E85.0
BillableNon-neuropathic heredofamilial amyloidosis
HCC Category Mapping
V28HCC 50 — Glycogen/Amino-Acid/Other Metabolic Disorders
0.289V24HCC 23 — Other Significant Endocrine and Metabolic Disorders
0.230ESRDHCC 23 — Other Significant Endocrine and Metabolic Disorders
0.000What This Code Means
Non-neuropathic heredofamilial amyloidosis is a rare genetic disorder where abnormal protein deposits accumulate in organs like the heart and kidneys, but without nerve damage.
Coding Tips
- •Distinguish this from neuropathic amyloidosis (E85.1) by confirming absence of peripheral neuropathy in the clinical documentation
- •Document which organs are affected by amyloid deposits to support the diagnosis and treatment plan
Clinical Significance
Non-neuropathic heredofamilial amyloidosis includes familial Mediterranean fever-associated amyloidosis and other inherited forms that primarily affect organs other than the nervous system, such as kidneys, heart, and liver. These progressive protein misfolding diseases require ongoing monitoring and significantly impact patient care complexity.
Documentation Requirements
- ✓Confirmed amyloidosis diagnosis (tissue biopsy with Congo red staining or typing)
- ✓Hereditary/familial etiology established (genetic testing, family history)
- ✓Documentation that the form is non-neuropathic (organ involvement specified)
- ✓Affected organ systems and functional status (renal function, cardiac function)
- ✓Current treatment regimen (colchicine for FMF-associated, supportive care)
- ✓Monitoring plan for progressive organ involvement
Excludes 2 — Not included here, may code separately
- Transthyretin-related (ATTR) familial amyloid cardiomyopathy (E85.4)
Code Also
- associated disorders, such as:
- autoinflammatory syndromes (M04.-)
Commonly Confused Codes
E85.1 — Neuropathic heredofamilial amyloidosis: use when peripheral or autonomic neuropathy is the primary manifestationE85.81 — Light chain (AL) amyloidosis: acquired form from plasma cell dyscrasia, NOT hereditaryE85.82 — Wild-type transthyretin-related (ATTR) amyloidosis: acquired/age-related, not familialE85.4 — Organ-limited amyloidosis: use when amyloid deposits are confined to a single organ
Code Hierarchy
└E85Amyloidosis└E85.0Non-neuropathic heredofamilial amyloidosis
└E85.0Non-neuropathic heredofamilial amyloidosis