Pathophysiology of Autoimmune Orbital Diseases and Target Therapy for Orbital Inflammatory and Neoplastic Diseases Publisher



Pakdel F¹ ; Sullivan TJ² ; Pirmarzdashti N³ Show Affiliations
Source: Translational Autoimmunity: Autoimmune Diseases in Different Organs Published:2022

Abstract

Autoimmune orbital diseases are the common cause of orbital inflammatory conditions. They include thyroid eye disease (TED), idiopathic orbital inflammatory syndromes (IOIS), and specific orbital inflammatory diseases. Recent studies showed the key role of TSH receptor antibodies and insulin-like growth factor-1 (IGF-1) as the main autoantigens in TED. Treatment of active TED with monoclonal anti-IGFR-1 antibody has shown promising results. Several studies highlighted the role of anti-CD20, antitumor necrosis factor-α (TNF-α), anti-IL-6 monoclonal antibodies in the treatment of TED and IOIS. Thus, targeted therapy in autoimmune orbital inflammatory diseases is now considered as a promising treatment. Understanding the pivotal role of the Hedgehog pathway and immune checkpoints established effective targeted therapies in several orbital and periocular malignancies. Currently, Hedgehog and immune checkpoint inhibitors have received FDA approval for locally advanced basal cell carcinoma and squamous cell carcinomas, respectively. © 2022 Elsevier Inc. All rights reserved.