Disorder of the immune system, NOS: Difference between revisions
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* adult-onset Still's Disease | * adult-onset Still's Disease | ||
* multisystem inflammatory syndrome in children (MIS-C) for COVID 19 related link with [[Post COVID-19 condition]] | * multisystem inflammatory syndrome in children (MIS-C) for COVID 19 related link with [[Post COVID-19 condition]] | ||
* | * 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.[1][2][3][4][5] The name VEXAS is an acronym deriving from the core features of disease:[6] | |||
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. | |||
== Alternate ICD10s to consider coding instead or in addition == | == Alternate ICD10s to consider coding instead or in addition == | ||
*[[Immune reconstitution syndrome]] | *[[Immune reconstitution syndrome]] |
Revision as of 11:00, 2022 September 7
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.
Additional Info
Includes:
- autoimmune disorders/autoimmune diseases
- adult-onset Still's Disease
- multisystem inflammatory syndrome in children (MIS-C) for COVID 19 related link with Post COVID-19 condition
- 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.[1][2][3][4][5] The name VEXAS is an acronym deriving from the core features of disease:[6]
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.
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
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