Disorder of the immune system, NOS: Difference between revisions
		
		
		
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== Additional Info ==  | == Additional Info ==  | ||
Includes  | '''Includes'''   | ||
* Antisynthetase syndrome  | * 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:  | * 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.  | **V: Vacuoles are often identified in the bone marrow stem cells of patients presenting with VEXAS.  | ||
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**A: Patients with VEXAS present with a wide array of Autoinflammatory conditions  | **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.  | **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  | |||
* CRS- cytokine release syndrome combine with [[Iatrogenic, complication of medical or surgical care NOS]] and if appropriate in comorbid [[Past history of Chimeric Antigen Receptor T-cell Immunotherapy (CAR-T)|Past history of Chimeric Antigen Receptor T-cell Immunotherapy]]  | |||
'''Excludes'''  | |||
* Adult-onset Still's Disease [[Inflammatory/immune arthritis, NOS]]  | |||
== Alternate ICD10s to consider coding instead or in addition ==  | == Alternate ICD10s to consider coding instead or in addition ==  | ||
Latest revision as of 21:01, 20 June 2025
| 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: | |
| Data Dependencies(Reports/Indicators/Data Elements): | No results | 
| External ICD10 Documentation | |
This diagnosis is a part of ICD10 collection.
Additional Info
Includes
- 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
 - CRS- cytokine release syndrome combine with Iatrogenic, complication of medical or surgical care NOS and if appropriate in comorbid Past history of Chimeric Antigen Receptor T-cell Immunotherapy
 
Excludes
- Adult-onset Still's Disease Inflammatory/immune arthritis, NOS
 
Alternate ICD10s to consider coding instead or in addition
- Immune reconstitution syndrome
 - Combined immunodeficiency
 - Common variable immunodeficiency (CVID)
 - Immunodeficiency state, NOS
 - Immunodeficiency due to antibody deficiency or defect, hereditary or acquired
 
Candidate Combined ICD10 codes
Related CCI Codes
Data Integrity Checks (automatic list)
none found
Related Articles
Show all ICD10 Subcategories