ICD-11 classes
14 Diseases of the skin
Inflammatory dermatoses
Nonorgan specific systemic autoimmune disorders involving...
4A43 — Overlap or undifferentiated nonorgan specific systemic...
4A43.2 — Sjögren syndrome

ICD-11 4A43.2 — Sjögren syndrome

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'').

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code 4A43.2 contains 5 clarifying diagnoses:

1. 4A43.20 — Primary Sjögren syndrome
2. 4A43.21 — Secondary Sjögren syndrome
3. 4A43.22 — Paediatric onset Sjögren syndrome
4. 9A79 — Keratoconjunctivitis sicca
5. 4A43.2Y — Other specified sjögren syndrome

The diagnosis is coded elsewhere:

• Overlap or undifferentiated nonorgan specific systemic autoimmune disease #3652
• Sjögren syndrome #3659
• Overlap or undifferentiated nonorgan specific systemic autoimmune disease #18380
• Sjögren syndrome #18387