Insights Into the Pathophysiology of Superior Limbic Keratoconjunctivitis in Chronic Ocular Graft-Versus-Host Disease

Superior limbic keratoconjunctivitis (SLK) is a chronic inflammatory ocular surface disorder affecting the superior conjunctiva and limbus. Its pathogenesis entails a vicious cycle of microtrauma and inflammation. Besides its well-known association with thyroid disorders, SLK has also been associated with systemic conditions involving severe ocular surface disease. One condition that can secondarily develop SLK is ocular graft-versus-host disease (oGVHD). Ocular graft-versus-host disease, a complication of allogeneic hematopoietic stem-cell transplantation, is a pan-ocular surface disease that can lead to severe ocular complications and vision loss. Importantly, the occurrence of SLK in the setting of oGVHD is a marker of an augmented ocular surface distress that requires immediate aggressive management to prevent irreversible ocular surface damage, notably limbal stem-cell deficiency. Treatment of SLK in oGVHD includes aggressive lubrication, anti-inflammatory drops, serum/plasma drops, and large diameter contact lenses. In particular, scleral contact lenses are currently the most effective long-term treatment for SLK in oGVHD and can significantly reduce the risk of irreversible loss of vision. Of note, conjunctival resection or destruction (e.g., cauterization) is not recommended for treatment of SLK in ocular graft-versus-host disease. This review examines the pathophysiology and treatment modalities for SLK in oGVHD aimed at improving long-term outcomes.