M04.1
BillablePeriodic fever syndromes
Periodic fever syndromes
Coding Notes
Inclusion Terms
Alternative clinical terms for this condition
- Familial Mediterranean fever
- Hyperimmunoglobin D syndrome
- Mevalonate kinase deficiency
- Tumor necrosis factor receptor associated periodic syndrome [TRAPS]
Excludes 2
Conditions not included here, but the patient may have both
- 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)
- Crohn's disease (K50.-)
Also Known As / Clinical Terms
SNOMED CT
- Cervical adenitis3502005
- Cervical lymphadenitis3502005
- Benign paroxysmal peritonitis12579009
- FMF - Familial Mediterranean fever12579009
- Familial Mediterranean fever12579009
- Familial paroxysmal polyserositis12579009
- Familial recurrent polyserositis12579009
- MEF - Familial Mediterranean fever12579009
- Paroxysmal polyserositis12579009
- Periodic disease12579009
- Periodic familial peritonitis12579009
- Periodic peritonitis12579009
- Periodic polyserositis12579009
- Recurrent polyserositis12579009
- Pericarditis associated with familial Mediterranean fever111301006
- Pericarditis due to familial Mediterranean fever111301006
- Deficiency of mevalonate kinase124327008
- Mevalonate kinase deficiency124327008
- Haemophagocytic lymphohistiocytosis234437005
- Haemophagocytic syndrome234437005
- Hemophagocytic lymphohistiocytosis234437005
- Hemophagocytic syndrome234437005
- Hyperinflammatory lymphohistiocytosis234437005
- Amyloid of familial Mediterranean fever367528006
- Amyloidosis due to familial Mediterranean fever367528006
- Recurrent aphthous ulcer398870000
- Recurrent aphthous ulceration398870000
- Hereditary periodic fever402790006
- Familial autosomal dominant periodic fever403833009
- TNF receptor-associated periodic fever syndrome403833009
- TNF receptor-associated periodic fever syndrome (TRAPS)403833009
- TRAPS - TNF receptor-associated periodic fever syndrome403833009
- Tumor necrosis factor (TNF) receptor-associated periodic fever syndrome403833009
- Tumor necrosis factor receptor-associated periodic fever syndrome403833009
- Tumour necrosis factor (TNF) receptor-associated periodic fever syndrome403833009
- Tumour necrosis factor receptor-associated periodic fever syndrome403833009
- HIDS - hyper-IgD periodic fever syndrome403834003
- Hyper-IgD periodic fever syndrome403834003
- Hyper-immunoglobulin D periodic fever syndrome403834003
- Hyperimmunoglobulinaemia D with periodic fever403834003
- Hyperimmunoglobulinemia D with periodic fever403834003
- Periodic fever Dutch type403834003
- Aphthous stomatitis426965005
- Aphthous ulcer of mouth426965005
- Canker sore426965005
- Oral aphthae426965005
- Haemophagocytic lymphohistiocytosis with rheumatologic disease430478003
- Hemophagocytic lymphohistiocytosis with rheumatologic disease430478003
- Macrophage activation syndrome430478003
- Reactive haemophagocytic syndrome430478003
- Reactive hemophagocytic syndrome430478003
- Marshall syndrome with periodic fever717231003
- PFAPA syndrome717231003
- Periodic fever and aphthous stomatitis with pharyngitis and cervical lymphadenitis syndrome717231003
- Recurrent mouth ulcer723177002
- Recurrent oral ulceration723177002
- Recurrent ulcer of mouth723177002
- Congenital sideroblastic anaemia, B-cell immunodeficiency, periodic fever, developmental delay syndrome765327005
- Congenital sideroblastic anemia, B-cell immunodeficiency, periodic fever, developmental delay syndrome765327005
- FCAS2 - familial cold autoinflammatory syndrome type 2783146009
- Familial cold autoinflammatory syndrome type 2783146009
- NLR family pyrin domain containing 12-associated hereditary periodic fever syndrome783146009
- NLRP12-associated hereditary periodic fever syndrome783146009
- Secondary systemic amyloidosis858580008
- Luteal phase dependent febrile episode1169366007
- Luteal phase dependent periodic fever1169366007
- Menstrual cycle dependent febrile episode1169366007
- Menstrual cycle dependent periodic fever1169366007
- NLRC4-related autoinflammatory syndrome with macrophage activation syndrome1197594000
- NLRC4-related infantile enterocolitis, autoinflammatory syndrome1197594000
- NLRC4-related macrophage activation syndrome1197594000
