ICD-10 Diagnosis Code M04.1

Diagnostic Related Groups
The diagnosis code M04.1 is grouped in the following Diagnostic Related Group(s) (MS-DRG V35.0)

• 545 - CONNECTIVE TISSUE DISORDERS WITH MCC
• 546 - CONNECTIVE TISSUE DISORDERS WITH CC
• 547 - CONNECTIVE TISSUE DISORDERS WITHOUT CC/MCC

Convert to ICD-9

• 277.31 - Fam Mediterranean fever (Approximate Flag)

Synonyms

• Amyloid of familial Mediterranean fever
• Aphthous ulcer of mouth
• Deficiency of mevalonate kinase
• Familial Mediterranean fever
• Hereditary inflammatory disorder involving skin
• Hereditary periodic fever
• Hyper-IgD periodic fever syndrome
• Inflammatory hereditary disorder
• Pericarditis associated with familial Mediterranean fever
• Pericarditis due to metabolic disease
• PFAPA syndrome
• TNF receptor-associated periodic fever syndrome

Index to Diseases and Injuries
References found for the code M04.1 in the Index to Diseases and Injuries:

• - Arthritis, arthritic (acute) (chronic) (nonpyogenic) (subacute) - M19.90
  • - in (due to)
    • - Mediterranean fever, familial - See Also: subcategory M14.8-; - M04.1

• - Deficiency, deficient
  • - mevalonate kinase - M04.1

• - Fever (inanition) (of unknown origin) (persistent) (with chills) (with rigor) - R50.9
  • - Mediterranean - See Also: Brucellosis; - A23.9
    • - familial - M04.1
  • - periodic (Mediterranean) - M04.1

• - Mediterranean
  • - fever - See: Brucellosis;
    • - familial - M04.1

• - Syndrome - See Also: Disease;
  • - hyperimmunoglobulin D - M04.1
  • - periodic fever - M04.1
  • - tumor necrosis factor receptor associated periodic (TRAPS) - M04.1

• - TRAPS (tumor necrosis factor receptor associated periodic syndrome) - M04.1

Tabular List of Diseases and Injuries
References found for the code M04.1 in the Tabular List of Diseases and Injuries:

• Inclusion Terms:
  • Familial Mediterranean fever
  • Hyperimmunoglobin D syndrome
  • Mevalonate kinase deficiency
  • Tumor NEC rosis factor receptor associated periodic syndrome [TRAPS]

Replacement Code
This code replaces the following previously assigned ICD-10 code(s) listed below:

• E85.0 - Non-neuropathic heredofamilial amyloidosis

General Equivalence Map Definitions
The ICD-10 and ICD-9 GEMs are used to facilitate linking between the diagnosis codes in ICD-9-CM and the new ICD-10-CM code set. The GEMs are the raw material from which providers, health information vendors and payers can derive specific applied mappings to meet their needs.

• Approximate Flag - The approximate flag is on, indicating that the relationship between the code in the source system and the code in the target system is an approximate equivalent.
• No Map Flag - The no map flag indicates that a code in the source system is not linked to any code in the target system.
• Combination Flag - The combination flag indicates that more than one code in the target system is required to satisfy the full equivalent meaning of a code in the source system.

Index of Diseases and Injuries Definitions

• And - The word "and" should be interpreted to mean either "and" or "or" when it appears in a title.
• Code also note - A "code also" note instructs that two codes may be required to fully describe a condition, but this note does not provide sequencing direction.
• Code first - Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. For such conditions, the ICD-10-CM has a coding convention that requires the underlying condition be sequenced first followed by the manifestation. Wherever such a combination exists, there is a "use additional code" note at the etiology code, and a "code first" note at the manifestation code. These instructional notes indicate the proper sequencing order of the codes, etiology followed by manifestation.
• Type 1 Excludes Notes - A type 1 Excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
• Type 2 Excludes Notes - A type 2 Excludes note represents "Not included here". An excludes2 note indicates that the condition excluded is not part of the condition represented by the code, but a patient may have both conditions at the same time. When an Excludes2 note appears under a code, it is acceptable to use both the code and the excluded code together, when appropriate.
• Includes Notes - This note appears immediately under a three character code title to further define, or give examples of, the content of the category.
• Inclusion terms - List of terms is included under some codes. 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.
• NEC "Not elsewhere classifiable" - This abbreviation in the Alphabetic Index represents "other specified". When a specific code is not available for a condition, the Alphabetic Index directs the coder to the "other specified” code in the Tabular List.
• NOS "Not otherwise specified" - This abbreviation is the equivalent of unspecified.
• See - The "see" instruction following a main term in the Alphabetic Index indicates that another term should be referenced. It is necessary to go to the main term referenced with the "see" note to locate the correct code.
• See Also - A "see also" instruction following a main term in the Alphabetic Index instructs that there is another main term that may also be referenced that may provide additional Alphabetic Index entries that may be useful. It is not necessary to follow the "see also" note when the original main term provides the necessary code.
• 7th Characters - Certain ICD-10-CM categories have applicable 7th characters. The applicable 7th character is required for all codes within the category, or as the notes in the Tabular List instruct. The 7th character must always be the 7th character in the data field. If a code that requires a 7th character is not 6 characters, a placeholder X must be used to fill in the empty characters.
• With - The word "with" should be interpreted to mean "associated with" or "due to" when it appears in a code title, the Alphabetic Index, or an instructional note in the Tabular List. The word "with" in the Alphabetic Index is sequenced immediately following the main term, not in alphabetical order.

Present on Admission
The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement.