AutoICD API

M04.8

Billable

Other autoinflammatory syndromes

Other autoinflammatory syndromes

Status

Billable / Specific

Block

M04-M04

Parent Code

M04

Coding Notes

Inclusion Terms

Alternative clinical terms for this condition

  • 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]

Excludes 2

Conditions not included here, but the patient may have both

Related Codes(3)
M04.1

Periodic fever syndromes

BillableM04.2

Cryopyrin-associated periodic syndromes

BillableM04.9

Autoinflammatory syndrome, unspecified

Billable
Also Known As / Clinical Terms(286)

SNOMED CT

UMLS

Clinical Terms

  • CRM01
  • Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome [PAPA]
  • Autoinflammatory disease due to interleukin-1 receptor antagonist deficiency
  • Acute pustular eruption of skin
  • Hereditary pediatric Behçet-like disease
  • Neonatal anemia
  • Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis due to HOIP deficiency
  • Hemophagocytic lymphohistiocytosis
  • Blau syndrome
  • Congenital dyserythropoietic anemia and chronic recurrent multifocal osteomyelitis
  • Familial recurrent arthritis
  • Deficiency of the Interleukin-1 Receptor Antagonist
  • Acute generalised pustular psoriasis
  • BLAUS
  • Sporadic Blau syndrome
  • Familial juvenile systemic granulomatosis
  • Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis
  • FRA
  • Familial granulomatosis, Blau type
  • OTULIN (OTU deubiquitinase with linear linkage specificity) related autoinflammatory syndrome
  • Disorder of haematopoietic system in newborn
  • Nucleotide binding oligomerization domain containing 2-associated autoinflammatory disease
  • CDA - congenital dyserythropoietic anemia
  • Early onset sarcoidosis
  • Deficiency Of Interleukin 1 Receptor Antagonist
  • Deficiency of interleukin(IL)-1 receptor antagonist
  • Behçet-like disease due to haploinsufficiency of A20
  • PAPA syndrome
  • Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome [PFAPA]
  • Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis due to HOIL-1 deficiency
  • PAPAS
  • Majeed syndrome
  • Type I interferonopathy
  • Acute blistering eruption of skin
  • NOD2-associated autoinflammatory disease
  • Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis due to RNF31 mutation
  • Chronic recurrent multifocal osteomyelitis, congenital
  • Otulipenia
  • Autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation
  • Paediatric granulomatous arthritis
  • NOCARH syndrome
  • Arthrocutaneouveal granulamotosis
  • APLAID - autoinflammation phospholipase C gamma 2 associated antibody deficiency and immune dysregulation
  • Interleukin-1 receptor antagonist deficiency
  • CHRONIC RECURRENT MULTIFOCAL OSTEOMYELITIS 1, WITH CONGENITAL DYSERYTHROPOIETIC ANEMIA, WITH OR WITHOUT NEUTROPHILIC DERMATOSIS
  • OTULIN-related autoinflammatory syndrome
  • Hereditary paediatric Behçet-like disease
  • Hyperinflammatory lymphohistiocytosis
  • Generalized pustular psoriasis of von Zumbusch
  • Chronic recurrent multifocal osteomyelitis, congenital dyserythropoietic anaemia and neutrophilic dermatosis
  • Chronic recurrent multifocal osteomyelitis, congenital dyserythropoietic anemia and neutrophilic dermatosis
  • Congenital dyserythropoietic anaemia
  • CHRONIC RECURRENT MULTIFOCAL OSTEOMYELITIS 2, WITH PERIOSTITIS AND PUSTULOSIS
  • INTERLEUKIN 1 RECEPTOR ANTAGONIST DEFICIENCY
  • Pyogenic Arthritis, Pyoderma Gangrenosum and Acne
  • Haemophagocytic lymphohistiocytosis
  • OTU deubiquitinase with linear linkage specificity related autoinflammatory syndrome
  • Pediatric granulomatous arthritis
  • Autoinflammation phospholipase C gamma 2 associated antibody deficiency and immune dysregulation
  • Familial granulomatous inflammatory arthritis, dermatitis and uveitis
  • Disorder of hematopoietic system in newborn
  • Neonatal purpura
  • Neonatal anaemia
  • NOD2 (nucleotide binding oligomerization domain containing 2) associated autoinflammatory disease
  • Dyserythropoietic anemia, and neutrophilic dermatosis
  • Behçet-like disease due to HA20
  • Monogenic autoinflammatory syndrome
  • MJDS
  • Neonatal onset cytopenia, autoinflammation, rash, episodes of hemophagocytic lymphohistiocytosis syndrome
  • Chronic Recurrent Multifocal Osteomyelitis, Congenital Dyserythropoietic Anemia, and Neutrophilic Dermatosis
  • Pyogenic Sterile Arthritis, Pyoderma Gangrenosum, and Acne
  • Sterile multifocal osteomyelitis with periostitis and pustulosis (disorder)
  • Acute pustular skin rash
  • CRMO1
  • OSTEOMYELITIS, STERILE MULTIFOCAL, WITH PERIOSTITIS AND PUSTULOSIS
  • Deficiency of interleukin 36 receptor antagonist
  • USP18 deficiency
  • Haploinsufficiency of A20
  • Pyogenic arthritis, pyoderma gangrenosum, acne syndrome
  • Complex multigenic autoinflammatory syndrome
  • VEXAS syndrome
  • Mendelian susceptibility to mycobacterial disease due to complete interleukin 12 subunit beta deficiency
  • Blau syndrome (disorder)
  • Chronic recurrent multifocal osteomyelitis, congenital dyserythropoietic anemia and neutrophilic dermatosis (disorder)
  • Pyogenic arthritis, pyoderma gangrenosum, acne syndrome (disorder)
  • PAPA (pyogenic arthritis, pyoderma gangrenosum, acne) syndrome
  • OMPP
  • Granulomatous inflammatory arthritis, dermatitis and uveitis, familial
  • Acute generalized pustular psoriasis
  • SAMD9L-SAAD - SAMD9L-associated autoinflammatory disease
  • Vesicular eruption
  • HA20 (haploinsufficiency A20)
  • Deficiency of interleukin-1 receptor antagonist
  • Sterile alpha motif domain containing 9 like-associated autoinflammatory syndrome
  • Acquired pancytopenia
  • Type I interferon-associated autoinflammatory disease
  • Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome
  • OMPP - sterile osteomyelitis, multifocal with periostitis and pustulosis
  • Deficiency of IL-36Ra (interleukin 36 receptor antagonist)
  • Infantile-onset periodic fever, panniculitis, dermatosis syndrome
  • Vesicular rash
  • Deficiency of interleukin 1 receptor antagonist [DIRA]
  • Pyogenic arthritis, pyoderma gangrenosum, and acne
  • Haemophagocytic syndrome
  • Congenital dyserythropoietic anemia
  • Sterile multifocal osteomyelitis with periostitis and pustulosis
  • Deficiency of interleukin-1 receptor antagonist (DIRA)
  • DITRA - deficiency of interleukin 36 receptor antagonist
  • Generalised pustular psoriasis
  • Pyogenic sterile arthritis pyoderma gangrenosum and acne syndrome
  • Bullous eruption
  • Acute pustular skin eruption
  • CDA - congenital dyserythropoietic anaemia
  • SAMD9L-associated autoinflammatory syndrome
  • CRMO2
  • Mendelian susceptibility to mycobacterial disease due to complete IL12B deficiency
  • Yao syndrome
  • Pyogenic arthritis, pyoderma gangrenosum, and severe cystic acne
  • Type 1 interferonopathy
  • Neonatal thrombocytopenia
  • Hemophagocytic syndrome
  • Pancytopenia - acquired
  • Chronic recurrent multifocal osteomyelitis-congenital dyserythropoietic anemia-neutrophilic dermatosis syndrome
  • Generalized pustular psoriasis
  • DIRA
  • A20 haploinsufficiency
  • Majeed's syndrome
  • Generalised pustular psoriasis of von Zumbusch
  • Deficiency of IL-36R antagonist
  • Purpura of newborn
  • Acute blistering skin eruption
  • Ubiquitin specific peptidase 18 deficiency
  • Arthrocutaneouveal granulomatosis
  • PAPA
Frequently Asked Questions
What is the ICD-10 code for other autoinflammatory syndromes?

