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Sjögren’s syndrome (SS) is a chronic, slowly progressive autoimmune exocrinopathy characterised by mixed cellular infiltration of exocrine glands, notably the lacrimal glands and salivary glands. Xerostomia and xerophthalmia are prominent features of SS, but every epithelial surface can be affected such as nose, throat and vagina. The term autoimmune epitheliitis has been suggested recently. The combination of dry eyes and dry mouth is often referred to as sicca syndrome. SS can be primary or secondary. Secondary SS can be seen in patients with other autoimmune diseases such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), scleroderma (SCL) etc. The first description of SS is credited to the Polish surgeon Johann von Mickulicz who, in 1892, described a 42-year-old man with enlargement of the parotid and lacrimal glands associated with a round-cell infiltrate and acinar atrophy. In 1930, Henrik Sjögren coined the term keratoconjunctivitis sicca (KCS) clarifying that this type of xerophthalmia had no relation to vitamin A deficiency. In literature, SS replaced the term keratoconjunctivitis sicca as a more general term.
References and Further Reading
- Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, Rasmussen A, Scofield H, Vitali C, Bowman SJ, Mariette X; International Sjogren’s Syndrome Criteria Working Group. 2016 American College of Rheumatology/European league against rheumatism Classifiaction criteria for primary Sjögren’s syndrome: a consensus and data-driven methodology involving three international patient cohorts. Arthritis Rheumatol. 2017;69(1):35–45.CrossRefGoogle Scholar
- Skopouli FN, Moutsopoulos HM. Autoimmune epitheliitis: Sjögren’s syndrome. Clin Exp Rheumatol. 1994;Suppl11:S9–11.Google Scholar