International Chronic Ocular Graft-vs-Host-Disease (GVHD) Consensus Group: proposed diagnostic criteria for chronic GVHD (Part I)

Abstract

The International Chronic Ocular GVHD Consensus Group held 4 working meetings to define new diagnostic metrics for chronic ocular graft-versus-host disease (GVHD). After considering the factors currently used to diagnose chronic ocular GVHD, the Consensus Group identified 4 subjective and objective variables to measure in patients following allogeneic hematopoietic stem cell transplantation (HSCT): OSDI, Schirmer's score without anesthesia, corneal staining, and conjunctival injection. Each variable was scored 0-2 or 0-3, with a maximum composite score of 11. Consideration was also given to the presence or the absence of systemic GVHD. On the basis of their composite score and the presence or absence of systemic GVHD, patients were assigned to one of three diagnostic categories: NO, PROBABLE, or DEFINITE ocular GVHD. New diagnostic criteria for chronic ocular GVHD are presented by the Consensus Group. Validation studies are needed to identify the best combination of the proposed metrics to maximize diagnostic sensitivity and specificity.

Figure 1. Typical ocular surface findings of chronic ocular GVHD.

(A) A representative biomicroscopic image of fluorescein staining (Severity score 3) is shown. Note diffuse punctate epitheliopathy on the cornea of a patient with chronic GVHD related dry eye. Gray lines (Meibolines on upper and lower lid margin) are distorted. (B) Note severe conjunctival injection on damaged ocular surface. (Severity score 2. (C) Note Rose Bengal staining of conjunctiva on severely damaged ocular surface.

Figure 2. Histological findings of lacrimal gland ducts and clinical findings of orifices of meibomian gland and Zeiss gland on eye lid margin.

(A) Typical histological findings of lacrimal gland focusing on medium sized duct and from a patient of chronic GVHD for diagnostic purpose and clinical findings around ductal orfices of meibomian and Zeiss gland in the lid margin of a 21-year-old male patient with chronic GVHD. CD45 immunostaining of inflammatory cells in lacrimal gland from a chronic GVHD patient. An abundant CD45⁺ inflammatory cell infiltration at periductal areas of lacrimal gland chronic GVHD. A 39-year-old female patient at 2 months after onset of cGVHD related dry eye. Original magnification: X630. (B) Note total obstruction of meibomian gland and Zeis gland orifices and severely fibrotic tarsal conjunctiva. Upper eye lid, right eye. A 21-year-old male suffering from chronic GVHD.

Figure 3. Slitlamp micrograph of grading scale for corneal fluorescein staining.

Grading scale of corneal fluorescein staining score ranged from 0 to 3 points (Grade 0 = no staining, Grade 1 = minimal staining, Grade 2 = mild/moderate staining, Grade 3 = severe staining).

Figure 4. Slit lamp micrograph of grading scale for conjunctival injection.

Grading scale of conjunctival injection score ranged from 0 to 2 points (Grade 0 = none, Grade 1 = mild/moderate injection, Grade 2 = severe injection).