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Christiane Blecha, Daniel Wolff, David Maerker, Horst Helbig, Tina Dietrich-Ntoukas; Characterization of patients with ocular chronic graft-versus-host disease and evaluation of a new grading scale. Invest. Ophthalmol. Vis. Sci. 2013;54(15):5420.
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Ocular chronic graft-versus-host disease (cGVHD) is one of the most frequent complications after allogeneic hematopoietic stem cell transplantation (alloHSCT). It often leads to reduced quality of life due to severe ocular discomfort or even vision loss in severe cases. Purpose of the study was to characterize patients with ocular cGVHD.
All patients with ocular symptoms in the HSCT clinic were examined ophthalmologically. Complete ocular examination was performed and subjective symptoms of keratoconjunctivitis sicca (KCS) assessed. Diagnosis of cGVHD was based on the NIH consensus criteria for cGVHD. A new grading scale was applied, which was proposed by the Consensus Conference on Clinical Practice in cGVHD (Dietrich-Ntoukas et al., 2011). Statistical analyses were performed using SPSS Version 20 (IBM SPSS, Armonk, NY, USA).
35 patients (male n=25, female n=10, mean age 50 years) with ocular cGVHD were included. 95% of the patients suffered from a cGVHD of other organs, mostly of oral mucosa (62%) and the skin (62%). 94% of the patients showed blepharitis, 91% conjunctival manifestations. 71% suffered from corneal involvement (punctate keratopathy (97%), filamentary keratitis (26%), corneal scarring (23%), corneal erosion (17%), corneal ulcer (12%)). The new grading scale revealed inflammatory activity in all patients (mild (26%), moderate (43%) or severe (31%)). Results of the NIH scoring system: 0% score 0 (no symptoms); 0% score 1 (mild symptoms of dry eye or asymptomatic signs of KCS); 43% score 2 (moderate dry eye symptoms partially affecting activities of daily living, requiring drops > 3x per day or punctual plugs, without vision impairment); 57% score 3 (severe dry eye symptoms significantly affecting daily activities or loss of vision because of KCS). All 35 patients used artificial tears, 29 used topical cyclosporine and 7 autologous serum eye drops.
Patients with ocular cGVHD frequently have severe ocular surface disease based on impaired function of the lacrimal gland but also of the conjunctiva and the lids, mostly associated with ongoing inflammatory activity. The latter is currently not covered by the NIH grading. Ophthalmological assessment of patients after alloHSCT including lids, conjunctiva and inflammatory activity is necessary to confirm the diagnosis, to optimize the therapy and to prevent irreversible complications.
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