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Abstract: Introduction: Acute adrenal insufficiency during antiphospholipid syndrome is usually due to a bilateral adrenal hemorrhage. For a patient, an unilateral involvement has induce an acute adrenal insufficiency. Case report: A patient with an antiphopsholipid syndrome has developed an acute adrenal insufficiency with an unilateral adrenal hemorrhage. This complication was caused by a concurrent steroid therapy, for an associated lupus, which resulted in a suppression of the controlateral adrenal gland. Conclusion: In patients with antiphospholipid syndrome and concurrent steroid therapy, usually for lupus, unilateral adrenal hemorrhage can cause acute adrenal insufficiency. Withdrawal of steroid might be difficult in case of undetected bilateral hemorrhage. [Copyright &y& Elsevier]

Controlling long-term inflammation during non-infectious intermediate, posterior or panuveitis while limiting side effects remains challenging. There is no standardized pre-therapeutic evaluation providing diagnostic certainty, but some simple tests allow us to identifiy the main etiologies. The ophthalmologist identifies the type of uveitis, and the internist completes the investigations according to the ophthalmologist's findings. Fundus photographs, optical coherence tomography, and fluorescein and indocyanine green angiography should be considered during diagnosis and follow-up. Ocular complications of uveitis are numerous. They require close monitoring and specific medical and sometimes surgical management. The growing number of available drugs makes it possible to optimize the management of these conditions with varied etiologies and presentations. Currently, systemic corticosteroids remain the mainstay of therapy, and other alternatives are considered in the case of poor tolerance, steroid resistance or dependence. The choice of a systemic, periocular or intravitreal treatment depends on several factors: chronicity or recurrence of uveitis, duration, bilaterality, association with a systemic inflammatory disease, the presence of contraindications to certain treatments, and also socioeconomic constraints. It is of the utmost importance to find the best compromise allowing tight control of ocular inflammation by means of adapted systemic and/or local treatment while avoiding the main complications., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Introduction: Hyper-IgG4 syndrome is a rare cause of bilateral proptosis. It must always be considered after ruling out orbital lymphoma., Case Report: We report a case of progressive bilateral proptosis for 4 years in a 34-year-old man. Orbital MRI showed an infiltrative process extending to the orbital fat, extraocular muscles and lacrimal glands. Lacrimal gland biopsy with immunohistochemical study showed a lymphoplasmocytic infiltrate rich in IgG4 and fibrosis. The diagnosis of orbital hyper-IgG4 syndrome was suggested. The patient responded well to systemic steroid treatment., Discussion: Orbital hyper-IgG4 syndrome manifests most often as pseudo-tumoral bilateral proptosis. Elevated IgG4 levels are neither sensitive nor specific. Biopsy with immunohistochemical study is the key to diagnosis. Systemic steroid treatment is the gold standard, but recurrences may occur., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Introduction: Orbital inflammatory syndromes include a wide variety of inflammatory intraorbital processes which are very different in terms of clinical presentation and prognosis. We currently prefer to differentiate so-called "specific" inflammations, for which an etiology is able to be identified, from idiopathic orbital inflammatory syndromes (IOIS), for which the etiology remains unknown and the histology is nonspecific., Purpose: To propose an efficient diagnostic approach for clinicians managing patients with non-Graves' orbital inflammations., Materials and Methods: This is a retrospective and prospective study concerning 61 patients managed by the medical team for non-Graves' orbital inflammations between May, 1999 and May, 2013 in the ophthalmology departments of Nice and Limoges university hospitals in France. Seventeen specific inflammations, 19 orbital lymphomas and 25 idiopathic orbital inflammatory syndromes were included. Patients were divided into two groups. Thirty-six patients (group 1) underwent primary biopsy, while for the other 25 (group 2), therapy was begun empirically without biopsy. We could therefore compare both approaches in terms of diagnostic efficiency and time until identification of a specific etiology., Results: Our statistical results show that an approach without primary biopsy leads to a number of specific diagnoses statistically much lower than that obtained by the approach with primary biopsy. Also, the risk of missing a specific inflammation (with as a consequence an inappropriate treatment and a risk of functional sequelae as well as a fatal risk of missing a lymphoproliferative pathology) is very clearly higher in the case of not performing primary biopsy. Finally, the average time elapsed between the initial consultation with the ophthalmologist and a specific diagnosis was one month in the case of the first approach, while this delay was almost three times higher with the second approach, with a mean of 2.91 months (P<0.01)., Discussion: Our study shows that biopsy should be the mainstay of diagnostic management. A trial of empiric treatment is only performed first in myositis or in locations where biopsy could jeopardize functional prognosis. It should only be done after biopsy in all other cases. Of course, in all cases of relapse or recurrence after treatment, biopsy should be performed or repeated., Conclusion: The diagnostic work-up of a patient with an orbital inflammatory process must of course include blood testing and orbital imaging, but also a systematic primary biopsy for histological examination in the vast majority of cases. It must be repeated at least in the case of any doubt about the diagnosis or in the case of any recurrence or resistance to treatment., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)