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The Pathophysiology of Thyroid Eye Disease

Shan, Shannon J. C. MD, MSc; Douglas, Raymond S. MD, PhD

Section Editor(s): Biousse, Valérie MD; Galetta, Steven MD

Journal of Neuro-Ophthalmology: June 2014 - Volume 34 - Issue 2 - p 177–185
doi: 10.1097/WNO.0000000000000132
State-of-the-Art Review

Abstract: The pathophysiology of thyroid eye disease (TED) is complex and incompletely understood. Orbital fibroblasts (OFs) seem to be the key effector cells that are responsible for the characteristic soft tissue enlargement seen in TED. They express potentially pathogenic autoantigens, such as thyrotropin receptor and insulin-like growth factor-1 receptor. An intricate interplay between these autoantigens and the autoantibodies found in Graves disease may lead to the activation of OFs, which then leads to increased hyaluronan production, proinflammatory cytokine synthesis, and enhanced differentiation into either myofibroblasts or adipocytes. Some of the OFs in TED patients seem to be derived from infiltrating fibrocytes. These cells originate from the bone marrow and exhibit both fibroblast and myeloid phenotype. In the TED orbit, they may mediate the orbital expansion and inflammatory infiltration. Last, lymphocytes and cytokines are intimately involved in the initiation, amplification, and maintenance of the autoimmune process in TED.

Wilmer Eye Institute (SJCS), The Johns Hopkins University School of Medicine, Baltimore, Maryland; and Kellogg Eye Center (RSD), University of Michigan, Ann Arbor, Michigan.

Address correspondence to Raymond S. Douglas, MD, PhD, Kellogg Eye Center, University of Michigan, 1000 Wall Street, Ann Arbor, MI 48105; E-mail: raydougl@med.umich.edu

The authors report no conflicts of interest.

© 2014 by North American Neuro-Ophthalmology Society