2024 ICD-10-CM Diagnosis Code M04.9

Autoinflammatory syndrome, unspecified

ICD-10-CM Code:
M04.9
ICD-10 Code for:
Autoinflammatory syndrome, unspecified
Is Billable?
Yes - Valid for Submission
Chronic Condition Indicator: [1]
Chronic
Code Navigator:

Code Classification

  • Diseases of the musculoskeletal system and connective tissue
    (M00–M99)
    • Autoinflammatory syndromes
      (M04)
      • Autoinflammatory syndromes
        (M04)

M04.9 is a billable diagnosis code used to specify a medical diagnosis of autoinflammatory syndrome, unspecified. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024.

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.

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Autoinflammatory disease
  • Hemolytic erythrophagocytic syndrome
  • Hemophagocytic lymphohistiocytosis
  • Macrophage activation syndrome
  • Periodic fever, infantile enterocolitis, autoinflammatory syndrome

Clinical Classification

Clinical Information

  • Macrophage Activation Syndrome

    a serious complication of childhood systemic inflammatory disorders that is thought to be caused by excessive activation and proliferation of t-lymphocytes and macrophages. it is seen predominantly in children with systemic onset juvenile idiopathic arthritis.
  • 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|NLRP3|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 following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Convert M04.9 to ICD-9-CM

  • ICD-9-CM Code: 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 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • 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

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.