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Objective To provide an original observation of Behçet’s disease, revealed by an inflammatory pseudotumor of the orbit. Methods We reported an observation of an inflammatory pseudotumor of the orbit that revealed Behçet’s disease. Results Twenty-Eight years old patient was admitted to the internal medicine department for painful edema in the right eye with headache and loss of visual acuity. Ophthalmologic examination revealed eye protrusion with conjunctival hyperemia. Orbital MRI resonance imaging revealed periorbital inflammatory thickening associated with inflammatory myositis. Behçet’s was diagnosed based on a history of recurrent oral aphthous since childhood, pseudofolliculitis, pathergy test positivity, and negativity of the rest of the etiological investigations. The evolution was spectacular with boli methylprednisolone and colchicine prescriptions. Conclusion Although the association is rare, Behçet’s disease should be included in the workup of inflammatory pseudotumors of the orbit. more...

To assess the usefulness of FDG-PET/CT as a potential diagnostic tool for detecting underlying systemic diseases (SD) in patients with orbital inflammatory disorders (OID). All consecutive patients managed for new-onset OID between 2011 and 2018 in a tertiary referral center, who underwent FDG-PET/CT as part of the etiological diagnostic workup were evaluated. To quantify the incremental value of FDG-PET/CT over standard diagnostic workup, the Net Reclassification Index (NRI) and Integrated Discrimination Index (IDI) were used. Among the 22 patients enrolled, 11 (50%) had a positive FDG-PET/CT. After clinicobiological evaluation, FDG-PET/CT correctly reclassified 4(29%) of 14 patients with SD (p =.04) and 1(13%) of 8 with idiopathic orbital inflammation syndrome (p =.32). NRI and IDI were 0.41 ± 0.17 (p =.03) and 0.38 ± 0.08 (p <.001), respectively. FDG-PET/CT successfully detected asymptomatic lesions in all (n = 4) patients with lymphoma. FDG-PET/CT enabled accurate reclassification of more than one-quarter of patients with SD, especially extraorbital lymphomas. [ABSTRACT FROM AUTHOR] more...

Idiopathic sclerosing orbital inflammatory syndrome (ISOIS) is a rare, progressive and hard to control disease. There is a deep gap of evidence regarding application of disease-modifying drugs (DMD) regimen as a potentially effective treatment for orbital inflammatory diseases. We aimed to report the results of using DMDs and discuss the concept of applying this modality of treatment in patients with ISOIS. This was a prospective interventional case series conducted in a tertiary university-based hospital. Biopsy proven patients with active ISOIS were included. Systematic criteria were developed to define and measure disease activity and monitor response to treatment. A DMD regimen including an anti-tumor necrosis factor alpha (anti-TNF alpha) agent plus azathioprine and low-dose corticosteroids were used. Comprehensive ophthalmic, orbital and systemic assessments were performed during each visit. Five eligible patients with primary ISOIS were included. Mean age was 34.20 (SD = 13.33, range 19–53) years. Three had unilateral and two had bilateral involvement. Four had diffuse orbital involvement pattern and progressive worsening of visual functions, reduced extraocular motility and proptosis. In one patient the disease was localized to extraocular muscle and lacrimal gland. Disease activity was decreased and stabilized after DMDs regimen in all patients. Mean follow up was 32.80 (SD = 30.80, range: 12–86) months. Biologic DMD (b-DMD) including anti-TNF alpha, corticosteroid and azathioprine were effective in decreasing disease activity and could change course of the disease. This study supports the concept of using b-DMD regimen in treatment of ISOIS. [ABSTRACT FROM AUTHOR] more...