The ICD-10-CM code for other autoinflammatory syndromes is M04.8. The full clinical description is "Other autoinflammatory syndromes". M04.8 is a billable/specific code that can be used on insurance claims and medical billing.

What does ICD-10 code M04.8 mean?

ICD-10-CM code M04.8 represents “Other autoinflammatory syndromes”. It is classified under Chapter 13: Diseases of the Musculoskeletal System and Connective Tissue and is a billable/specific code that can be used on a claim.

Is M04.8 a billable code?

Yes, M04.8 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.

What chapter is M04.8 in?

M04.8 is in Chapter 13: Diseases of the Musculoskeletal System and Connective Tissue (codes M00-M99).

What SNOMED CT codes does M04.8 map to?

M04.8 maps to 31 SNOMED CT concepts: 1290916005, 778004006, 5876000, 721542002, 238612002, and 26 more. SNOMED CT is a clinical terminology used in electronic health records.

What are the UMLS CUIs for M04.8?

M04.8 is linked to 6 UMLS Concept Unique Identifiers: C5201146, C2748507, C1864997, C1858361, C4268693, and 1 more. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.

How does M04.8 relate to ICF functioning codes?

ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like other autoinflammatory syndromes affect a person's functioning — body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.

What is the ICD-11 equivalent of M04.8?

There is no direct ICD-11 mapping available for M04.8 in the WHO crosswalk tables. This may mean the concept is classified differently in ICD-11. Use the ICD-10 to ICD-11 converter to search for related codes.

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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.