M04.1 Periodic fever syndromes
The ICD-10-CM code for Periodic fever syndromes is M04.1 (FY2026). It is a billable, claim-ready diagnosis code.
Classification
- Section
- M04: Autoinflammatory syndromes (M04)
- Category M04
- 5 codes (4 billable)
- FY2026 Status
- Stable since FY2024
Also Known As
ICD-10-CM Alphabetic Index entries that lead to M04.1:
- TRAPS (tumor necrosis factor receptor associated periodic syndrome)
- Syndrome › tumor necrosis factor receptor associated periodic (TRAPS)
- Syndrome › hyperimmunoglobulin D
- Syndrome › periodic fever
- Fever (inanition) (of unknown origin) (persistent) (with chills) (with rigor) › periodic (Mediterranean)
- Deficiency, deficient › mevalonate kinase
- Arthritis, arthritic (acute) (chronic) (nonpyogenic) (subacute) › in (due to) › Mediterranean fever, familial
- Fever (inanition) (of unknown origin) (persistent) (with chills) (with rigor) › Mediterranean › familial
Inclusion Terms
- Familial Mediterranean fever
- Hyperimmunoglobin D syndrome
- Mevalonate kinase deficiency
- Tumor necrosis factor receptor associated periodic syndrome [TRAPS]
U.S. Hospital Utilization
- An estimated 830 U.S. inpatient stays in 2023 included M04.1 among the documented diagnoses.
- 170 stays listed it as the principal diagnosis.
Source: National Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality, 2016–2023. National survey-weighted estimates.
Official Coding Guidelines
Osteoporosis without pathological fracture (M81)Category M81, Osteoporosis without current pathological fracture, is for use for patients with osteoporosis who do not currently have a pathologic fracture due to the osteoporosis, even if they have had a fracture in the past. For patients with a history of osteoporosis fractures, status code Z87.310, Personal history of (healed) osteoporosis fracture, should follow the code from M81.
Osteoporosis with current pathological fracture (M80)Category M80, Osteoporosis with current pathological fracture, is for patients who have a current pathologic fracture at the time of an encounter. The codes under M80 identify the site of the fracture. A code from category M80, not a traumatic fracture code, should be used for any patient with known osteoporosis who suffers a fracture, even if the patient had a minor fall or trauma, if that fall or trauma would not usually break a normal, healthy bone.
Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026