C176600Level 6
Primary Immune Regulatory Disorder
**Semantic type:** Disease or Syndrome
**Definition:** A group of immune diseases characterized predominantly by immune dysregulation leading to organ-specific autoimmunity, excessive inflammation, and non-malignant lymphoproliferation. Unlike classical primary immunodeficiencies, susceptibility to infections is typically less prominent in these disorders.
**Synonyms:** - PIRD - Primary Immune Dysregulation Disorder - Primary Immune Dysregulation Disorder
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