ICD-10-CM Code M04.1

Periodic fever syndromes

Version 2020 Billable Code

Valid for Submission

M04.1 is a billable code used to specify a medical diagnosis of periodic fever syndromes. The code is valid for the year 2020 for the submission of HIPAA-covered transactions. The ICD-10-CM code M04.1 might also be used to specify conditions or terms like amyloid of familial mediterranean fever, aphthous ulcer of mouth, congenital sideroblastic anemia, b-cell immunodeficiency, periodic fever, developmental delay syndrome, deficiency of mevalonate kinase, familial mediterranean fever, hereditary inflammatory disorder involving skin, etc

Short Description:Periodic fever syndromes
Long Description:Periodic fever syndromes

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code M04.1:

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

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code M04.1 are found in the index:


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
  • Congenital sideroblastic anemia, B-cell immunodeficiency, periodic fever, developmental delay syndrome
  • Deficiency of mevalonate kinase
  • Familial Mediterranean fever
  • Hereditary inflammatory disorder involving skin
  • Hereditary periodic fever
  • Hyper-IgD periodic fever syndrome
  • Pericarditis associated with familial Mediterranean fever
  • Pericarditis due to metabolic disease
  • PFAPA syndrome
  • TNF receptor-associated periodic fever syndrome

Diagnostic Related Groups

The ICD-10 code M04.1 is grouped in the following groups for version MS-DRG V37.0 What are Diagnostic Related Groups?
The Diagnostic Related Groups (DRGs) are a patient classification scheme which provides a means of relating the type of patients a hospital treats. The DRGs divides all possible principal diagnoses into mutually exclusive principal diagnosis areas referred to as Major Diagnostic Categories (MDC).
applicable from 10/01/2019 through 09/30/2020.


Replacement Code

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

  • E85.0 - Non-neuropathic heredofamilial amyloidosis

Convert M04.1 to ICD-9

  • 277.31 - Fam Mediterranean fever (Approximate Flag)

Code Classification

  • Diseases of the musculoskeletal system and connective tissue (M00–M99)
    • Autoinflammatory syndromes (M04)
      • Autoinflammatory syndromes (M04)

Code History

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