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      The 2016 European Thyroid Association/European Group on Graves' Orbitopathy Guidelines for the Management of Graves' Orbitopathy

      guideline

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          Abstract

          Graves' orbitopathy (GO) is the main extrathyroidal manifestation of Graves' disease, though severe forms are rare. Management of GO is often suboptimal, largely because available treatments do not target pathogenic mechanisms of the disease. Treatment should rely on a thorough assessment of the activity and severity of GO and its impact on the patient's quality of life. Local measures (artificial tears, ointments and dark glasses) and control of risk factors for progression (smoking and thyroid dysfunction) are recommended for all patients. In mild GO, a watchful strategy is usually sufficient, but a 6-month course of selenium supplementation is effective in improving mild manifestations and preventing progression to more severe forms. High-dose glucocorticoids (GCs), preferably via the intravenous route, are the first line of treatment for moderate-to-severe and active GO. The optimal cumulative dose appears to be 4.5-5 g of methylprednisolone, but higher doses (up to 8 g) can be used for more severe forms. Shared decision-making is recommended for selecting second-line treatments, including a second course of intravenous GCs, oral GCs combined with orbital radiotherapy or cyclosporine, rituximab or watchful waiting. Rehabilitative treatment (orbital decompression surgery, squint surgery or eyelid surgery) is needed in the majority of patients when GO has been conservatively managed and inactivated by immunosuppressive treatment.

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          Most cited references117

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          Graves' ophthalmopathy.

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            Shared decision making: really putting patients at the centre of healthcare

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              Clinical activity score as a guide in the management of patients with Graves' ophthalmopathy.

              Approximately 35% of patients with Graves' ophthalmopathy do not respond to immunosuppressive treatment. A possible explanation for this finding is that only patients with active ophthalmopathy respond to immunosuppressive treatment, whereas patients with fibrotic end stage disease do not. To distinguish between these two groups and to predict the outcome of immunosuppressive treatment, we developed a clinical activity score (CAS) based on four of the five classical signs of inflammation and tested its efficacy in a double-blind, prospective study. The CAS was determined by an opthalmologist before, on the day of, and after the start of either oral prednisone or retrobulbar irradiation in 43 patients with moderate to severe Graves' ophthalmopathy. The therapeutic outcome was determined by a second ophthalmologist unaware of the CAS stores given. Success of treatment was defined as an improvement in NOSPECS class or grade. Responders (22) and non-responders (21) did not differ in age, sex, duration or severity of their Graves' ophthalmopathy. The pretreatment CAS, however, was significantly higher in responders than in non-responders. Twelve of 22 responders and three of 21 non-responders had a CAS > or = 4 (55% vs 14%; P or = 4 had a similar duration of Graves' ophthalmopathy as patients with a CAS < 4. The clinical activity score has a high predictive value for the outcome of immunosuppressive treatment in Graves' ophthalmopathy. Disease activity, and not disease duration, is the prime determinant of therapeutic outcome.
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                Author and article information

                Journal
                ETJ
                ETJ
                10.1159/issn.2235-0640
                European Thyroid Journal
                S. Karger AG
                2235-0640
                2235-0802
                2016
                March 2016
                02 March 2016
                : 5
                : 1
                : 9-26
                Affiliations
                aDepartment of Clinical and Experimental Medicine, University of Insubria, Endocrine Unit, Ospedale di Circolo, Varese, Italy; bDepartment of Ophthalmology, Hospital Saint Luc, Catholic University of Louvain, Brussels, Belgium; cOphthalmology Department, Aristotle University of Thessaloniki, Thessaloniki, Greece; dZentrum für Augenheilkunde, Universitätsklinikum Essen, Essen, and eDepartment of Medicine I, Johannes Gutenberg University (JGU) Medical Center, Mainz, Germany; fDepartment of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy; gDepartment of Endocrinology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK; hGraves' Orbitopathy Center, Endocrinology, Fondazione Ca' Granda IRCCS, University of Milan, Milan, Italy; iDepartment of Endocrinology and Metabolism, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
                Author notes
                *Prof. Luigi Bartalena, Department of Clinical and Experimental Medicine, University of Insubria, Endocrine Unit, Ospedale di Circolo, Viale Borri 57, IT-21100 Varese (Italy), E-Mail luigi.bartalena@uninsubria.it
                Author information
                https://orcid.org/0000-0001-7320-5574
                Article
                443828 PMC4836120 Eur Thyroid J 2016;5:9-26
                10.1159/000443828
                PMC4836120
                27099835
                f16b6a09-f76f-4429-adfb-88eadf8c0be7
                © 2016 European Thyroid Association Published by S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                : 05 October 2015
                : 05 January 2016
                Page count
                Figures: 2, Tables: 5, References: 134, Pages: 18
                Categories
                Guidelines

                Endocrinology & Diabetes,Neurology,Nutrition & Dietetics,Sexual medicine,Internal medicine,Pharmacology & Pharmaceutical medicine
                Squint surgery,Glucocorticoids,Selenium,Eyelid surgery,Graves’ orbitopathy,Rituximab,Orbital decompression,Cyclosporine,Orbital radiotherapy

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