Graves’ orbitopathy as a rare disease in Europe: a European Group on Graves’ Orbitopathy (EUGOGO) position statement

Perros, Petros; Hegedűs, László; Bartalena, Luigi; Marcocci, Claudio; Kahaly, George J.; Baldeschi, Lelio; Salvi, Mario; Lazarus, John H.; Eckstein, Anja; Pitz, Susanne; Boboridis, Kostas; Anagnostis, Panagiotis; Ayvaz, Göksün; Boschi, Antonella; Brix, Thomas Heiberg; Currò, Nicola; Konuk, Onur; Marinò, Michele; Mitchell, Anna L.; Stanković, Branislav; Törüner, Fosun Baloş; Arx, George von; Žarković, Miloš; Wiersinga, Wilmar M.

doi:10.1186/s13023-017-0625-1

Cited by 131 publications

(104 citation statements)

References 38 publications

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“…This prospective BOSU study into DON is the first in the United Kingdom. The annual incidence of DON is estimated to be 0.96 cases per million population per annum [6]. Assuming the UK population is 65 million we can extrapolate that there should be~62 cases of DON in the UK per annum.…”

Section: Discussionmentioning

confidence: 99%

A British Ophthalmological Surveillance Unit (BOSU) study into dysthyroid optic neuropathy in the United Kingdom

Wong

Dickinson²,

Perros

et al. 2018

Eye

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This prospective British Ophthalmological Surveillance Unit (BOSU) study on dysthyroid optic neuropathy (DON) determines the incidence, presenting features and management throughout the UK. New cases were identified through the BOSU yellow card and an initial questionnaire and a subsequent 9-month follow-up questionnaire were posted out. From August 2015 to August 2016 DON was reported in 49 patients with 71 eyes affected, 22 patients had bilateral DON. The most common presenting symptom was blurred vision (83%) and the most common examination finding was upgaze restriction (85%). 85% of patients were initially treated with 3 days of either 1 g or 500 mg intravenous methyl prednisolone. We received 25 follow-up questionnaires (51% of the initial cohort) with 38 eyes treated for DON and 13 bilateral cases. The average steroid dose over 9 months was 4.5 g and 47% of patients had a surgical orbital decompression. The mean visual acuity gain after 9 months of follow-up for all patients was 0.25 LogMAR. The mean visual acuity gain after just medical therapy was 0.25 LogMAR and after both medical therapy and orbital decompression it was 0.24 LogMAR. In conclusion, the incidence of DON in the UK from this study is 0.75 per million population per annum. The majority of patients are treated with initial medical therapy and almost half of all patients subsequently went on to have an orbital decompression. With either medical therapy or medical and surgical therapy, vision can improve in patients with DON.

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Section: Discussionmentioning

confidence: 99%

A British Ophthalmological Surveillance Unit (BOSU) study into dysthyroid optic neuropathy in the United Kingdom

Wong

Dickinson²,

Perros

et al. 2018

Eye

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“…More than half of newly generated B lymphocytes have some degree of autoreactivity [23]. These immature autoreactive B cells are extreme sensitive to apoptosis because they express high levels of death receptor Fas, a mechanism to eliminate autoreactive B cells [24].…”

Section: Discussionmentioning

confidence: 99%

Skewed expression profile of receptors for BAFF on peripheral blood B lymphocytes in Graves’ Disease

