ICD-11 classes
14 Diseases of the skin
Inflammatory dermatoses
Nonorgan specific systemic autoimmune disorders involving...
4A43 — Overlap or undifferentiated nonorgan specific systemic...
4A43.0 — IgG4 related disease

ICD-11 4A43.0 — IgG4 related disease

IgG4 related syndrome (IgG4-related disease: IgG4-RD) is a clinical disease characterised by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. The diagnostic criteria for IgG4 related syndrome have been proposed, and it may be present in a certain population of patients with a wide variety of diseases, including Mikulicz disease, autoimmune pancreatitis, hypophysitis, Riedel thyroiditis, interstitial pneumonitis, interstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory aortic aneurysm, and inflammatory pseudo tumour.

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code 4A43.0 contains 2 clarifying diagnoses:

1. EK91.2 — Primary cutaneous plasmacytosis
2. Benign dermal lymphocytic or lymphoplasmacytic infiltrations or proliferations
   It contains 2 clarifying diagnoses.

The diagnosis is coded elsewhere:

• Overlap or undifferentiated nonorgan specific systemic autoimmune disease #3652
• Overlap or undifferentiated nonorgan specific systemic autoimmune disease #18380