Corneal Endothelial Decompensation Necessitating Descemetʼs Membrane Endothelial Keratoplasty (DMEK) in a Series of Patients with Sjögren Syndrome

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

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