M04.1
Periodic fever syndromes
M04.1 (also written M041) is a billable ICD-10-CM diagnosis code for periodic fever syndromes. It is part of the FY2027 code set, effective October 1, 2026. It is one of the 5 codes in category M04 (Autoinflammatory syndromes). The Alphabetic Index lists it under 9 entries. Its coding notes include 11 Excludes2 entries.
Excludes2
Not included here. Both codes may be reported if the patient has both conditions.
Also from chapter/block level (10 notes)
- 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)
Care settings
How CMS programs treat this code. These are CMS claim edits and list entries, not coverage or eligibility rules.
Home Health Primary
Accepted as the primary (principal) diagnosis on a home health claim. The Home Health Grouper assigns it to the MMTA - Other clinical group.
Source: CMS Home Health Grouper Software v07.2.26, effective October 1, 2026Inclusion terms
Conditions classified to this code.
- Familial Mediterranean fever
- Hyperimmunoglobin D syndrome
- Mevalonate kinase deficiency
- Tumor necrosis factor receptor associated periodic syndrome [TRAPS]
Notes
Also from chapter/block level (1 note)
- Use an external cause code following the code for the musculoskeletal condition, if applicable, to identify the cause of the musculoskeletal condition
Alphabetic Index terms
Terms the official Alphabetic Index lists for this code. 9 entries
- Arthritis, arthritic (acute) (chronic) (nonpyogenic) (subacute), in (due to), Mediterranean fever, familial
- Deficiency, deficient, mevalonate kinase
- Fever (inanition) (of unknown origin) (persistent) (with chills) (with rigor), Mediterranean, familial
- Fever (inanition) (of unknown origin) (persistent) (with chills) (with rigor), periodic (Mediterranean)
- Mediterranean, fever, familial
- Syndrome, hyperimmunoglobulin D
- Syndrome, periodic fever
- Syndrome, tumor necrosis factor receptor associated periodic (TRAPS)
- TRAPS (tumor necrosis factor receptor associated periodic syndrome)