AutoICD API

M04.1

Billable

Periodic fever syndromes

Periodic fever syndromes

Status

Billable / Specific

Block

M04-M04

Parent Code

M04

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

Related Codes(3)
M04.2

Cryopyrin-associated periodic syndromes

BillableM04.8

Other autoinflammatory syndromes

BillableM04.9

Autoinflammatory syndrome, unspecified

Billable
Also Known As / Clinical Terms(281)

SNOMED CT

UMLS

Clinical Terms

  • Hyper-immunoglobulin D periodic fever syndrome
  • FCAS2 - familial cold autoinflammatory syndrome type 2
  • NLRC4-related familial cold autoinflammatory syndrome
  • Polyserositis, Familial Paroxysmal
  • Periodic Fever Syndrome
  • Hemophagocytic lymphohistiocytosis
  • Amyloidosis due to familial Mediterranean fever
  • Mevalonic Aciduria
  • Hyperimmunoglobulinemia D with periodic fever
  • NLRC4-related macrophage activation syndrome
  • Deficiency of mevalonate kinase (disorder)
  • Periodic fever and aphthous stomatitis with pharyngitis and cervical lymphadenitis syndrome
  • NLRC4-related autoinflammatory syndrome with macrophage activation syndrome
  • PFAPA syndrome
  • Familial cold autoinflammatory syndrome type 2
  • fmf
  • Wolff periodic disease
  • Reimann periodic disease
  • Marshall syndrome with periodic fever
  • MEF
  • Polyserositides, Recurrent
  • Wolffs Periodic Disease
  • FMF - Familial Mediterranean fever
  • FAMILIAL MEDITERRANEAN FEVER
  • Amyloid of familial Mediterranean fever
  • Macrophage activation syndrome
  • Deficiency of mevalonate kinase
  • Disease, Wolff's Periodic
  • Kinase Deficiencies, Mevalonate
  • Benign Paroxysmal Peritonitis
  • Paroxysmal polyserositis
  • Hyperinflammatory lymphohistiocytosis
  • Familial recurrent polyserositis
  • Haemophagocytic lymphohistiocytosis with rheumatologic disease
  • Recurrent oral ulceration
  • NLR family caspase recruitment domain-containing 4-related familial cold autoinflammatory syndrome
  • Tumour necrosis factor receptor-associated periodic fever syndrome
  • Disease, Wolff Periodic
  • HIDS - hyper-IgD periodic fever syndrome
  • Haemophagocytic lymphohistiocytosis
  • Menstrual cycle dependent febrile episode
  • WOLFF PERIODIC DIS
  • Aphthous ulcer of mouth
  • Peritonitis, Benign Paroxysmal
  • Cervical lymphadenitis
  • Recurrent ulcer of mouth
  • PERIODIC DIS
  • Mevalonate Kinase Deficiencies
  • Periodic Disease, Wolffs
  • Benign Paroxysmal Peritonitides
  • Polyserositides, Familial Paroxysmal
  • Reactive hemophagocytic syndrome
  • Pericarditis due to familial Mediterranean fever
  • Recurrent polyserositis
  • Periodic familial peritonitis
  • Periodic Disease
  • Congenital sideroblastic anaemia, B-cell immunodeficiency, periodic fever, developmental delay syndrome
  • Secondary systemic amyloidosis
  • Complex multigenic autoinflammatory syndrome
  • Peritonitis, Periodic
  • NLRC4-related familial cold urticaria
  • Periodic fever Dutch type
  • Aphthous stomatitis
  • Canker sore
  • Disease, Periodic
  • POLYSEROSITIS, RECURRENT
  • Tumor necrosis factor receptor associated periodic syndrome [TRAPS]
  • Familial paroxysmal polyserositis
  • Hyperimmunoglobin D syndrome
  • Hyperimmunoglobulinaemia D with periodic fever
  • Mediterranean Fever, Familial
  • mediterranean fever familial
  • Luteal phase dependent febrile episode
  • Familial autosomal dominant periodic fever
  • Hereditary periodic fever
  • Menstrual cycle dependent periodic fever
  • TNF receptor-associated periodic fever syndrome (TRAPS)
  • Recurrent Polyserositides
  • Periodic polyserositis
  • Mevalonate kinase deficiency
  • Paroxysmal Peritonitis, Benign
  • Pericarditis associated with familial Mediterranean fever
  • Peritonitides, Benign Paroxysmal
  • Tumor necrosis factor (TNF) receptor-associated periodic fever syndrome
  • Reactive haemophagocytic syndrome
  • Paroxysmal Polyserositides, Familial
  • Recurrent mouth ulcer
  • PERIODIC DIS WOLFFS
  • Oral aphthae
  • NLRC4-related infantile enterocolitis, autoinflammatory syndrome
  • Haemophagocytic syndrome
  • Periodic fever, infantile enterocolitis, autoinflammatory syndrome
  • NLR family pyrin domain containing 12-associated hereditary periodic fever syndrome
  • armenian disease
  • Luteal phase dependent periodic fever
  • Tumor necrosis factor receptor-associated periodic fever syndrome
  • Familial Paroxysmal Polyserositides
  • Periodic Diseases
  • Cervical adenitis
  • Paroxysmal Peritonitides, Benign
  • FCAS4 - familial cold autoinflammatory syndrome 4
  • Hemophagocytic syndrome
  • MEF - Familial Mediterranean fever
  • Tumour necrosis factor (TNF) receptor-associated periodic fever syndrome
  • TRAPS - TNF receptor-associated periodic fever syndrome
  • Periodic Peritonitis
  • WOLFFS PERIODIC DIS
  • TNF receptor-associated periodic fever syndrome
  • Wolff's Periodic Disease
  • Periodic Peritonitides
  • Congenital sideroblastic anemia, B-cell immunodeficiency, periodic fever, developmental delay syndrome
  • Paroxysmal Polyserositis, Familial
  • Familial Mediterranean fever (disorder)
  • Peritonitides, Periodic
  • Hemophagocytic lymphohistiocytosis with rheumatologic disease
  • Kinase Deficiency, Mevalonate
  • Hyper-IgD periodic fever syndrome
  • Diseases, Periodic
  • NLRP12-associated hereditary periodic fever syndrome
  • Periodic Disease, Wolff's
  • mediterranean familial fever
  • Recurrent aphthous ulceration
  • Periodic Disease, Wolff
  • Recurrent aphthous ulcer
Frequently Asked Questions
What is the ICD-10 code for periodic fever syndromes?

The ICD-10-CM code for periodic fever syndromes is M04.1. The full clinical description is "Periodic fever syndromes". M04.1 is a billable/specific code that can be used on insurance claims and medical billing.

What does ICD-10 code M04.1 mean?

ICD-10-CM code M04.1 represents “Periodic fever syndromes”. It is classified under Chapter 13: Diseases of the Musculoskeletal System and Connective Tissue and 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.

How does M04.1 relate to ICF functioning codes?

ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like periodic fever syndromes affect a person's functioning — body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.

What is the ICD-11 equivalent of M04.1?

There is no direct ICD-11 mapping available for M04.1 in the WHO crosswalk tables. This may mean the concept is classified differently in ICD-11. Use the ICD-10 to ICD-11 converter to search for related codes.

Automate ICD-10 Coding With AI

Send clinical text to the AutoICD API and get back structured ICD-10 codes with confidence scores. Integrates into any EHR or billing system in minutes.

Try FreeView API Docs

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.