Chronic ocular graft versus host disease

INTRODUCTION. Chronic ocular graft versus host disease is one of the most common manifestations of chronic GVHD (cGVHD). It has been well recognized from the early allogeneic transplantation experience. Ocular GVHD affects approximately 60–80% of patients with cGVHD and can cause significant decrease in quality of life with high morbidity. The most common form of chronic ocular GVHD is dry eye syndrome (DES) with lacrimal gland dysfunction (reduced aqueous tear production) and keratoconjunctivitis sicca (KCS). Much of the early chronic ocular GVHD literature came from the experience at Johns Hopkins and elsewhere in the early 1980s. Since then, various studies and observations have been made in pathophysiology and in clinical experience in chronic ocular GVHD, in particular in treatment paradigm of organ-specific GVHD in the era of graft versus tumor considerations. National Institute of Health (NIH) Consensus Development Project on Criteria for Clinical Trials in Chronic Graft versus Host Disease published six papers, of which four papers are relevant to the chronic ocular GVHD. This chapter will describe in detail the clinical manifestations, diagnosis, pathophysiology, and management of chronic ocular GVHD and summarize the relevance of the NIH Consensus to ophthalmology. CLINICAL MANIFESTATIONS. Dry eye syndrome/KCS is the hallmark of chronic ocular GVHD. An earlier study noted that over 80% of patients with cGVHD experienced KCS. A later, multicenter retrospective study showed that 69% of patients who experienced KCS posttransplantation had cGVHD.