Corneal complications of Sjögren's disease: Insights from a large tertiary ophthalmological care center

Sjögren's disease (SjD) is a chronic, progressive systemic autoimmune condition characterized by inflammation and lymphocytic infiltration of exocrine glands, leading to their atrophy and dysfunction [1]. The condition predominantly affects women between the fourth and sixth decades of life, with dryness being the primary clinical feature, affecting more than 95 % of patients [2]. The estimated incidence of SjD is 4 per 1000 people per year, with a prevalence of approximately 0.5 %, making it one of the most common rheumatological diseases [2,3].

SjD may occur as a standalone, primary autoimmune disease or may present concurrently with other autoimmune diseases [4]. The most frequently associated systemic autoimmune conditions with SjD include rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis, idiopathic inflammatory myopathies, mixed connective tissue disease, and dermatomyositis [4,5].

The clinical spectrum of SjD is variable, with the majority of the patients suffering from sicca (xerophthalmia and xerostomia), musculoskeletal pain, and fatigue. Although the occurrence of extraglandular systemic manifestations of SjD is well known, extraglandular ocular complications, such as corneal ulceration and corneal perforation, are less well-recognized and may be missed, particularly in the absence of a previously established diagnosis [6]. The incidence of such vision-threatening complications is estimated to be 2.5 %–3.6 % among SjD patients [7,8]. The diagnosis of SjD can be challenging, as it requires a comprehensive, multidisciplinary clinical evaluation combined with specific laboratory tests [9,10]. As a result, delays in diagnosis and appropriate management are common, often leading to disease-related complications.

This study aims to review a longitudinal cohort of patients with SjD from a large tertiary care-based sample, focusing on clinical characteristics, diagnostic approaches, and corneal extraglandular manifestations.

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