What is the management of Graves' orbitopathy (thyroid eye disease)?

From the Guidelines

Management of thyroid eye disease should prioritize a multidisciplinary approach, with initial treatment focusing on achieving euthyroidism and utilizing teprotumumab, an insulin-like growth factor-1 receptor inhibitor, for moderate-to-severe active disease, as it has shown significant efficacy in reducing proptosis and diplopia 1.

Key Considerations

• Conservative measures such as artificial tears, lubricating ointments, and elevation of the head while sleeping can help reduce periorbital edema in mild cases.
• Selenium supplementation may help reduce disease progression in mild cases with selenium deficiency 1.
• Oral glucocorticoids like prednisone can be used for moderate-to-severe active disease, with intravenous methylprednisolone pulse therapy considered for cases resistant to oral steroids.
• Teprotumumab has been shown to improve subjective diplopia scores in both active and chronic thyroid eye disease and reduce the need for strabismus surgery 1.

Surgical Interventions

• Surgery is required in most patients with persistent diplopia in primary or reading positions of gaze, usually undertaken when the inflammatory stage has subsided and the angle of deviation has been stable for at least 6 months 1.
• Recessions of the restricted eye muscles are the mainstay of surgical correction, with recession of the inferior rectus muscle frequently complicated by consecutive hypertropia 1.
• A systematic approach appraising preoperative and intraoperative forced ductions of all extraocular muscles and attention to preoperative and intraoperative ocular torsion can minimize the risk of an undesired postoperative result 1.

Additional Recommendations

• Smoking cessation is essential as smoking worsens disease progression and reduces treatment efficacy.
• Regular monitoring of disease activity using clinical activity scores helps guide treatment decisions and timing of surgical interventions.
• Orbital radiation and surgical interventions like orbital decompression are typically reserved for inactive disease or sight-threatening complications 1.

From the Research

Management of Thyroid Eye Disease

• The management of thyroid eye disease (TED) can be challenging and requires a team approach, with corticosteroids, orbital radiotherapy (ORT), and orbital decompression being the mainstay treatment modalities for active TED 2.
• Methotrexate has been shown to be an effective steroid-sparing agent in a subset of patients with TED, allowing for the discontinuation of prednisone therapy and improving visual acuity and ocular motility 3.
• The active inflammatory phase of TED is mediated by the innate immune system, and management is aimed at aborting this self-limited period of autoimmune activity, with immunosuppression reducing the long-term sequelae of acute inflammation 4.
• Novel therapies, including biologic immunosuppressive agents such as etanercept, adalimumab, and tocilizumab, have been shown to be effective in reducing inflammatory signs and preventing relapse of the disease, with teprotumumab controlling disease activity and preventing progression 5.
• A consensus statement by the American Thyroid Association and the European Thyroid Association provides guidance on the management of TED, emphasizing the importance of endocrinologists in diagnosis, initial management, and selection of patients who require referral to specialist care 6.

Treatment Options

• Corticosteroids are the primary medical therapy for TED, with oral and intravenous treatment regimens having different efficacy and side-effect profiles 5.
• Immunosuppressive agents, such as methotrexate and mycophenolate, can be used as steroid-sparing medications, while biologic agents like etanercept, adalimumab, and tocilizumab may provide a deep and long-standing block of inflammatory activity in TED 5.
• Orbital decompression and ORT may be required in some cases, with elective surgery often necessary following moderate TED to address proptosis, diplopia, lid retraction, or to debulk the eyelid 2, 4.

Patient Assessment and Risk Factor Modification

• Patients with TED must be assessed for disease activity and severity to determine the best course of action, with risk factor modification beginning with smoking cessation and attaining euthyroid status 2, 4.
• The first-line treatment for moderate-to-severe TED or dysthyroid optic neuropathy is systemic corticosteroids, with a multimodality approach often required, including ORT or orbital decompression 2.