2024 ICD-10-CM Diagnosis Code M04.1

Periodic fever syndromes

ICD-10-CM Code:
ICD-10 Code for:
Periodic fever syndromes
Is Billable?
Yes - Valid for Submission
Chronic Condition Indicator: [1]
Code Navigator:

Code Classification

  • Diseases of the musculoskeletal system and connective tissue
    • Autoinflammatory syndromes
      • Autoinflammatory syndromes

M04.1 is a billable diagnosis code used to specify a medical diagnosis of periodic fever syndromes. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024.

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Amyloid of familial Mediterranean fever
  • Aphthous ulcer of mouth
  • Cervical lymphadenitis
  • Congenital sideroblastic anemia, B-cell immunodeficiency, periodic fever, developmental delay syndrome
  • Deficiency of mevalonate kinase
  • Familial Mediterranean fever
  • Hemolytic erythrophagocytic syndrome
  • Hemophagocytic lymphohistiocytosis
  • Hereditary periodic fever
  • Hyperimmunoglobulinemia D with periodic fever
  • Macrophage activation syndrome
  • Menstrual cycle dependent periodic fever
  • NLRP12-associated hereditary periodic fever syndrome
  • Pericarditis due to familial Mediterranean fever
  • Periodic fever, infantile enterocolitis, autoinflammatory syndrome
  • PFAPA syndrome
  • Recurrent aphthous ulcer
  • Recurrent ulcer of mouth
  • Secondary systemic amyloidosis
  • TNF receptor-associated periodic fever syndrome

Clinical Classification

Clinical Information

  • Familial Mediterranean Fever

    a group of hereditary autoinflammation diseases, characterized by recurrent fever, abdominal pain, headache, rash, pleurisy; and arthritis. orchitis; benign meningitis; and amyloidosis may also occur. homozygous or compound heterozygous mutations in marenostrin gene encoding pyrin result in autosomal recessive transmission; simple heterozygous, autosomal dominant form of the disease also exists with mutations in the same gene.
  • Macrophage Activation Syndrome

    a serious complication of childhood systemic inflammatory disorders that is thought to be caused by excessive activation and proliferation of t-lymphocytes and macrophages. it is seen predominantly in children with systemic onset juvenile idiopathic arthritis.
  • Cervical Lymphadenitis

    inflammation of the cervical lymph nodes.

Tabular List of Diseases and Injuries

The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.

Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Familial Mediterranean fever
  • Hyperimmunoglobin D syndrome
  • Mevalonate kinase deficiency
  • Tumor necrosis factor receptor associated periodic syndrome TRAPS

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Replacement Code

M041 replaces the following previously assigned ICD-10-CM code(s):

  • E85.0 - Non-neuropathic heredofamilial amyloidosis

Convert M04.1 to ICD-9-CM

  • ICD-9-CM Code: 277.31 - Fam Mediterranean fever
    Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018


[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.