What Ophthalmologists Should Know About Sjögren’s Syndrome

Abstract

Dry-eye syndrome (DES) is a common ocular disorder, affecting an estimated 25% of patients presenting to general ophthalmology clinics. 1 The incidence of DES varies significantly depending on the age of the population studied and the signs and/or symptoms considered for making the diagnosis. Recent studies suggested that over nine million Americans suffer from a clinically significant form of DES and many more millions are thought to have either a milder form of the disease or intermittent manifestations. SS is more common in females, with a female:male ratio of 9:1. 11,12Although SS can be seen in all age groups, it is most commonly encountered in the fourth and fifth decades of life. 6 Over 90% of SS patients are females in their mid-50s, around the age of menopause. to conserve tears. Preservative-free tear substitutes, topical steroids, cyclosporine A, autologous serum eye-drops and punctal plugs are commonly used for this purpose.

Sjögren’s Syndrome as an Ocular Problem: Signs and Symptoms, Diagnosis, Treatment

“…Systemic manifestations of SS are divided into visceral (lung, heart, kidney, endocrine, nervous system, gastrointestinal) and non-visceral (skin, myalgia, arthralgia). The risk of lymphoma is higher in patients with SS than in general population [1,4,[6][7][8]. SS can be primary-pSS (without any other accompanying symptoms) or secondary-sSS (with other autoimmune diseases: systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), polyarteritis nodosa, systemic sclerosis, granulomatosis with polyangiitis (GPA), primary biliary cholangitis (PBC), mixed connective tissue disease, occult thyroid eye disease) [1,[3][4][5][6][9][10][11].…”

“…Sjögren's syndrome (SS) is a rheumatic autoimmune disease in which exocrine glands (salivary and lacrimal glands) are involved that results in clinical symptoms of dry mouth and dry eye. For the first time, SS-like clinical condition was described by Mikulicz in 1892 [1]. However, the disease was fully described (clinically and histopathology) later by the Swedish ophthalmologist Henrik Sjögren in 1930.…”

“…However, the disease was fully described (clinically and histopathology) later by the Swedish ophthalmologist Henrik Sjögren in 1930. He detected coincidence of rheumatism and hyposecretion of lacrimal and salivary glands and introduced the new term keratoconjunctivitis sicca to describe significant conjunctival and corneal staining with both dyes: methylene blue and rose bengal [1][2][3]. He also compared keratoconjunctivitis sicca and xerophthalmia seen in vitamin A deficiency and postulated that these cases represented a systemic disease.…”

“…Though his academic career was over, he lived on the day when the disease was named after him [2]. SS affects the exocrine glands; lymphocytic infiltration leads to sicca syndrome of the eyes, oral cavity, pharynx, larynx, and/or vagina [1,[3][4][5][6]. Systemic manifestations of SS are divided into visceral (lung, heart, kidney, endocrine, nervous system, gastrointestinal) and non-visceral (skin, myalgia, arthralgia).…”

“…Patients with DED seek medical help for many years prior to SS diagnosis-about 25% of DED patients have an underlying rheumatic condition, commonly pSS. Only one-third of patients with SS carried final diagnosis prior to ocular symptoms [1,11,12]. Some studies demonstrated coincidence of DED and SS in 46.7% cases [1,11].…”