The management of active thyroid eye disease (TED) can be a challenging therapeutic dilemma. The pathogenic complexity, disease heterogeneity, clinical unpredictability, and ocular morbidity associated with TED necessitate a team approach. EVIDENCE ACQUISITION:: A literature search ending on December 31, 2013, was performed using PubMed (http://www.ncbi.nlm.nih.gov/pubmed) with the following search terms: Graves disease, hyperthyroidism, hypothyroidism, Graves orbitopathy, Graves ophthalmopathy, thyroid eye disease, thyroidectomy, antithyroid medications, radioactive iodine, orbital decompression, orbital radiotherapy (ORT), proptosis, and optic neuropathy. The search included manuscripts in English only. Additional articles and textbooks were retrieved from the reference list of articles that were obtained from the original PubMed literature search. RESULTS:: Corticosteroids, ORT, and orbital decompression have been the mainstay treatment modalities for active TED for more than 50 years. Few randomized controlled studies have systematically evaluated these treatment strategies, and of those trials that have been executed, they are difficult to compare and contrast because of inconsistencies in study design and outcome measures. Newer immunosuppressive and immunomodulating agents are being investigated with anecdotal evidence of improved efficacy compared with traditional treatments. CONCLUSIONS:: All patients with TED must be assessed for disease activity and severity to determine the best course of action. Risk factor modification begins with smoking cessation and attaining euthyroid status. The first-line treatment for moderate-to-severe TED or dysthyroid optic neuropathy is systemic corticosteroids; but often a multimodality approach with the addition of ORT or orbital decompression may be required. The development of novel therapeutic agents against specific immunological targets will improve upon the current treatment armamentarium available to clinicians and patients with TED. Uniformly accepted, scientifically reliable and clinically valid outcome measures integrated into well-designed clinical trials are needed to advance the management of TED to a more evidence-based approach.
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