Clinical Trial: Survey of Patients With Idiopathic Orbital Inflammation Syndrome

Study Status: Active, not recruiting
Recruit Status: Active, not recruiting
Study Type: Observational

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

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 Summary: 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.
Sponsor: Assistance Publique - Hôpitaux de Paris

Current Primary Outcome: 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.



Original Primary Outcome: Same as current

Current Secondary Outcome:

  • 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.
  • 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.
  • 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
  • Immunologic features of IOIS patients [ Time Frame: at inclusion ]
    the IgG4 level and ANA in sera will be assessed
  • Cumulated dose of prednisone [ Time Frame: at remission, or relapse, or resistance ]
  • Incidence of orbital lymphomas [ Time Frame: at 6, 12, 18, 24 months. ]


Original Secondary Outcome: Same as current

Information By: Assistance Publique - Hôpitaux de Paris

Dates:
Date Received: June 15, 2011
Date Started: March 2012
Date Completion: June 2017
Last Updated: November 3, 2016
Last Verified: November 2016