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      Debulking Surgery Combined with Low-Dose Oral Prednisolone and Azathioprine for Intractable IgG4-Related Orbital Disease: A Case Report

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          Abstract

          A case of intractable IgG4-related orbital disease (IgG4-ROD) was successfully treated by debulking surgery combined with low-dose prednisolone and azathioprine as maintenance therapy. A 64-year-old man visited our clinic with progressive bilateral upper eyelid swelling and right eye fullness of a year’s duration. He was previously treated with systemic corticosteroids for the IgG4-ROD and experienced a partial clinical response but relapsed upon prednisolone cessation. The patient underwent debulking surgery of the right lacrimal gland and right upper eyelid. His post-operative medication was oral prednisolone (5 mg) every other day and 50 mg azathioprine per day. The patient’s right eye remained asymptomatic during the 18 months of follow-up. Debulking surgery combined with low-dose prednisolone and azathioprine, as a maintenance therapy, is an effective and alternative treatment for the long-term control of intractable IgG4-ROD.

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          Most cited references15

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          IgG4-related disease.

          IgG4-related disease is a protean condition that mimics many malignant, infectious, and inflammatory disorders. This multi-organ immune-mediated condition links many disorders previously regarded as isolated, single-organ diseases without any known underlying systemic condition. It was recognised as a unified entity only 10 years ago. Histopathology is the key to diagnosis. The three central pathology features of IgG4-related disease are lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis. The extent of fibrosis is an important determinant of responsiveness to immunosuppressive therapies. IgG4-related disease generally responds to glucocorticoids in its inflammatory stage, but recurrent or refractory cases are common. Important mechanistic insights have been derived from studies of patients treated by B-cell depletion. Greater awareness of this disease is needed to ensure earlier diagnoses, which can prevent severe organ damage, disabling tissue fibrosis, and even death. Identification of specific antigens and T-cell clones that drive the disease will be the first steps to elucidate the pathogenesis of IgG4-related disease.
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            International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease.

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              Predictors of disease relapse in IgG4-related disease following rituximab.

              IgG4-related disease (IgG4-RD) is a relapsing-remitting condition responsible for fibroinflammatory lesions that can lead to organ damage and life-threatening complications at nearly any anatomical site. The duration of remission following treatment varies and predictors of relapse are unclear. The objectives of this study were to review our experience with rituximab as remission induction in IgG4-RD, to clarify the duration of efficacy and to identify predictors of flare following treatment.
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                Author and article information

                Contributors
                Role: Academic Editor
                Journal
                Medicina (Kaunas)
                Medicina (Kaunas)
                medicina
                Medicina
                MDPI
                1010-660X
                1648-9144
                04 May 2021
                May 2021
                : 57
                : 5
                : 448
                Affiliations
                [1 ]Department of Ophthalmology, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei City 231, Taiwan; serenahsuan@ 123456gmail.com
                [2 ]Division of Allergy, Immunology and Rheumatology, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei City 231, Taiwan; kasper4730@ 123456hotmail.com
                [3 ]School of Medicine, Tzu Chi University, Hua-Liang County 970, Taiwan
                Author notes
                [* ]Correspondence: cgmheye@ 123456gmail.com ; Tel.: +886-2-6628-9779 (ext. 5703); Fax: +886-2-662-8900
                Author information
                https://orcid.org/0000-0002-1864-4993
                Article
                medicina-57-00448
                10.3390/medicina57050448
                8147960
                78d5c933-784b-4d68-a6af-05bd86f86abe
                © 2021 by the authors.

                Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license ( https://creativecommons.org/licenses/by/4.0/).

                History
                : 19 March 2021
                : 29 April 2021
                Categories
                Case Report

                igg4-related orbital disease,debulking surgery,azathioprine,prednisolone

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