" /> Sjögren syndrome - CISMeF





ICD-11 code : 4A43.2;

Preferred Label : Sjögren syndrome;

ICD-11 definition : Sjögren syndrome (SS) is a slowly progressive, systemic inflammatory autoimmune disease affecting primarily the exocrine glands. Lymphocytic infiltrates replace functional epithelium, leading to oral and ocular dryness. Characteristic autoantibodies (e.g., anti-Ro/SS-A and/or anti-La/SS-B) are produced. The disorder can occur alone (it is then known as primary SS) or in association with another autoimmune disease (it is then known as secondary SS).;

ICD-11 synonym : Sicca syndrome;

ICD-11 inclusion : Sjögren syndrome with renal tubulo-interstitial disorders; Sjögren syndrome with myopathy;

ICD-11 "other" category code : 4A43.2Y;

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Sjögren syndrome (SS) is a slowly progressive, systemic inflammatory autoimmune disease affecting primarily the exocrine glands. Lymphocytic infiltrates replace functional epithelium, leading to oral and ocular dryness. Characteristic autoantibodies (e.g., anti-Ro/SS-A and/or anti-La/SS-B) are produced. The disorder can occur alone (it is then known as primary SS) or in association with another autoimmune disease (it is then known as secondary SS).

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22/12/2025


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