Disorder of the immune system, NOS

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ICD10 Diagnosis
Dx: Disorder of the immune system, NOS
ICD10 code: D89.9
Pre-ICD10 counterpart: Immunological problems
Charlson/ALERT Scale: none
APACHE Como Component: none
APACHE Acute Component: none
Start Date:
Stop Date:
External ICD10 Documentation

This diagnosis is a part of ICD10 collection.

  • SMW
    • 2019-01-01
    • 2999-12-31
    • D89.9
  • Cargo

  • Categories
  • SMW
  • Cargo

  • Categories

Additional Info


  • Adult-onset Still's Disease
  • Antisynthetase syndrome
  • Autoimmune disorders/autoimmune diseases
  • Mast cell activation disorder
  • Multisystem inflammatory syndrome in children (MIS-C) for COVID 19 related link with Post COVID-19 condition
  • Pemphigus vulgaris
  • VEXAS syndrome- VEXAS syndrome is an adult-onset autoinflammatory disease primarily affecting males, caused by a somatic mutation of the UBA1 gene in hematopoietic progenitor cells. The name VEXAS is an acronym deriving from the core features of disease:
    • V: Vacuoles are often identified in the bone marrow stem cells of patients presenting with VEXAS.
    • E: The E1 ubiquitin conjugating enzyme encoded by the UBA1 gene is mutated in patients.
    • X: The mutated UBA1 gene is located on the X-chromosome and thus the disease is almost exclusively found in individuals with XY chromosomes and thus said to be X-linked.
    • A: Patients with VEXAS present with a wide array of Autoinflammatory conditions
    • S: The mutations which cause VEXAS are Somatic, meaning that they are acquired throughout life, not inherited, and unable to be passed onto one's offspring.
  • Evans syndrome
  • IgG4 disease
  • Immune dysregulation disorder

Alternate ICD10s to consider coding instead or in addition

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