M04.8
Other autoinflammatory syndromes
Billable FY2026 2025-10-01 → 2026-09-30
Exclusions 11 never code together
M00-M99chapter
Excludes2
- arthropathic psoriasis (L40.5-)L40.5-
- certain conditions originating in the perinatal period (P04-P96)P04-P96
- certain infectious and parasitic diseases (A00-B99)A00-B99
- compartment syndrome (traumatic) (T79.A-)T79.A-
- complications of pregnancy, childbirth and the puerperium (O00-O9A)O00-O9A
- congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)Q00-Q99
- endocrine, nutritional and metabolic diseases (E00-E88)E00-E88
- injury, poisoning and certain other consequences of external causes (S00-T88)S00-T88
- neoplasms (C00-D49)C00-D49
- symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)R00-R94
Excludes2 — not included here, but the patient may have both. Code both when documented.
Instructional notes 7
M04.8code
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]
M00-M25block
Includes
- Disorders affecting predominantly peripheral (limb) joints
M00-M99chapter
Notes
- Use an external cause code following the code for the musculoskeletal condition, if applicable, to identify the cause of the musculoskeletal condition
Index entries leading here 6
- Syndrome — see also Disease
- Disease, diseased — see also Syndrome
- Deficiency, deficient
- of interleukin 1 receptor antagonist [DIRA] M04.8
- DIRA (deficiency of interleukin 1 receptor antagonist) M04.8
- PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne syndrome) M04.8
- PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome) M04.8
Alongside this code 3 same parent — M04
Additional references
AHA Coding Clinic® Licence required
Official coding advice for this code. Published by the American Hospital Association. Not included in this installation.