Subscribe to RSS
DOI: 10.1055/a-2445-6522
Corneal Endothelial Decompensation Necessitating Descemetʼs Membrane Endothelial Keratoplasty (DMEK) in a Series of Patients with Sjögren Syndrome
Hornhautendotheldekompensation mit der Notwendigkeit einer Descemet-Membran-Endothel-Keratoplastik (DMEK) bei Patienten mit Sjögren-Syndrom
Introduction
Sjögrenʼs syndrome (SS) is a chronic multisystemic autoimmune disorder characterized by lymphocytic infiltration of the exocrine glands, including the lacrimal and salivary glands, leading to dry eye disease and dry mouth [1]. SS may occur alone (primary SS) or as part of other chronic inflammatory diseases (secondary SS). According to a meta-analysis, the global prevalence of primary SS is 0.06%, and 90% of the patients are women [2]. Furthermore, it has been found that about 10% of patients with dry eye disease also suffer from SS, and about two-thirds of these patients remain undiagnosed, with an average diagnostic delay of as long as 10 years [2].
The pathomechanisms of dry eye disease in relation to SS have been extensively examined, including corneal epithelial, stromal, and neural abnormalities [3]. Furthermore, changes of retinal vasculature have been reported, such as reduced retinal vessel densities of the superficial vascular complex and retinal vessel rarefication of the deep vascular complex [4]. Unfortunately, the number of studies investigating the impact of SS disease on the endothelium is limited. The corneal endothelium is crucial for maintaining corneal clarity. Studies have shown that corneal endothelial cell loss is evident in patients with dry eye disease [5]. However, the underlying mechanism remains unclear.
Descemet membrane endothelial keratoplasty (DMEK) has become increasingly popular as a treatment for corneal endothelial cell loss and decompensation because of its advantages, such as rapid visual rehabilitation and recovery, relatively low rejection rate, improved refractive stability, and vision-related quality of life [6], [7].
This case series aims to shed light on the potential impact of SS on endothelial decompensation and its successful treatment with DMEK.
Publication History
Received: 04 September 2024
Accepted: 15 October 2024
Article published online:
10 January 2025
© 2024. Thieme. All rights reserved.
Georg Thieme Verlag KG
Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany
-
References
- 1 Sumida T, Azuma N, Moriyama M. et al. Clinical practice guideline for Sjögrenʼs syndrome 2017. Mod Rheumatol 2018; 28: 383-408
- 2 Qin B, Wang J, Yang Z. et al. Epidemiology of primary Sjögrenʼs syndrome: a systematic review and meta-analysis. Ann Rheum Dis 2015; 74: 1983-1989
- 3 Shah R, Amador C, Tormanen K. et al. Systemic diseases and the cornea. Exp Eye Res 2021; 204: 108455
- 4 Wolf E, Wicklein R, Aly L. et al. Optical coherence tomography angiography suggests different retinal pathologies in multiple sclerosis and Sjögrenʼs syndrome. J Neurol 2024; 271: 4610-4619
- 5 Kheirkhah A, Saboo US, Abud TB. et al. Reduced Corneal Endothelial Cell Density in Patients With Dry Eye Disease. Am J Ophthalmol 2015; 159: 1022-1026.e2
- 6 Bayyoud T, Gelisken F, Rohrbach JM. et al. Outcomes after Descemet membrane endothelial keratoplasty over a period of 7 years at a tertiary referral center: endothelial cell density, central corneal thickness, and visual acuity. Graefes Arch Clin Exp Ophthalmol 2021; 259: 1907-1914
- 7 Gellert A, Unterlauft JD, Rehak M. et al. Descemet membrane endothelial keratoplasty (DMEK) improves vision-related quality of life. Graefes Arch Clin Exp Ophthalmol 2022; 260: 3639-3645
- 8 Sekhon AS, He B, Iovieno A. et al. Pathophysiology of Corneal Endothelial Cell Loss in Dry Eye Disease and Other Inflammatory Ocular Disorders. Ocul Immunol Inflamm 2023; 31: 21-31
- 9 Matsumoto Y, Ibrahim OMA, Kojima T. et al. Corneal In Vivo Laser-Scanning Confocal Microscopy Findings in Dry Eye Patients with Sjögrenʼs Syndrome. Diagnostics (Basel) 2020; 10: 497
- 10 Akpek EK, Bunya VY, Saldanha IJ. Sjögrenʼs Syndrome: More Than Just Dry Eye. Cornea 2019; 38: 658-661
- 11 Tuominen IS, Konttinen YT, Vesaluoma MH. et al. Corneal innervation and morphology in primary Sjögrenʼs syndrome. Invest Ophthalmol Vis Sci 2003; 44: 2545-2549
- 12 Kheirkhah A, Satitpitakul V, Hamrah P. et al. Patients With Dry Eye Disease and Low Subbasal Nerve Density Are at High Risk for Accelerated Corneal Endothelial Cell Loss. Cornea 2017; 36: 196-201
- 13 Sanak F, Baenninger P, Iselin K. et al. VEGF-A in Serum and Plasma Rich in Growth Factors (PRGF) Eye Drops. Klin Monbl Augenheilkd 2022; 239: 378-381