Verification of the new grading scale for ocular chronic graft-versus-host disease developed by the German-Austrian-Swiss consensus conference on chronic GVHD

Abstract

The purpose of the study was to validate a recently proposed new grading system for ocular manifestations of chronic graft-versus-host disease (cGVHD). Diagnosis of cGVHD was based on the NIH consensus criteria. In addition, a grading scale was applied, which has been developed by the German-Austrian-Swiss Consensus Conference on Clinical Practice in cGVHD. Sixty-six patients (male n = 46, female n = 20, mean age 48 years) with ocular cGVHD were included. Application of the proposed Consensus Conference grading revealed inflammatory activity in all patients with mild (33 %), moderate (44 %), or severe inflammation (23 %). Clinical scoring by the NIH scoring system showed that 6 % of patients had mild symptoms; 59 % of patients had moderate dry eye symptoms partially affecting activities of daily living, without vision impairment; and 35 % of patients had severe dry eye symptoms significantly affecting daily activities. Clinical characterization and grading by the Consensus Conference grading scale revealed that ocular cGVHD (1) frequently leads to severe ocular surface disease based on impaired function of the lacrimal glands and involvement of cornea, conjunctiva, and lids; (2) is mostly associated with ongoing inflammatory activity; (3) often leads to functional impairment and reduced quality of life; and (4) is associated with an increased risk for severe, sight-threatening complications.

Comparison among different diagnostic criteria for chronic ocular graft-versus-host disease applied with and without pre-transplant ophthalmological examination

“…Currently, tear instability is not included in the criteria in use in hematological patients, despite the increasing evidence on the impairment of the lids and meibomian glands even before and in the early stage following HSCT [12,[21][22][23]. In addition, the recent recommendations from the German-Austrian-Swiss Consensus Conference [13] and the GVHD Consortium for clinical trials [24] specifically targeting ocular GVHD agreed that other objective ophthalmological criteria with more detailed measures might be helpful to document ocular chronic GVHD activity.…”

Update on ocular graft-versus-host disease

“…[ 28 ] [Tables 3 and 4 ] The most commonly used are the NIH and ICCGVHD scoring systems. The other grading criteria described include the German/Austrian/Swiss (GAS) Consensus Conference[ 29 30 ] and Japanese Dry eye score. [ 25 ]…”

“…The extent of ocular surface involvement including the eye and MGs, severity of inflammation, and complications such as corneal perforation, secondary glaucoma, and deterioration of visual acuity are documented. [ 29 30 ]…”

Adeno-Associated Virus Mediated Gene Therapy for Corneal Diseases

“…Broadly, dry eye etiologies can be subdivided into aqueous deficient dry eye, evaporative dry eye, or a combination of both [ 66 ]. A particularly severe form of the aqueous deficient dry eye is associated with chronic graft vs. host disease (GVHD) that develops from chronic inflammation resulting in impaired function of the lacrimal glands and associated blepharitis, conjunctivitis, and keratitis [ 91 ]. Dry eye disease is managed in most patients through antigen avoidance and symptomatic therapy such as artificial tears, antihistamines, and anti-inflammatory medications [ 92 ]; however, these therapies can be often inadequate in managing the underlying symptoms or have side-effects such as eye irritation, eye pain, or allergic reactions.…”