2025 ICD-10-CM Diagnosis Code E85.0

Non-neuropathic heredofamilial amyloidosis

• E85.0 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
• The 2025 edition of ICD-10-CM E85.0 became effective on October 1, 2024.
• This is the American ICD-10-CM version of E85.0 – other international versions of ICD-10 E85.0 may differ.

Applicable To

• Hereditary amyloid nephropathy

Code Also

• associated disorders, such as:
• autoinflammatory syndromes (M04.-)

Type 2 Excludes

• Transthyretin-related (ATTR) familial amyloid cardiomyopathy (E85.4)

The following code(s) above E85.0 contain annotation back-references

 that may be applicable to E85.0:

• E00-E89  Endocrine, nutritional and metabolic diseases
• E70-E88  Metabolic disorders
• E85  Amyloidosis

Approximate Synonyms

• Familial mediterranean fever
• Familial non-neuropathic amyloidosis
• Nonneuropathic heredofamilial amyloidosis

Clinical Information

• 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 result in autosomal recessive transmission; simple heterozygous, autosomal dominant form of the disease.
• A usually autosomal recessive inherited inflammatory disorder caused by mutations in the mefv gene. It is characterized by recurrent painful inflammatory attacks in the abdomen, joints, and chest. The inflammatory attacks are associated with fever.

ICD-10-CM E85.0 is grouped within Diagnostic Related Group(s) (MS-DRG v42.0):

• 545 Connective tissue disorders with mcc
• 546 Connective tissue disorders with cc
• 547 Connective tissue disorders without cc/mcc

Convert E85.0 to ICD-9-CMCode History

• 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
• 2017 (effective 10/1/2016): No change
• 2018 (effective 10/1/2017): No change
• 2019 (effective 10/1/2018): No change
• 2020 (effective 10/1/2019): No change
• 2021 (effective 10/1/2020): No change
• 2022 (effective 10/1/2021): No change
• 2023 (effective 10/1/2022): No change
• 2024 (effective 10/1/2023): No change
• 2025 (effective 10/1/2024): No change

Code annotations containing back-references to E85.0:

• Type 1 Excludes: K65, K65

Diagnosis Index entries containing back-references to E85.0:

• Amyloidosis (generalized) (primary) E85.9
  • non-neuropathic heredofamilial E85.0
• MyopathyG72.9
  • in (due to)
    • amyloidosis E85.0
• NephropathyN28.9 – see also Nephritis
  • amyloid, hereditary E85.0
• Peritonitis (adhesive) (bacterial) (fibrinous) (hemorrhagic) (idiopathic) (localized) (perforative) (primary) (with adhesions) (with effusion) K65.9
  • paroxysmal, familial E85.0
    • benign E85.0
  • periodic, familial E85.0
• Polyserositis
  • periodic, familial E85.0