Diagnosis Code

M04.1 PERIODIC FEVER SYNDROMES


Code Information

Diagnosis Code: M04.1

Short Description: Periodic fever syndromes

Long Description: Periodic fever syndromes

The code M04.1 is VALID for claim submission

Code Classification:

  • Diseases of the musculoskeletal system and connective tissue (M00–M99)
    • Autoinflammatory syndromes (M04)
      • Autoinflammatory syndromes (M04)
        • M04.1 Periodic fever syndromes

Code Version: 2022 ICD-10-CM


Synonyms

  • 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

Diagnostic Related Group(s)

The code M04.1 is grouped in the following Diagnostic Related Group(s) (MS-DRG v39.0)
  • Connective Tissue Disorders With Mcc (545)
  • Connective Tissue Disorders With Cc (546)
  • Connective Tissue Disorders Without Cc/mcc (547)

References to Index of Diseases and Injuries

The code M04.1 has the following ICD-10-CM references to the Index of Diseases and Injuries
  • Inclusion Terms:
    • Familial Mediterranean fever
    • Hyperimmunoglobin D syndrome
    • Mevalonate kinase deficiency
    • Tumor necrosis factor receptor associated periodic syndrome [TRAPS]

Crosswalk Information

ICD-10 Code ICD-9 Code ICD-9 Description
M04.1 Right Arrow 277.31 Fam Mediterranean fever

This ICD-10 to ICD-9 data is based on the 2018 General Equivalency Mapping (GEM) files published by the Centers for Medicare & Medicaid Services (CMS) for informational purposes only. The data is not an ICD-10 conversion tool and doesn’t guarantee clinical accuracy.

Similar Codes

ICD-10 Code ICD-10 Description
M04 Autoinflammatory syndromes
M04.2 Cryopyrin-associated periodic syndromes
M04.8 Other autoinflammatory syndromes
M04.9 Autoinflammatory syndrome, unspecified


This page was last updated on: 10/1/2023