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

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.

Prevalence and risk factors associated with development of ocular GVHD defined by NIH consensus criteria

“…More recently, the German-Austrian-Swiss group has outlined consensus criteria for diagnosis and management of ocular GVHD, which remains to be validated, but realizes the limitation of the NIH criteria. 18 The NIH consensus criteria have developed a scoring system for various clinical domains of chronic GVHD. Each domain is scored from 0 to 3, reflecting no involvement to severe manifestations in the clinical domain ( Table 2).…”

Meibography and meibomian gland measurements in ocular graft-versus-host disease

“…The most common form is chronic ocular GvHD, which demonstrates symptoms such as irritation, photophobia, redness, itchiness, foreign body sensation, burning, partial excessive tearing and blurred vision. 1,2 Until now, there are no specific symptoms, clinical signs or biomarkers available that would allow for a definite diagnosis of ocular GvHD. This disease mimics other immunologically mediated inflammatory diseases such as Sjögren or nonSjögren-associated dry-eye disease and blepharitis, which makes it difficult to definitively diagnose ocular GvHD, particularly in milder cases.…”

“…Further observed changes in ocular GvHD include changes in the lid margin with blepharitis and, in particular, alterations in the meibomian glands. 1,3 Recently, much attention has been paid to meibomian glands, which are sebaceous glands located in the lower and upper tarsus of the eyelid. Each gland consists of multiple acini connected to a long common central duct whose orifice ends at the posterior lid margin.…”

Incidence and risk factors for ocular GVHD after allogeneic hematopoietic stem cell transplantation

“…The manifestations of chronic GVHD may be restricted to a single organ or tissue or may be widespread. 3,4 Ocular manifestations of GVHD are known to occur in 60-90% of patients with chronic GVHD, and these manifestations primarily affect the cornea, conjunctiva, lacrimal glands, eyelid and meibomian glands. 5,6 Dry eye disease (DED), or keratoconjunctivitis sicca (KCS), is the most common manifestation of ocular GVHD and induces ocular discomfort, decreases patients' quality of life and can even result in loss of vision.…”