Update on Graves Disease

Advances in Treatment of Mild, Moderate and Severe Thyroid Eye Disease

Diego Strianese


Curr Opin Ophthalmol. 2017;28(5):505-513. 

In This Article

Severe Disease

First-line treatment of CON is high-dose i.v. steroid therapy.[14] If this treatment fails, or results are inadequate, further therapy is required. At this point, the treatments available include surgical decompression, RT or biologic immunosuppressive agents. In order to plan surgical decompression, we suggest careful evaluation of the clinical appearance and imaging features, taking into account the reported CT or MRI-based methods.[49,50] Patients presenting with a decrease in vision without significant proptosis usually show apical compression on imaging with evidence of a congested and crowded orbit. These patients should undergo intensive medical treatment with RT or immunosuppressive agents. Such cases require an aggressive reduction of the soft tissue edema and swelling due to the inflammatory process, to decrease the high intraorbital pressure due to a tight orbital septum anteriorly, limiting the forward displacement of the eye.[51] Patients presenting with visual deterioration, severe proptosis and signs of a crowded apex syndrome on CT scan and evidence of a stretched optic nerve need an adequate orbital decompression to address the proptosis and the threatened optic nerve. These cases may require multiple surgical interventions.[36] Traditionally, RT has been used in association with steroid and decompression to treat CON and the rate of success is variable.[17] If the RT is chosen as adjuvant therapy, we strongly recommend to combine this therapy with oral steroid at the dose of 0.5 gm of prednisolone per kilogram of weight, tapered over 4–8 weeks to prevent the risk of exacerbation of the inflammation which may occur at the beginning of the RT. The use of RTX for CON is controversial and seems to carry the risk of worsening or causing an onset of CON.[27] Recently, the use of infliximab as well as tolicizumab appears to be effective in treating steroid and orbital decompression-resistant patients with severe TED.[44,45] We also have personal experience in using such therapy in cases after failure of steroid and orbital decompression with a complete resolution after three doses per month for 3 months. Corneal breakdown due to eyelid retraction, proptosis and significant disease activity is sight threatening and may require urgent treatment to allow eyelid closure. Steroid, RT and other immunosuppressive agents have not been found to be effective in short term on proptosis and eyelid retraction. Tarsoraphy or urgent decompression is advisable; alternatively, botulinum toxin injection seems to be effective even in this acute phase[7]