How are thyroid eye disease symptoms managed?

Management of Thyroid Eye Disease Symptoms

Thyroid eye disease symptoms should be managed based on disease activity and severity, with careful monitoring for vision-threatening complications (optic neuropathy and exposure keratopathy), symptomatic treatment with ocular lubricants and beta-blockers for mild disease, and escalation to corticosteroids or teprotumumab for moderate-to-severe active disease, followed by surgical rehabilitation once the disease becomes inactive. 1, 2, 3

Initial Assessment and Risk Stratification

Identify Vision-Threatening Features Requiring Urgent Intervention

• Monitor closely for compressive optic neuropathy at the orbital apex and severe exposure keratopathy from eyelid retraction with exophthalmos—these require immediate referral to an orbital specialist. 1, 2
• Screen for optic neuropathy with visual acuity, color vision testing, visual fields, pupillary examination, and fundus examination at baseline and throughout treatment. 2
• Measure and document proptosis by exophthalmometry to establish baseline and track response. 2
• Assess for elevated intraocular pressure, which can occur as a complication. 1, 2

Determine Disease Activity Using Clinical Activity Score (CAS)

• A CAS ≥3 indicates active inflammation requiring medical treatment, while CAS <3 suggests inactive disease where surgical rehabilitation may be considered. 2, 4
• The CAS ranges from 0-7, with scores ≥5 combined with documented symptoms demonstrating active moderate-to-severe disease. 2
• Document diplopia using standardized grading to quantify baseline severity and track treatment response. 2

Assess Disease Severity

• Mild disease presents with soft tissue congestion, enlargement of preseptal fat pads, and eyelid retraction without significant functional impairment. 1, 2
• Moderate-to-severe disease involves exophthalmos, restrictive extraocular myopathy causing diplopia, and compensatory head posture (often chin-up position). 1, 2
• Very severe disease is characterized by compressive optic neuropathy or severe exposure keratopathy threatening vision. 2

Treatment Algorithm Based on Activity and Severity

Mild Disease (Any Activity Level)

• Ocular lubricants are almost always needed to combat exposure related to eyelid retraction and proptosis. 3
• Beta-blockers (atenolol or propranolol) provide symptomatic relief of associated hyperthyroid symptoms including tachycardia, tremor, and anxiety. 3
• Selenium supplementation (100 mcg twice daily) may reduce inflammatory symptoms in patients with milder thyroid eye disease. 3
• Fresnel or ground-in prisms can provide temporary relief from diplopia while awaiting definitive treatment. 3

Moderate-to-Severe Active Disease (CAS ≥3)

• High-dose intravenous methylprednisolone is the first-line treatment, with reported response rates of approximately 77% compared to 63% for oral glucocorticoids. 4, 5
• Consider adding antiproliferative agents like mycophenolate sodium to prevent deterioration after steroid cessation. 6
• Teprotumumab (IGF-1R inhibitor) substantially reduces proptosis and Clinical Activity Score in patients with active disease. 2, 3, 6
  • Critical caveat: Assess for pre-existing hearing loss before treatment and counsel patients about hearing risk, as hearing changes may be permanent in rare cases. 2
  • Establish baseline audiometry and monitor throughout treatment. 2
• Cyclosporin combined with oral glucocorticoids is more effective than glucocorticoids alone in moderately severe disease, though both drugs have considerable side effects requiring close monitoring. 5
• Orbital radiotherapy may be considered as adjunctive therapy in active disease. 4

Sight-Threatening Disease (Compressive Optic Neuropathy or Severe Exposure)

• Urgent orbital decompression is required for compressive optic neuropathy or severe exposure keratopathy threatening vision. 2
• Use high-dose intravenous methylprednisolone therapy with early consideration of tarsorrhaphy for severe exposure. 4
• Management of sight-threatening disease represents the absolute priority. 7

Inactive Disease (CAS <3) with Residual Deformity

• Surgical rehabilitation should follow a specific sequence: orbital decompression first (if needed), followed by strabismus surgery, then eyelid surgery. 1, 3
• The goal of strabismus surgery is to re-establish single binocular vision in primary gaze and reading position with a substantial usable field of single binocular vision. 1
• If orbital decompression is indicated, delay strabismus repair until after decompression, as prior orbital decompression increases the risk of strabismus. 1, 3

Coordination with Thyroid Management

Achieve and Maintain Euthyroidism

• Treatment of thyroid dysfunction is the widely accepted first therapeutic measure, as systematic management by both endocrinologist and ophthalmologist gives the best results for quality of life and vision. 4, 5
• Antithyroid drugs and thyroidectomy do not change the natural course of TED, while radioactive iodine carries a small but documented risk of TED progression. 4, 7
• In moderate-to-severe active TED, antithyroid drugs are preferred, but thyroidectomy is a valid option; radioactive iodine is generally avoided. 7
• If radioactive iodine must be used in patients with mild TED of recent onset, steroid prophylaxis with a short course of low-dose prednisone is strongly recommended. 7

Key Risk Factors to Address

Modifiable Risk Factors

• Smoking significantly increases both the risk and severity of thyroid eye disease and must be addressed aggressively. 1, 2
• Diseases causing reduced oxygen saturation (such as emphysema) worsen orbitopathy. 1, 2
• High anti-thyroid antibody titers and serum vitamin D deficiency are independent risk factors. 1, 2

Monitor for Coexistent Conditions

• The incidence of myasthenia gravis is increased in patients with TED, requiring assessment for coexistence. 1, 2
• Between 30-50% of patients with TED develop restrictive myopathy, most commonly affecting the inferior rectus muscle, followed by the medial rectus. 1, 2

Common Pitfalls to Avoid

• Failing to recognize and urgently treat compressive optic neuropathy or severe exposure keratopathy, which can result in permanent vision loss. 1, 2
• Not monitoring disease activity closely enough with serial CAS assessments to guide treatment escalation or de-escalation. 2, 4
• Using radioactive iodine without steroid prophylaxis in patients with active or recent-onset TED. 7
• Performing strabismus surgery before orbital decompression when both are needed, as decompression can alter ocular alignment. 1, 3
• Initiating surgical rehabilitation during the active inflammatory phase rather than waiting for disease inactivity. 4