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3 results on '"Isabelle, Badelon"'

1. Cellular pattern and orbital fat involvement are possible risk factors for the failure of corticosteroids in patients with pure idiopathic orbital inflammation syndrome: lessons from the French prospective SIOI cohort study (part II)

Author

David Saadoun, Eric Vicaut, Valérie Touitou, Jacques Lagier, Frédéric Mouriaux, Pierre-Yves Robert, Antoine Rousseau, Nicolas Schleinitz, Jean-Marie Michot, Loic Guillevin, Matthieu Groh, Antoine Martin, Philippe Blanche, Sébastien Abad, Robin Dhote, Felix Ackermann, Boris Bienvenu, Olivier Galatoire, Sophie Coffin-Pichonnet, Pierre Aucouturier, Françoise Heran, Bertrand Vabres, Ambre La Rosa, Stéphane Tran Ba, Nahla Cucherousset, Neila Sedira, Emmanuel Héron, Aïcha Abbas, Mboup Bassirou, Isabelle Badelon, Mathieu Zmuda, Alain Ducasse, Isabelle Larré, Amélie Brabant-Viau, Jean Luc George, Jean Maalouf, Anne-Laure Fisch, Nathalie Tieulé, Juliette Delmas, Alexandre Vallon, Natahlie Massart, Marie Alexandra Alyanakian, Pierre Olivier Schischmanoff, Nabila Pizzi, Nathalie Kingue-Elessa, Jad Abou Ghantous, and Solohaja-Faniaha Dimby

Subjects
Ophthalmology, RE1-994
Abstract

Purpose To better characterise the effects of corticosteroids on the course of pure idiopathic orbital inflammation syndrome (pIOIS).Methods This was a national, multicentre, prospective, non-interventional cohort study (SIOI). Among the 35 patients with histologically proven orbital inflammation who had previously been studied for their IgG4 immunostaining status, we selected those with a negative IgG4 status (ie, pIOIS) who received corticosteroids as single first-line treatment. Clinical, morphological and pathological findings at diagnosis and during follow-up from treatment initiation to study completion were analysed. Patients were assessed for their response to prednisone after the 24-month prospective phase in terms of remission (≤10 mg/d) or failure (>10 mg/d). Daily standard doses of prednisone (DSDP) were calculated at different time-points and compared between response groups.Results Of the 17 patients with pIOIS included in the final analysis, two-thirds received corticosteroids only. DSDP (mg/kg-day) were significantly higher at the time of failure in eight patients (47%) than in nine (53%) remitting at M24 (0.16 vs 0.045; p: 0.03). Notably, patients with pIOIS with a cellular pattern or orbital fat involvement tended to receive higher daily corticosteroid doses in the event of failure than remission (0.16 vs 0.045 and 0.12 vs 0.042, respectively). During treatment, maximal DSDP was 0.52 in failed patients.Conclusion The highest corticosteroid doses were insufficient to prevent failure in patients with pIOIS, particularly in those with a cellular pattern or orbital fat involvement. Large-scale interventional studies are now necessary to clarify prognostic factors and optimise corticosteroid management in patients with pIOIS.

Published
2024
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2. IgG4-related diffuse perineural disease

Author

Michael, Soussan, Aicha, Medjoul, Isabelle, Badelon, Alexis, Guyot, Alexis, Guillot, Antoine, Martin, and Sébastien, Abad

Subjects
Pathology, medicine.medical_specialty, Tomography Scanners, X-Ray Computed, genetic structures, Nerve root, Plasma cell, Autoimmune Diseases, Fibrosis, Fluorodeoxyglucose F18, Immunoglobulin g4, Biopsy, medicine, Humans, Right optic nerve, medicine.diagnostic_test, business.industry, Magnetic resonance imaging, Optic Nerve, Middle Aged, medicine.disease, Magnetic Resonance Imaging, eye diseases, medicine.anatomical_structure, Immunoglobulin G, Positron-Emission Tomography, Diffuse infiltration, Female, Neurology (clinical), business, Orbit
Abstract

A 55-year-old woman had right exophthalmia. Eleven years previously, she had orbital irradiation for refractory nonspecific orbital inflammation. PET/CT revealed FDG uptake in the right orbit and paravertebral masses (figure 1, A and C). MRI showed an enlargement of the right optic nerve and orbital muscles, and a diffuse infiltration involving lumbodorsal and sacral nerve roots (figure 1, B and D). The orbital biopsy demonstrated immunoglobulin G4 (IgG4)+ plasma cell infiltrate and a storiform fibrosis (figure 2), identical to the histopathologic features of the nerve root biopsy, and suggestive of IgG4-related diffuse perineural disease.1,2 No treatment was started in the absence of neurologic symptoms. One year later, the patient had no further symptoms.

Published
2014

3. [Graves' ophthalmopathy]

Author

Isabelle, Badelon and Serge, Morax

Subjects
Oculomotor Muscles, Optic Nerve Diseases, Humans, Severity of Illness Index, Graves Disease
Abstract

Graves' ophthalmopathy (GO) is a chronic autoimmune process that affects the retrobulbar tissue and has strong etiological links with graves' disease. Pathogenesis is incompletely understood. Symptoms include proptosis, extraocular muscle dysfunction, eyelid swelling and retraction. In its severe expression, it is a disfiguring and potentially sight threatening disease that profoundly influences and impairs the quality of life of affected individuals. Only a minority of patients develop severe expressions of the disease, requiring aggressive treatments. Glucocorticoids, orbital radiotherapy and decompressive orbital surgery represent a mainstay and a well established treatment for severe and active GO.

Published
2005

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