A plethora of ocular surface manifestations in a multidisciplinary ocular graft-versus-host disease unit

Abstract

To describe the experience in a recently created ocular graft-versus-host disease unit in a tertiary hospital and to detail ocular surface features and complications after allogeneic hematopoietic stem cell transplantation (allo-HSCT). This retrospective study included all patients who underwent allo-HSCT, with or without chronic GVHD and were being monitored in the Hematopoietic Stem Cell Transplantation Unit in the UNICAMP Clinical Hospital (Campinas, Sao Paulo, Brazil) from 2015 to 2020. Patients were concomitantly evaluated by hematology and ophthalmology teams of the Ocular GVHD Unit. Hematologists performed a comprehensive systemic evaluation searching and grading mouth, skin, lungs, gastrointestinal tract, liver and genitalia GVHD. While ophthalmologists evaluated ocular symptoms through specific questionnaire (Ocular Surface Disease Index—OSDI) and a protocol of distinct ocular surface parameters for dry eye disease (1) and ocular complications, which encompassed meniscometry, non-invasive tear break-up time (NITBUT) measurement, conjunctival hyperemia quantification, meibography, fluorescein and lissamine staining and Schirmer’s test. Patients were diagnosed with chronic GVHD using the National Institutes of Health (NIH) Consensus Criteria for Chronic Graft-versus-Host Disease. The International Chronic Ocular GVHD Consensus Group (ICOGCG) score was obtained at the onset of ocular disease presentation or afterwards. A total of 82 patients underwent allo-HSCT (97.6% full matched and 2.4% haploidentical), mainly for cases of leukemia and 73.2% had chronic GVHD. Mean onset time for chronic GVHD was 232 ± 7.75 days. The mouth, skin, and eyes were the main organs involved (63%, 50%, and 48%, respectively). Symptom scores and all ocular surface parameters differ in patients with and without chronic GVHD and along different timepoints of the follow-up. Ocular complications mostly involved were severe DED and meibomian gland dysfunction, conjunctival scarring, cataract and infections resulting in keratitis and corneal perforation. As therapeutic strategies, 73% patients received preservative-free lubricants, 27% autologous serum, 48% topical steroids, 27% oral tetracycline derivatives, 22% mucolytic eye drops and 3 patients needed bandage contact lens. Ocular GVHD is a complex and challenging disease with varied manifestations, resulting in a broad range of ocular test endpoints, and inconsistent treatment responses. The main ocular presentations were dry eye, meibomian gland dysfunction and cataracts. The therapeutic approach often involves topical steroids and autologous serum tears. It is important to monitor these patients closely, so the ocular GVHD Unit may improve the care, providing prompt identification of ocular manifestations and faster treatment of complications.

Lessons Learned From Ocular Graft versus Host Disease: An Ocular Surface Inflammatory Disease of Known Time of Onset

“…35 Meibography images obtained at baseline and 1-year post-HSCT revealed an 80% meibomian gland dropout, with 19% of cases with persistent dropout despite treatment. 37 In another study, Trindade et al 38 reported a significant difference in the prevalence of conjunctival scarring, MGD, corneal opacity, and corneal neovascularization in post-HSCT patients with and without oGVHD.…”

Augenbeteiligung bei Graft-versus-Host-Erkrankung

“…1 b) bis hin zu einem Narbenentropium kommen [10 -13]. Im Rahmen der Keratoconjunctivitis siccaund insbesondere der Entzündung der Augenoberfläche durch aktivierte T-Zellen und dendritische Zellenfinden sich oft auch eine Chemose und lidkantenparallele konjunktivale Falten (LIPCOF) [13,14]. Das Spektrum der Hornhautveränderungen reicht von einer Keratopathia punctata superficialis (▶ Abb.…”

“…1 b) right up to a scar entropion [10 -13]. In the context of keratoconjunctivitis siccaand particularly inflammation of the ocular surface caused by activated T cells and dendritic cellschemosis and lid-parallel conjunctival folds (LIPCOF) are also often found [13,14]. The spectrum of corneal changes ranges from superficial punctate keratopathy (▶ Fig.…”

Challenges and concepts in the diagnosis and management of ocular graft-versus-host disease

“…Increasing the lubrification may also reduce the concentrations of proinflammatory cytokines on the ocular surface ( 5 , 119 ). Mucolytic eye drops, i.e., topical N-acetylcysteine 5%–10%, should be considered in filamentary keratitis, which is often observed in eyes with a very dry ocular surface ( 5 , 120 ).…”