Thyroid eye disease.

Thyroid eye disease (TED), also referred to Graves' ophthalmopathy, is a disfiguring and vision-threatening autoimmune disease of the retroocular tissues occurring in patients with Graves' disease (GD). Among the extrathyroidal manifestations of GD, TED is the most frequent, being observed in roughly 30-50% of patients. In the immunopathogenesis of TED, the TSH-R/IGF-1R complex has a determinant role. TED is commonly bilateral, and during its active phase it presents with ocular redness, eyelid edema, photophobia, proptosis, and diplopia. In the most severe cases, the involvement of the optic nerve (dysthyroid optic neuropathy) may endanger vision and requires urgent treatment. Recommendations for the management of TED encompass three aspects: general measures, thyroid treatment, and eye treatment. The management of TED consists in control of hyperthyroidism, local therapies, intravenous pulses of methylprednisolone, and immunosuppressive agents. Recently, the deeper knowledge of TED pathophysiology has led to new potential therapeutic targets, and new biological drugs, such as teprotumumab, that is able to ameliorate clinical activity score and proptosis in these patients. Additional agents are currently under investigation, with promising results that suggest potential advances in the treatment of this disease. The aim of this review is to provide an updated outline of TED, in terms of immunopathogenesis, diagnosis, clinical management and current innovative drugs.