The pathogenesis of ocular surface disease in graft-vs-host disease (GVHD) is multifactorial but has 2 main components: (1) conjunctival inflammation with or without subepithelial fibrosis and (2) severe keratoconjunctivitis sicca (KCS) from lacrimal gland infiltration. KCS occurs in 40%–60% of patients with chronic GVHD (cGVHD).
GVHD is a relatively common complication of allogeneic bone marrow transplantation, which is performed most commonly for hematopoietic malignancies. In this condition, the grafted cells can attack the patient’s tissues, including the skin, gut, lungs, liver, gastrointestinal tract, and eyes. GVHD can be acute or chronic (developing more than 3 months after a bone marrow transplant), with most ocular complications occurring as a manifestation of cGVHD.
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The clinical features of ocular GVHD (eg, KCS, cicatricial conjunctivitis, scleritis) mirror those of other ocular inflammatory conditions associated with autoimmune and collagen-vascular diseases. Conjunctival inflammation in GVHD, which can be severe, may be associated with limbal stem cell deficiency and secondary corneal scarring, although fortunately, this inflammation is rare.
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These patients should be approached in a stepwise, multimodal fashion in consultation with their hematologist and/or oncologist. Aggressive ocular lubrication and punctal occlusion are the mainstays of local therapy. Punctal fibrosis is common and must be monitored closely because it can lead to plug extrusion. Severe filamentary keratitis can be treated with mucolytic agents (acetylcysteine 10%) or bandage contact lenses. Topical cyclosporine or tacrolimus may also be useful in controlling ocular GVHD. Visual disturbances are more commonly due to surface irregularity, but these patients also have a high rate of posterior subcapsular cataracts, which contribute to decreased vision. Autologous serum tears may also be considered. Gas-permeable scleral contact lenses, therapeutic soft contact lenses (Fig 11-14), and the PROSE treatment (discussed earlier in the chapter) can be important management tools for patients with severe ocular surface disease. Keratoprosthesis may be a last-resort option for patients with end-stage ocular surface disease who are not candidates for other conventional corneal procedures. Severe GVHD may require systemic therapy.
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A, Patient with graft-vs-host disease fitted with a therapeutic scleral contact lens. The inferior paracentral cornea demonstrates subepithelial scarring. B, High magnification shows the space between the contact lens and cornea.
(Courtesy of Charles S. Bouchard, MD.)
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.