Noninflammatory Thyroid Eye Disease

Abstract

Purpose: Patients presenting with thyroid eye disease (TED) usually follow a well-defined self-remitting course characterized by an active inflammatory phase followed by an inactive fibrotic phase. We present 3 cases where patients presented primarily with signs of progressive fibrosis and no signs of prior active inflammation.

Methods: We reviewed the clinical notes and investigations of 3 patients who presented to our center between January 2015 and August 2017.

Results: All patients included in the study presented primarily with severe, progressive fibrosis without evidence of a previous active inflammatory condition. Although there were no signs of inflammation, each case was progressive, with 2 of the cases developing dysthyroid optic neuropathy that was relatively recalcitrant. We found that these patients were older than the general population of TED patients and that their disease course represents a departure from the common narrative.

Conclusions: This subgroup of TED patients do not conform to the typical inflammatory natural history of TED. We propose that the heterogeneity of the orbital fibroblast pool and their function may be different in this subgroup. Further work will be required to reveal the pathophysiology of this atypical TED process, potentially revealing links between aging and the inflammatory mediators in TED.