- Periodic fever, infantile enterocolitis, autoinflammatory syndrome1197594000
- FCAS4 - familial cold autoinflammatory syndrome 41264006004
- NLR family caspase recruitment domain-containing 4-related familial cold autoinflammatory syndrome1264006004
- NLRC4-related familial cold autoinflammatory syndrome1264006004
- NLRC4-related familial cold urticaria1264006004
- Complex multigenic autoinflammatory syndrome1295181006
UMLS
- Benign Paroxysmal PeritonitidesC0031069
- Benign Paroxysmal PeritonitisC0031069
- Benign paroxysmal peritonitisC0031069
- Disease, PeriodicC0031069
- Disease, Wolff PeriodicC0031069
- Disease, Wolff's PeriodicC0031069
- Diseases, PeriodicC0031069
- FAMILIAL MEDITERRANEAN FEVERC0031069
- FMFC0031069
- FMF - Familial Mediterranean feverC0031069
- Familial Mediterranean FeverC0031069
- Familial Mediterranean feverC0031069
- Familial Mediterranean fever (disorder)C0031069
- Familial Paroxysmal PolyserositidesC0031069
- Familial Paroxysmal PolyserositisC0031069
- Familial mediterranean feverC0031069
- Familial paroxysmal polyserositisC0031069
- Familial recurrent polyserositisC0031069
- MEFC0031069
- MEF - Familial Mediterranean feverC0031069
- Mediterranean Fever, FamilialC0031069
- PERIODIC DISC0031069
- PERIODIC DIS WOLFFSC0031069
- POLYSEROSITIS, FAMILIAL PAROXYSMALC0031069
- POLYSEROSITIS, RECURRENTC0031069
- Paroxysmal Peritonitides, BenignC0031069
- Paroxysmal Peritonitis, BenignC0031069
- Paroxysmal Polyserositides, FamilialC0031069
- Paroxysmal Polyserositis, FamilialC0031069
- Paroxysmal polyserositisC0031069
- Periodic DiseaseC0031069
- Periodic Disease, WolffC0031069
- Periodic Disease, Wolff'sC0031069
- Periodic Disease, WolffsC0031069
- Periodic DiseasesC0031069
- Periodic PeritonitidesC0031069
- Periodic PeritonitisC0031069
- Periodic diseaseC0031069
- Periodic familial peritonitisC0031069
- Periodic peritonitisC0031069
- Periodic polyserositisC0031069
- Peritonitides, Benign ParoxysmalC0031069
- Peritonitides, PeriodicC0031069
- Peritonitis, Benign ParoxysmalC0031069
- Peritonitis, PeriodicC0031069
- Polyserositides, Familial ParoxysmalC0031069
- Polyserositides, RecurrentC0031069
- Polyserositis, Familial ParoxysmalC0031069
- Polyserositis, RecurrentC0031069
- Recurrent PolyserositidesC0031069
- Recurrent PolyserositisC0031069
- Recurrent polyserositisC0031069
- Reimann periodic diseaseC0031069
- WOLFF PERIODIC DISC0031069
- WOLFFS PERIODIC DISC0031069
- Wolff Periodic DiseaseC0031069
- Wolff periodic diseaseC0031069
- Wolff's Periodic DiseaseC0031069
- Wolffs Periodic DiseaseC0031069
- armenian diseaseC0031069
- familial Mediterranean feverC0031069
- familial mediterranean feverC0031069
- fmfC0031069
- mediterranean familial feverC0031069
- mediterranean fever familialC0031069
- periodic diseaseC0031069
- periodic diseasesC0031069
- recurrent polyserositisC0031069
- Deficiency of mevalonate kinaseC0342731
- Deficiency of mevalonate kinase (disorder)C0342731
- Kinase Deficiencies, MevalonateC0342731
- Kinase Deficiency, MevalonateC0342731
- Mevalonate Kinase DeficienciesC0342731
- Mevalonate Kinase DeficiencyC0342731
- Mevalonate kinase deficiencyC0342731
- Mevalonic AciduriaC0342731
- Hyperimmunoglobin D syndromeC4268690
- Periodic Fever SyndromeC3889979
- Periodic fever syndromesC3889979
- Tumor necrosis factor receptor associated periodic syndrome [TRAPS]C4268691
Frequently Asked Questions
What is ICD-10 code M04.1?
ICD-10-CM code M04.1 represents "Periodic fever syndromes". It is a billable/specific code that can be used on a claim.
Is M04.1 a billable code?
Yes, M04.1 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.
What chapter is M04.1 in?
M04.1 is in Chapter 13: Diseases of the Musculoskeletal System and Connective Tissue (codes M00-M99).
What SNOMED CT codes does M04.1 map to?
M04.1 maps to 21 SNOMED CT concepts: 367528006, 426965005, 12579009, 3502005, 1295181006, and 16 more. SNOMED CT is a clinical terminology used in electronic health records.
What are the UMLS CUIs for M04.1?
M04.1 is linked to 5 UMLS Concept Unique Identifiers: C0031069, C0342731, C4268690, C3889979, C4268691. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.
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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.