2026 ICD-10-CM Diagnosis Code M04.8Other autoinflammatory syndromes

ICD-10-CM Codes›M00–M99›M04›M04

ICD-10-CM M04.8
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

M04.8 is a billable ICD-10-CM diagnosis code for other autoinflammatory syndromes. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 545 through 547. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Autoinflammatory syndromes.

Code Identity

ICD-10-CM Code
M04.8
Billable Status
Yes — Valid for Submission
Code Describes
Other autoinflammatory syndromes
Short Description
Other autoinflammatory syndromes
Same as the full description in the CMS dataset.
Parent Code
M04
Autoinflammatory syndromes

Code Classification

ChapterM00–M99Diseases of the musculoskeletal system and connective tissue
SectionM04Autoinflammatory syndromes
CategoryM04Autoinflammatory syndromes
This CodeM04.8Other autoinflammatory syndromes

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Acquired pancytopenia
  • Acute blistering eruption of skin
  • Acute pustular skin eruption
  • Autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation
  • Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis
  • Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis due to HOIP deficiency
  • Blau syndrome
  • Bullous eruption
  • Complex multigenic autoinflammatory syndrome
  • Congenital dyserythropoietic anemia
  • Deficiency of interleukin 36 receptor antagonist
  • Disorder of hematopoietic system in newborn
  • Generalized pustular psoriasis
  • Granulomatous inflammatory arthritis, dermatitis and uveitis, familial
  • Haploinsufficiency of A20
  • Hemophagocytic lymphohistiocytosis
  • Hereditary pediatric Behçet-like disease
  • Majeed syndrome
  • Mendelian susceptibility to mycobacterial disease due to complete interleukin 12 subunit beta deficiency
  • Monogenic autoinflammatory syndrome
  • Neonatal anemia
  • Neonatal thrombocytopenia
  • NOCARH syndrome
  • OTULIN-related autoinflammatory syndrome
  • SAMD9L-associated autoinflammatory syndrome
  • Sporadic Blau syndrome
  • Type I interferonopathy
  • USP18 deficiency
  • Vesicular eruption
  • VEXAS syndrome
  • Yao syndrome

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Blau syndrome
  • Deficiency of interleukin 1 receptor antagonist DIRA
  • Majeed syndrome
  • Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome PFAPA
  • Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome PAPA

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Deficiency, deficient
      • of interleukin 1 receptor antagonist [DIRA]
    • DIRA(deficiency of interleukin 1 receptor antagonist)
    • Disease, diseased
      • autoinflammatory
        • NOD2-associated
    • Disease, diseased
      • autoinflammatory
        • specified type NEC
    • PAPA(pyogenic arthritis, pyoderma gangrenosum, and acne syndrome)
    • PFAPA(periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome)
    • Syndrome
      • autoinflammatory
        • specified type NEC
    • Syndrome
      • Blau
    • Syndrome
      • Majeed
    • Syndrome
      • periodic fever, aphthous stomatitis, pharyngitis, and adenopathy [PFAPA]
    • Syndrome
      • pyogenic arthritis, pyoderma gangrenosum, and acne [PAPA]
    • Syndrome
      • Yao

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR MUS036
Autoinflammatory syndromes
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • CDAN1 Gene|CDAN1|CDAN1|Congenital Dyserythropoietic Anemia, Type I Gene

    this gene may be involved in nuclear membrane maintenance.

  • CDAN1 wt Allele|CDA-I|CDA1|CDAI|Codanin Gene|Congenital Dyserythropoietic Anemia, Type I wt Allele|DLT|Discs Lost Homolog Gene|Discs Lost, Drosophila, Homolog of Gene|PRO1295|UNQ664/PRO1295

    human cdan1 wild-type allele is located in the vicinity of 15q15.2 and is approximately 14 kb in length. this allele, which encodes codanin-1 protein, may play a role in the maintenance of the nuclear envelope. mutation of the gene is associated with congenital dyserythropoietic anemia type i.

  • Congenital Dyserythropoietic Anemia

    a rare group of disorders that result in anemia that is caused by ineffective erythropoiesis, which is associated with multinuclear erythroblasts, and which may present in childhood. the most common mutations are in the cdan1 and sec23b genes.

  • Congenital Dyserythropoietic Anemia Type II|CDA II|CDAN2|HEMPAS|Hereditary Erythroblastic Multinuclearity with Positive Acidified-Serum Test|SEC23B-CDG

    an autosomal recessive subtype of congenital dyserythropoietic anemia caused by mutation(s) in the sec23b gene, encoding protein transport protein sec23b.

  • Congenital Dyserythropoietic Anemia Type IV|CDAN4

    an autosomal dominant sub-type of congenital dyserythropoietic anemia caused by mutation(s) in the klf1 gene, encoding krueppel-like factor 1.

  • SEC23B wt Allele|CDA-II|CDAII|CDAN2|Congenital Dyserythropoietic Anemia, Type II Gene|HEMPAS|RP11-379J5.1|Sec23 Homolog B (S. cerevisiae) wt Allele

    human sec23b wild-type allele is located in the vicinity of 20p11.23 and is approximately 54 kb in length. this allele, which encodes protein transport protein sec23b, is involved in the transport of vesicles from the endoplasmic reticulum to the golgi. mutation of the gene is associated with congenital dyserythropoietic anemia type ii.

  • Majeed Syndrome

    an autoinflammatory disease caused by mutations in the lpin2 gene. it is characterized by early-onset chronic recurrent multifocal osteomyelitis, congenital dyserythropoietic anemia and inflammatory dermatosis.

  • Generalized Pustular Psoriasis

    a genetically heterogenous autoinflammatory condition characterized by sterile non-follicular pustules over wide areas of the body. it is a rare and severe form of psoriasis, and may result in death if not treated.

  • Neonatal Alloimmune Thrombocytopenia|NAIT|Neonatal Thrombocytopenia due to Platelet Alloimmunization

    thrombocytopenia that occurs in neonates as a consequence of transplacental passage of maternal alloantibodies directed against fetal platelet antigens.

  • Neonatal Thrombocytopenia

    a condition characterized by a decrease in the number of platelets in the blood below established reference ranges in a newborn.

Patient EducationClinical

Autoimmune Diseases

Your immune system protects you from disease and infection by attacking germs that get into your body, such as viruses and bacteria. Your immune system can tell that the germs aren't part of you, so it destroys them. If you have an autoimmune disease, your immune system attacks the healthy cells of your organs and tissues by mistake.

The full article covers:

  • What are autoimmune diseases?
  • What causes autoimmune diseases?
  • Who is at risk for autoimmune diseases?
  • What are the symptoms of autoimmune diseases?
  • How are autoimmune diseases diagnosed?
  • What are the treatments for autoimmune diseases?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert M04.8 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
710.8 Diff connect tis dis NEC
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2018AddedAdded to the ICD-10-CM code setEffective October 1, 2017.
FY 2019–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About M04.8Overview

Is M04.8 (Autoinflammatory syndromes) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report other autoinflammatory syndromes on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does M04.8 group to?

When other autoinflammatory syndromes is the principal diagnosis on an inpatient stay, it groups to MS-DRG 545, 546, 547, with relative weights from 0.8362 to 2.4817 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of M04.8?

Under the General Equivalence Mappings, other autoinflammatory syndromes converts to ICD-9-CM 710.8 (diff connect tis dis NEC). The mapping is approximate, so confirm the match fits the documentation.

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.