Abstract

(1) Background: Graves’ orbitopathy (GO) is an autoimmune inflammation of the orbital tissues and the most common extra-thyroid symptom of Graves’ disease (GD). Mild cases of GO are often misdiagnosed, which prolongs the diagnostic and therapeutic process, leading to exacerbation of the disease. A severe course of GO may cause permanent vision loss. (2) Methods: The article presents an analysis of GO—its etiopathogenesis, diagnostics, current treatment and potential future therapeutic options based on a review of the currently available literature of the subject. (3) Results: Current treatment of the active GO consists predominantly in intravenous glucocorticoids (GCs) administration in combination with orbital radiotherapy. The growing knowledge on the pathogenesis of the disease has contributed to multiple trials of the use of immunosuppressive drugs and monoclonal antibodies which may be potentially effective in the treatment of GO. Immunosuppressive treatment is not effective in patients in whom a chronic inflammatory process has caused fibrous changes in the orbits. In such cases surgical treatment is performed—including orbital decompression, adipose tissue removal, oculomotor muscle surgery, eyelid alignment and blepharoplasty. (4) Conclusions: Management of GO is difficult and requires interdisciplinary cooperation in endocrinology; ophthalmology, radiation oncology and surgery. The possibilities of undertaking a reliable assessment and comparison of the efficacy and safety of the therapeutic strategies are limited due to the heterogeneity of the available studies conducted mostly on small group of patients, with no comparison with classic systemic steroid therapy. The registration by FDA of Teprotumumab, an IGF1-R antagonist, in January 2020 may be a milestone in future management of active GO. However, many clinical questions require to be investigated first.

Highlights

  • The Graves’ orbitopathy (GO) is an autoimmune inflammation of the orbital tissues and the most common extra-thyroid symptom of Graves’ disease (GD)

  • The guidelines emphasize the importance of individualizing the treatment strategy, so that potential benefits do not outweigh the possible side effects. It is recommended in routine clinical practice that the quality of life of the patients is estimated based on the use of a validated GO-specific quality of life tool (GOQoL), which can be accessed on the European Group on Graves’ Orbitopathy (EUGOGO) website [53]

  • The adverse effects of intravenous glucocorticoid therapy that have been reported in the literature include: hypertension; hyperglycemias; arrhythmias; acute coronary syndromes; cerebral venous thrombosis; acute liver injury; psychoses and infections

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Summary

Introduction

The Graves’ orbitopathy (GO) is an autoimmune inflammation of the orbital tissues and the most common extra-thyroid symptom of Graves’ disease (GD). 25–50% of patients with GD, the literature shows that subclinical ocular lesions can be observed in the majority of patients with GD when high-quality imaging techniques are used [1,2,3,4,5]. Immunosuppressive treatment is not effective in patients in whom a chronic inflammatory process has caused fibrous changes in the orbits [9]. For this reason, early identification of patients at risk of severe GO is of highest significance, since effective treatment of hyperthyroidism, careful observation of ocular lesions and prompt implementation of appropriate treatment can significantly alleviate the course of the disease

Etiopathogenesis
Risk Factors
Clinical Picture and Diagnosis
Inflammation of the caruncle or plica
1: Present but without redundant tissues
Mild: equivocal or minimal redness
Transient or no diplopia
Moderate or severe soft tissue involvement
Image Evaluation
Modifiable Risk Factors
General Principles of GO Management
Active Moderate-to-Severe GO-Glucocorticotherapy—First Line Treatment
Active Moderate-to-Severe GO—Second Line Treatment
Alternative Treatments with Potential Efficacy
Inactive Moderate-to-Severe GO—Surgical Treatment
Treatment of Sight-Threatening GO
Findings
Conclusions
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