Mary E.D. Flowers, Alicia Rovó, Gerhard C. Hildebrandt, Kristina Teär Fahnehjelm, Robert Peter Gale, Catherine J. Lee, Mahmoud Aljurf, Pinki Prasad, Baldeep Wirk, Yoshihiro Inamoto, Ami J. Shah, Jason Law, Minoo Battiwalla, Amir Steinberg, Bronwen E. Shaw, Igor Petriček, Linda J. Burns, Natalie S. Callander, Grzegorz W. Basak, Seth J. Rotz, Rafael F. Duarte, Hassan B. Alkhateeb, Jean A. Yared, Ibrahim Ahmed, Ravi Pingali, Amer Beitinjaneh, André Tichelli, Raquel M. Schears, Olaf Penack, Erich Horn, Rammurti T. Kamble, Aisha Ahmed, Ann A. Jakubowski, Saurabh Chhabra, Nosha Farhadfar, Nuria Valdés-Sanz, Bipin N. Savani, Siddhartha Ganguly, Neel S. Bhatt, Aditya Shreenivas, Dave Buchbinder, Peiman Hematti, Asim Ali, Vaibhav Agrawal, Drazen Pulanic, Zachariah DeFilipp, Khalid Tabbara, Shahrukh K. Hashmi, Sunita Nathan, and Michael Byrne
Ocular graft-versus-host disease (GVHD) occurs in more than one-half of patients who develop chronic GVHD after allogeneic hematopoietic cell transplantation (HCT), causing prolonged morbidity that affects activities of daily living and quality of life. Here we provide an expert review of ocular GVHD in a collaboration between transplantation physicians and ophthalmologists through the Late Effects and Quality of Life Working Committee of the Center for International Blood and Marrow Transplant Research and the Transplant Complications Working Party of the European Society of Blood and Marrow Transplantation. Recent updates in ocular GVHD regarding pathophysiology, preclinical models, risk factors, prevention, screening, diagnosis, response criteria, evaluation measures, and treatment are discussed. Ocular GVHD involves at least 3 biological processes: lacrimal gland dysfunction, meibomian gland dysfunction, and corneoconjunctival inflammation. Preclinical models have identified several novel pathogenic mechanisms, including the renin angiotensin system and endoplasmic reticulum stress signaling, which can be targeted by therapeutic agents. Numerous studies have identified reliable tests for establishing diagnosis and response assessment of ocular GVHD. The efficacy of systemic and topical treatment for ocular GVHD is summarized. It is important that all health professionals caring for HCT recipients have adequate knowledge of ocular GVHD to provide optimal care.