

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

    Time frame: 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 outcomes

  1. Histopathological classification of IOIS patients

    Time frame: 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.

  2. Ophthalmologic features (laterality, pain, visual acuity, eye movement and eyelid)

    Time frame: 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.

  3. MRI features (muscle enlargement, irregular borders, extension to the orbital fat, enhancement around globe) of patients with IOIS

    Time frame: at diagnosis and in case of remission, or relapse, or resistance.

    the lesional topography and the T1/T2 weighted sequences will be studied

  4. Immunologic features of IOIS patients

    Time frame: at inclusion

    the IgG4 level and ANA in sera will be assessed

  5. Cumulated dose of prednisone

    Time frame: at remission, or relapse, or resistance

  6. Incidence of orbital lymphomas

    Time frame: at 6, 12, 18, 24 months.

Sponsors and collaborators

Lead sponsor

Assistance Publique - Hôpitaux de Paris

Other

Collaborators

  • Ministry of Health, France

Registry information

Official study title

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

Acronym: SIOI

Important dates

Study start
2012
Primary completion
2017
Study completion
2017
First posted
Sep 29, 2011
Registry last updated
Nov 22, 2021

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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