A 67-year-old woman, a nonsmoker, presented with conjunctival injection, lid swelling, and proptosis for 8 weeks (Figure 1A). She was treated initially for allergic conjunctivitis and then bacterial conjunctivitis with no improvement. Two months later she was diagnosed with thyroid eye disease (TED) and mild asymptomatic hyperthyroidism, with a high thyroid-stimulating immunoglobulin (TSI) level of 419% (normal < 130%). She was treated with oral and then intravenous (IV) steroids (methylprednisolone 500 mg IV weekly × 6 weeks followed by 250 mg IV weekly × 6 weeks). She received methimazole with restoration of euthyroidism. She was counseled to avoid tobacco exposure and to take selenium 100 mcg twice a day. On week 4 of IV steroids she presented with worsening vision and color vision and diplopia (Figure 1B). Her visual acuity was 20/80 OD and 20/60 OS, she had a right relative afferent pupillary defect (APD), and on Ishihara plate assessment scored 1 out of 14 in the right eye and 14 out of 14 in the left. She was diagnosed with compressive optic neuropathy (CON) and underwent urgent evaluation by ophthalmology.
Case 1. A, Initial presentation of active moderate to severe thyroid eye disease with conjunctival injection, lid swelling and erythema, and proptosis. B, After high-dose oral steroids and 4 weeks of weekly intravenous (IV) 500-mg solumedrol, the patient presented with right compressive optic neuropathy and restrictive strabismus. Note the asymmetric pupil reflex with right hypotropia (inferior displacement of right eye), right upper lid retraction, worsening conjunctival injection, chemosis, eyelid erythema, and edema. C, Status post high-dose oral and IV steroids, orbital radiation, bilateral orbital decompression, strabismus surgery, and retraction repair.
A 55-year-old woman with no prior medical care presented to the emergency room with 3 days of right eye blurry vision, pain, and eyelid swelling. Her vision was 20/200 OD and 20/20 OS with loss of color vision (0/13 OD, 5/13 OS), a right relative APD, and significant bilateral restriction of eye movement. She had proptosis, eyelid retraction, lagophthalmos, periorbital edema and erythema, conjunctival injection, and chemosis (Figure 2A). Laboratory data revealed mild hyperthyroidism and an elevated TSI of 277% (normal < 140%). She was diagnosed with active sight-threatening TED and treated for right CON with high-dose IV steroids (1 gm solumedrol daily × 3) with little response. She underwent emergent right orbital decompression, with improvement in visual acuity to 20/25 OD, followed by IV solumedrol 500 mg weekly. After the fifth week, she developed progressive diplopia, left eye pain, redness, and proptosis (Figure 2B). Her vision worsened to 20/100 OS with reduced color vision of the left eye. She had steroid-resistant TED and developed significant confusion, tremors, and insomnia; therefore, steroids were discontinued.
Case 2. A, Initial presentation to the emergency room with loss of vision and bilateral eyelid swelling, chemosis, injection, proptosis, and restrictive strabismus. B, After high-dose intravenous (IV) steroids and right orbital decompression for compressive optic neuropathy (CON) and 5 weeks of weekly IV 500-mg solumedrol, the patient's photo shows persistent left proptosis, eyelid swelling, and injection, while her disease has progressed to left CON. C, After 8 infusions of IV teprotumumab, her active inflammatory signs have resolved, though she still has left relative proptosis compared to the right decompressed eye.
J Endo Soc. 2021;5(5) © 2021 Endocrine Society