All ICD10 Codes

M04

Non-Billable
ChapterM00–M99
Rankcategory
Official Description

Autoinflammatory syndromes

Metadata & Insights
  • •2026 ICD-10-CM Edition - Effective October 1, 2025.
  • •Non-billable category (requires more specific sub-code).
  • •Chapter 13: Diseases of the Musculoskeletal System and Connective Tissue (M00-M99).

CH13 Parent Instructions

Excludes2(Not included here)
  • arthropathic psoriasis (L40.5-)
  • certain conditions originating in the perinatal period (P04-P96)
  • certain infectious and parasitic diseases (A00-B99)
  • compartment syndrome (traumatic) (T79.A-)
  • complications of pregnancy, childbirth and the puerperium (O00-O9A)
  • congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
  • endocrine, nutritional and metabolic diseases (E00-E88)
  • injury, poisoning and certain other consequences of external causes (S00-T88)
  • neoplasms (C00-D49)
  • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)

Instructional Notes

Excludes2(Not included here)
  • Crohn's disease (K50.-)

Clinical Hierarchy

CH13Diseases of the musculoskeletal system and connective tissue (M00-M99)
M04Autoinflammatory syndromes
M04.1Periodic fever syndromes
M04.2Cryopyrin-associated periodic syndromes
M04.8Other autoinflammatory syndromes
M04.9Autoinflammatory syndrome, unspecified

Applicable Ancestor Context

The following parent sections contain instructions or notes that apply to M04:

chapterCH13
Diseases of the musculoskeletal system and connective tissue (M00-M99)
View context instructions

Alphabetic Index Mentions

These are Diagnosis Index lookup paths that route to M04. Use them as entry terms for search, then confirm final coding instructions in the Tabular List and notes above.

25 raw paths
Arthritis, arthritic (acute) (chronic) (nonpyogenic) (subacute)1 unique routes
in (due to)
Mediterranean fever, familial→M04
CINCA (chronic infantile neurological, cutaneous and articular syndrome)1 unique routes
Direct index entry→M04
Deficiency, deficient2 unique routes
mevalonate kinase→M04
of interleukin 1 receptor antagonist [DIRA]→M04
DIRA (deficiency of interleukin 1 receptor antagonist)1 unique routes
Direct index entry→M04
Disease, diseased3 unique routes
autoinflammatory→M04(3 variants)
NOD2-associated→M04
specified type NEC→M04
Disorder (of)1 unique routes
neonatal onset multisystemic inflammatory (NOMID)→M04
Fever (inanition) (of unknown origin) (persistent) (with chills) (with rigor)2 unique routes
Mediterranean
familial→M04
periodic (Mediterranean)→M04
Mediterranean1 unique routes
fever
familial→M04
NOMID (neonatal onset multisystemic inflammatory disorder)1 unique routes
Direct index entry→M04
PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne syndrome)1 unique routes
Direct index entry→M04
PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome)1 unique routes
Direct index entry→M04
Syndrome10 unique routes
autoinflammatory→M04(2 variants)
specified type NEC→M04
Blau→M04
chronic
infantile neurological, cutaneous and articular (CINCA)→M04
cryopyrin-associated periodic→M04
familial cold autoinflammatory→M04
hyperimmunoglobulin D→M04
Majeed→M04
Muckle-Wells→M04
Yao→M04

MS-DRG Classification

2026

No direct MS-DRG triggers identified.

Cross Reference

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