Other autoinflammatory syndromes
Billable / specificHCC V28 94
M04.8 is a billable, specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes in FY2027.
Inclusion terms
Applicable to
- 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]
Includes (from M04)
- Note: Use an external cause code following the code for the musculoskeletal condition, if applicable, to identify the cause of the musculoskeletal condition
Excludes2
Not included here — may be reported together when both conditions exist.
- arthropathic psoriasis (L40.5-) (from M04)
- certain conditions originating in the perinatal period (P04-P96) (from M04)
- certain infectious and parasitic diseases (A00-B99) (from M04)
- compartment syndrome (traumatic) (T79.A-) (from M04)
- complications of pregnancy, childbirth and the puerperium (O00-O9A) (from M04)
- congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99) (from M04)
- endocrine, nutritional and metabolic diseases (E00-E88) (from M04)
- injury, poisoning and certain other consequences of external causes (S00-T88) (from M04)
- neoplasms (C00-D49) (from M04)
- symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94) (from M04)
- Crohn's disease (K50.-) (from M04)
Also known as
- 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]
- Deficiency, deficient, of interleukin 1 receptor antagonist [DIRA]
- DIRA
- Disease, diseased, autoinflammatory, NOD2-associated
- Disease, diseased, autoinflammatory, specified type NEC
- PAPA
- PFAPA
- 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
Parent code
- M04Autoinflammatory syndromesHeader