Thyroid Eye Disease
Also known as: TED, Graves’ orbitopathy, Graves’ ophthalmopathy, Thyroid-associated orbitopathy
Overview
Thyroid eye disease (TED) is an autoimmune orbital inflammatory disorder most often associated with Graves’ hyperthyroidism, characterized by expansion of orbital fat and extraocular muscles. It produces proptosis, eyelid retraction, restrictive diplopia, and ocular surface exposure, and in its most severe form can threaten vision through dysthyroid optic neuropathy. Management spans an active inflammatory phase and a stable phase, with the insulin-like growth factor-1 receptor inhibitor teprotumumab now a targeted medical option.
- Nature
- Autoimmune orbitopathy
- Association
- Graves’ disease; smoking worsens it
- Hallmarks
- Proptosis, lid retraction, diplopia
- Targeted therapy
- Teprotumumab (IGF-1R inhibitor)
Key clinical points
- TED is most commonly linked to Graves’ hyperthyroidism but can occur in euthyroid or hypothyroid patients.
- Smoking is the strongest modifiable risk factor and markedly worsens severity and treatment response.
- The Clinical Activity Score (CAS) distinguishes the active inflammatory phase from the stable (fibrotic) phase.
- Dysthyroid optic neuropathy is a sight-threatening emergency requiring urgent intervention.
- Teprotumumab reduces proptosis and diplopia; orbital decompression and strabismus/eyelid surgery address the stable phase.
Pathophysiology and risk
TED is driven by autoantibodies against the TSH receptor and the IGF-1 receptor on orbital fibroblasts, triggering glycosaminoglycan deposition, inflammation, and expansion of the orbital fat and extraocular muscles within the bony orbit. This increase in orbital volume produces proptosis and, when the orbital apex is crowded, compressive optic neuropathy.
Risk factors include Graves’ disease, female sex, and—critically—cigarette smoking, which increases both the risk and severity of orbitopathy and reduces the effectiveness of therapy. Rapid correction of thyroid dysfunction and radioactive iodine treatment can also influence disease activity.
Clinical features and evaluation
Patients present with eyelid retraction, proptosis, restrictive strabismus with diplopia, ocular surface exposure, and periorbital edema. The Clinical Activity Score grades inflammatory signs such as pain, redness, and swelling to determine whether disease is active, while severity assessment guides treatment intensity.
Assessment of visual acuity, color vision, pupils, and optic nerve appearance screens for dysthyroid optic neuropathy. Orbital CT or MRI documents muscle enlargement and apical crowding and is essential for surgical planning; thyroid function and antibody testing coordinate care with endocrinology.
Treatment and management
Care is phase-dependent. During the active phase, smoking cessation, ocular surface protection, and selenium supplementation in mild disease are foundational, while moderate-to-severe active disease is treated with intravenous corticosteroids or teprotumumab, an IGF-1 receptor inhibitor shown to reduce proptosis and diplopia. Urgent decompression or steroids are required for dysthyroid optic neuropathy.
In the stable phase, rehabilitative surgery is sequenced as orbital decompression, then strabismus surgery, then eyelid repositioning. A team approach with endocrinology and consistent euthyroid control optimizes outcomes.
Frequently asked questions
- Is thyroid eye disease the same as Graves’ disease?
- They are related but distinct. Graves’ disease is the autoimmune thyroid disorder, while thyroid eye disease is the associated orbital inflammation. TED most often occurs with Graves’ hyperthyroidism but can also arise in euthyroid or hypothyroid patients.
- Why does smoking matter in thyroid eye disease?
- Smoking is the strongest modifiable risk factor for TED. It increases the risk and severity of orbitopathy and reduces the effectiveness of treatments, so smoking cessation is a core part of management.
- What is teprotumumab used for in thyroid eye disease?
- Teprotumumab is an insulin-like growth factor-1 receptor inhibitor used for active moderate-to-severe TED. It has been shown to reduce proptosis and diplopia, offering a targeted medical alternative to corticosteroids.
References & further reading
Related conditions
Dry Eye Disease
Dry eye disease is a multifactorial disease of the ocular surface characterized by loss of tear film homeostasis, accompanied by ocular symptoms, in which tear film instability, hyperosmolarity, inflammation, and neurosensory abnormalities play etiological roles. It is among the most common reasons patients present to eyecare providers. Management is staged and targets the underlying subtype—aqueous-deficient, evaporative, or mixed.
Clinical overviewPediatrics & Binocular VisionStrabismus
Strabismus is a misalignment of the eyes in which they do not point at the same target simultaneously, and it affects both children and adults. It can cause amblyopia and loss of binocular vision in childhood and diplopia in adults. Management ranges from refractive correction and prism to extraocular muscle surgery, depending on the type and cause.
Clinical overview