Wang¹,

Huang²,

Zhang³

et al. 2020

Preprint

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Background Previous studies have demonstrated altered serum B lymphocyte activating factor (BAFF) level and expression of BAFF and BAFF receptor (BAFF-R) in thyroid infiltrating lymphocytes in Graves’ disease (GD) patients. The aim of the present study was to investigate expression of receptors for BAFF on the peripheral blood B lymphocytes in addition to serum BAFF concentrations. Methods Fifty-two GD patients and twenty-three healthy controls (HC) were recruited in this study. Serum BAFF concentrations were measure by ELISA. Expression of receptors for BAFF on peripheral blood B lymphocytes, including BAFF-R and transmembrane activator and calcium-modulating and cyclophilin ligand interactor (TACI), was analyzed by flow cytometry. Serum BAFF levels, expression of BAFF-R and TACI on peripheral blood B lymphocytes were compared between GD and HC groups. Correlations between serum BAFF concentrations and thyroid function and thyrotropin receptor autoantibody (TRAb) titers were analyzed in GD patients. In 10 patients with Graves’ orbitopathy (GO) receiving steroids therapy, effects of steroids on serum BAFF levels and expression of BAFF-R and TACI were observed. Results Serum BAFF levels were significantly higher in GD patients (1.18 ± 0.33 ng/ml) than those in HC (0.93 ± 0.24 ng/ml, P = 0.0011). In GD patients, serum BAFF levels correlated positively with free thyroxine concentrations (Spearman r = 0.2460, P = 0.0394), but not TRAb titers (P = 0.4734). BAFF-R expression on peripheral B lymphocytes were elevated in GD (mean MFI: 4.52 ± 2.06 in GD vs 3.00 ± 0.87 in HC, P < 0.0001), while TACI expression on peripheral B lymphocytes were decreased in GD (mean MFI: 7.96 ± 4.06 in GD vs 9.10 ± 3.37 in HC, P = 0.0331). Steroids significantly suppressed serum BAFF concentrations ( from 1.18 ± 0.27 ng/ml to 0.97 ± 0.10 ng/ml, P = 0.0364) and BAFF-R expression on peripheral blood B lymphocytes in GO patients (mean MFI from 6.26 ± 4.91 to 4.05 ± 1.58, P = 0.0083). Change of TACI expression on B lymphocytes was not statistically significant after steroids therapy. Conclusions Skewed expression profile of receptors for BAFF on B lymphocytes may mediate the autoimmunity in GD. Restoring the normal expression profile of receptors for BAFF could be a new strategy for treating and cueing GD.

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“…Maryse Baillye and Lei Zhang have applied in vitro models to unravel the tissueremodelling processes at play in TED? Maryse is an expert on fibrosis, which remains one of the least-wellunderstood aspects of TED, and as noted above, can last forever [6]. She recently reported on the stem-cell properties of orbital fibroblasts [15] and also has a superb 3D in vitro model that will be invaluable in dissecting how pressure influences the disease [16], many TED patients having shown increased intra-ocular pressure.…”

Section: Introductionmentioning

confidence: 98%

“…The expansion occurs within an inflexible orbit and results in proptosis; indeed, even patients with less-severe exophthalmos may suffer sight-threatening optic neuropathy [5]. The extra-ocular muscles may become fibrosed and this can cause problems with eye motility; fibrosis may be considered a life-long condition [6].…”

Section: Introductionmentioning

confidence: 99%

Cambridge Ophthalmological Symposium 2018: introduction and reflections on the day

Ludgate¹

2018

Eye

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I was privileged to be one of the co-chairs, along with Professor Tim Sullivan (Brisbane, Australia), for the Cambridge Ophthalmological Society (COS) annual international symposium, which, this year, was dedicated to thyroid eye disease (TED). Together with the organisers, Miss Rachna Murthy and Professor Keith Martin from COS, we compiled an impressive programme covering all aspects of the condition from events happening in a single orbital cell to improved surgical approaches.

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Graves’ orbitopathy as a rare disease in Europe: a European Group on Graves’ Orbitopathy (EUGOGO) position statement

Cited by 131 publications

References 38 publications

A British Ophthalmological Surveillance Unit (BOSU) study into dysthyroid optic neuropathy in the United Kingdom

A British Ophthalmological Surveillance Unit (BOSU) study into dysthyroid optic neuropathy in the United Kingdom

Skewed expression profile of receptors for BAFF on peripheral blood B lymphocytes in Graves’ Disease

Cambridge Ophthalmological Symposium 2018: introduction and reflections on the day

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