ICD-11 code : 4A43.2;
Preferred Label : Sjögren syndrome;
ICD-11 definition : Sjögren syndrome 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;
Origin ID : 1923044320;
Automatic exact mappings (from CISMeF team)
Currated CISMeF NLP mapping
ICD-10 Mapping
Sjögren syndrome 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'').