Diagnosis and treatment of ocular chronic graft-versus-host disease: report from the German-Austrian-Swiss Consensus Conference on Clinical Practice in chronic GVHD

Abstract

Purpose: Ocular chronic graft-versus-host disease (cGVHD) is one of the most frequent long-term complications after hematopoietic stem cell transplantation and is often associated with significant morbidity and reduced quality of life.

Methods: The German/Austrian/Swiss Consensus Conference on Clinical Practice in cGVHD aimed to summarize the currently available evidence for diagnosis and (topical) treatment and to summarize different treatment modalities of ocular cGVHD. The presented consensus was based on a review of published evidence and a survey on the current clinical practice including transplant centers from Germany, Austria, and Switzerland.

Results: Ocular cGVHD often affects the lacrimal glands, the conjunctiva, the lids (including meibomian glands), and the cornea but can also involve other parts of the eye such as the sclera. Up to now, there have been no pathognomonic diagnostic features identified. The main therapeutic aim in the management of ocular cGVHD is the treatment of inflammation and dryness to relieve patients' symptoms and to maintain ocular integrity and function. Therapy should be chosen in the context of the patient's overall condition, systemic immunosuppressive therapy, symptoms, ocular surface integrity, and inflammatory activity. The consensus conference proposed new grading criteria and diagnostic recommendations for general monitoring of patients with graft-versus-host-disease for use in clinical practice.

Conclusion: The evidence levels for diagnosis and treatment of ocular cGVHD are low, and most of the treatment options are based on empirical knowledge. Topical immunosuppression, for example, with cyclosporine, represents a promising strategy to reduce inflammation and dryness in ocular cGVHD. Further clinical trials are necessary to elucidate risk factors for eye manifestation, complications, and visual loss and to evaluate staging criteria and diagnostic and therapeutic measures for ocular cGVHD.

Trial registration: ClinicalTrials.gov NCT00755040.