Clinical features, pathophysiology, and management of thyroid eye disease

Thyroid eye disease (TED) is a complex autoimmune disease commonly associated with auto-immune thyroid disease. It is characterized by progressive inflammation of the orbit and periocular tissues. TED is associated with a number of clinical presentations, and rarely may be vision threatening. TED pathogenesis consists of an autoimmune reaction to TSHR and IGF-1R on orbital fibroblasts resulting in inflammation, adipogenesis, fibrosis, and edema. TED may present in the active, inflammatory phase or the later, quiescent phase. A number of grading systems exist to evaluate TED severity and activity which can be used to dictate disease management. Medical management is generally indicated for active disease, whereas surgical intervention is generally indicated for inactive disease. With recent advances in understanding of TED pathophysiology, medical management has shifted towards new targeted therapies that interfere with the TED inflammatory cascade. In addition, non-FDA approved agents are currently being studied for efficacy in TED patients and may offer insight on better management and treatment of TED. In this paper, we review the current understanding of TED pathophysiology, clinical presentation, and grading. We also discuss current medical and surgical management of TED, as well as new biologics currently under investigation as potential treatment options.