E85.1
BillableNeuropathic 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
Neuropathic heredofamilial amyloidosis is a genetic disorder causing abnormal protein deposits that damage nerves, leading to progressive weakness and loss of sensation, often starting in the feet.
Coding Tips
- •Confirm the presence of neuropathy symptoms in documentation; this distinguishes it from non-neuropathic amyloidosis
- •Code any associated organ involvement separately if significant (e.g., cardiac amyloidosis)
Clinical Significance
Neuropathic heredofamilial amyloidosis, including familial amyloid polyneuropathy (FAP), involves progressive peripheral and autonomic neuropathy caused by inherited mutations (most commonly transthyretin). Disease-modifying therapies like tafamidis and gene-silencing agents make accurate diagnosis and coding essential for treatment access and risk adjustment.
Documentation Requirements
- ✓Confirmed amyloidosis diagnosis with tissue biopsy or genetic confirmation
- ✓Hereditary/familial etiology established (TTR mutation, other amyloidogenic mutation)
- ✓Documentation of neuropathic manifestations (peripheral neuropathy, autonomic neuropathy)
- ✓Nerve conduction studies or other neurologic assessment
- ✓Current disease-modifying therapy (tafamidis, patisiran, inotersen)
- ✓Assessment of non-neurologic organ involvement (cardiac, renal, GI)
Commonly Confused Codes
E85.0 — Non-neuropathic heredofamilial amyloidosis: hereditary form WITHOUT neuropathy as primary featureE85.82 — Wild-type transthyretin-related (ATTR) amyloidosis: age-related, NOT hereditaryE85.81 — Light chain (AL) amyloidosis: acquired form from plasma cell disorderG63 — Polyneuropathy in diseases classified elsewhere: may be used additionally but does not replace the amyloidosis code