M04.9 - Autoinflammatory syndrome, unspecified
| ICD-10: | M04.9 |
| Short Description: | Autoinflammatory syndrome, unspecified |
| Long Description: | Autoinflammatory syndrome, unspecified |
| Status: | Valid for Submission |
| Version: | ICD-10-CM 2023 |
| Code Classification: |
M04.9 is a billable ICD-10 code used to specify a medical diagnosis of autoinflammatory syndrome, unspecified. The code is valid during the fiscal year 2023 from October 01, 2022 through September 30, 2023 for the submission of HIPAA-covered transactions.
Unspecified diagnosis codes like M04.9 are acceptable when clinical information is unknown or not available about a particular condition. Although a more specific code is preferable, unspecified codes should be used when such codes most accurately reflect what is known about a patient's condition. Specific diagnosis codes should not be used if not supported by the patient's medical record.
Clinical Information
- Autoinflammation, Panniculitis, and Dermatosis Syndrome|AIPDS|ORAS|Otulin-Related Autoinflammatory Syndrome|Otulipenia-. an autosomal recessive condition caused by mutation(s) in the otulin gene, encoding ubiquitin thioesterase otulin. it is characterized by neonatal onset of recurrent fever, erythematous rash with painful nodules, painful joints, and lipodystrophy.
- Autoinflammatory Syndrome-. a group of disorders of the innate immune system characterized by attacks of seemingly unprovoked inflammation without significant levels of either autoantibodies or autoreactive t cells more characteristic of autoimmune disease.
- Familial Cold Autoinflammatory Syndrome|FCAS|FCAS-. an autoinflammatory disease caused by mutations in the nlrp3 gene which encodes cryopyrin. it is characterized by short episodes of fever, rash, and arthralgia after exposure to cold or rapid decrease in temperature.
- NACHT, LRR and PYD Domains-Containing Protein 3|Angiotensin/Vasopressin Receptor AII/AVP-Like|Cold Autoinflammatory Syndrome 1 Protein|Cryopyrin|PYRIN-Containing APAF1-Like Protein 1-. nacht, lrr and pyd domains-containing protein 3 (1034 aa, ~118 kda) is encoded by the human nlrp3 gene. this protein plays a role in the modulation of both inflammation and apoptosis.
- NEMO Deleted Exon 5 Autoinflammatory Syndrome|IKBKG/NEMO NDAS|NDAS-. an autoinflammatory syndrome caused by either gain-of-function splice site variants in the ikbkg gene resulting in a deletion in the c-terminal domain of the nemo (nf-kappa-b essential modulator) protein or by increased expression of an isoform lacking the domain encoded by exon 5. it is clinically distinct from the immunodeficiency syndromes caused by loss-of-function ikbkg mutations and has features more characteristic of chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (candle).
- NLRP3 wt Allele|AGTAVPR|AII|AII/AVP|AVP|C1orf7|CIAS1|CLR1.1|Cold Autoinflammatory Syndrome 1 Gene|Cryopyrin Gene|FCAS|FCU|FLJ95925|MWS|NALP3|NLR Family, Pyrin Domain Containing 3 wt Allele|Nucleotide-Binding Oligomerization Domain, Leucine Rich Repeat and Pyrin Domain Containing 3 Gene|PYPAF1-. human nlrp3 wild-type allele is located in the vicinity of 1q44 and is approximately 33 kb in length. this allele, which encodes nacht, lrr and pyd domains-containing protein 3, is involved in the regulation of both apoptosis and inflammation. mutation of the gene is associated with familial cold autoinflammatory syndrome, muckle-wells syndrome, and chronic infantile neurological cutaneous and articular syndrome.
- Proteasome-Associated Autoinflammatory Syndrome 1|CANDLE|CANDLE|Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated Temperature (CANDLE) Syndrome|Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated Temperature Syndrome|PRAAS1-. an autosomal recessive condition caused by mutation(s) in the psmb8 gene, encoding proteasome subunit beta type-8. it is characterized by early onset annular erythematous plaques, partial lipodystrophy, and recurrent fever.
Index to Diseases and Injuries References
The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for this diagnosis code are found in the injuries and diseases index:
- - Disease, diseased - See Also: Syndrome;
- - autoinflammatory - M04.9
- - Syndrome - See Also: Disease;
- - autoinflammatory - M04.9
Convert to ICD-9 Code
| Source ICD-10 Code | Target ICD-9 Code | |
|---|---|---|
| M04.9 | 710.9 - Diff connect tis dis NOS | |
| Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code. | ||
Code History
- FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
- FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
- FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
- FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
- FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
- FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
