What are the symptoms and treatment options for thyroid eye disease?

Symptoms and Treatment Options for Thyroid Eye Disease

Thyroid eye disease (TED) is an autoimmune disorder characterized by congestion and inflammation of the orbit and surrounding tissues, presenting with soft tissue congestion, eyelid retraction, exophthalmos, restrictive extraocular myopathy, and optic neuropathy. 1

Common Symptoms of Thyroid Eye Disease

Ocular and Periorbital Symptoms

• Soft tissue congestion with enlargement of the preseptal fat pads 1
• Eyelid retraction (particularly upper eyelid) 1, 2
• Exophthalmos (proptosis) 1, 2
• Periorbital edema and erythema 2
• Conjunctival redness and inflammation 2

Visual Function Symptoms

• Diplopia (double vision) due to restrictive extraocular muscle myopathy 1
• Compensatory head posture (often chin-up position) to minimize double vision 1
• Vision loss from:
  • Corneal exposure (exposure keratopathy) due to incomplete eyelid closure 1, 2
  • Compressive optic neuropathy from crowding at the orbital apex 1, 2
• Elevated intraocular pressure 1

Extraocular Muscle Involvement

• Restrictive myopathy affecting 30-50% of TED patients 1
• Most commonly affects the inferior rectus muscle, followed by the medial rectus 1
• Results in hypotropia with esotropia as the most frequent deviation 1

Risk Factors for Thyroid Eye Disease

• Female predominance (8:1 female to male ratio) 1
• Typical onset in fourth to fifth decade of life 1
• Smoking significantly increases risk and severity 1, 2
• Diseases causing reduced oxygen saturation (e.g., emphysema) 1, 2
• Exposure to ionizing radiation 1
• Radioactive iodine treatment 1
• High anti-thyroid antibody titers 1
• Serum vitamin D deficiency 1
• Prior orbital decompression increases risk of strabismus 1, 2

Treatment Approach for Thyroid Eye Disease

Monitoring and Conservative Management

• Achieve and maintain euthyroid state 3, 4
• Smoking cessation is essential 3, 4
• Ocular lubrication with artificial tears, gels, and ointments for irritation and dryness 3, 4
• Antioxidant therapy with selenium for mild orbital involvement 4
• Regular assessment of visual acuity, color vision, visual fields, and fundus examination 2

Medical Treatment for Active Inflammatory Disease

• Systemic corticosteroids (oral or intravenous) for active inflammation 3, 5
• High-dose intravenous methylprednisolone for vision-threatening disease 5
• External beam radiation for patients with residual inflammation after steroid treatment 3
• Immunomodulatory agents for steroid-resistant cases:
  • Cyclosporin 5
  • Mycophenolate 5
  • Rituximab 5
  • Teprotumumab (IGF-1 receptor antagonist) for moderate-to-severe TED 5, 6
  • Tocilizumab for steroid-resistant cases 6

Surgical Management (Typically in Sequence)

1. Orbital decompression surgery for:

   • Vision-threatening optic neuropathy unresponsive to medical therapy 4, 5
   • Severe proptosis 3, 4

2. Strabismus surgery for:

   • Stable diplopia (after at least 6 months of stability) 3, 4
   • Restoration of binocular vision in primary gaze and reading position 1

3. Eyelid surgery for:

   • Lid retraction with corneal exposure (after at least 6 months of stability) 3, 4
   • Options range from lateral tarsorrhaphy to recession of levator and/or Müller's muscle 3

Management of Vision-Threatening Complications

• Early tarsorrhaphy and orbital decompression for severe exposure keratopathy 5
• Urgent orbital decompression for compressive optic neuropathy unresponsive to medical therapy 4, 5

Clinical Pearls and Pitfalls

• TED can occur in euthyroid or even hypothyroid patients (up to 5% of cases) 6
• Disease presentation is typically bilateral but often asymmetric 2
• TED has active inflammatory and stable fibrotic phases that require different treatment approaches 5
• A small percentage of TED patients have co-existent myasthenia gravis 1
• Quality of life assessment is important in management decisions 6