Non-neuropathic heredofamilial amyloidosis

This code identifies a group of inherited systemic amyloidosis disorders that are not primarily characterized by neurological involvement. These conditions result from the abnormal deposition of amyloid fibrils in various organs, leading to organ dysfunction, but specifically exclude those with a predominant neuropathic presentation.

Assign this code for patients diagnosed with a genetically determined, non-neuropathic form of amyloidosis. Documentation should clearly state the hereditary nature of the amyloidosis and specify the absence of significant neurological symptoms, or indicate that neurological involvement is not the primary clinical manifestation.

• Differentiate carefully from neuropathic forms of heredofamilial amyloidosis, which are classified elsewhere.
• Do not use this code for transthyretin-related (ATTR) familial amyloid cardiomyopathy, as it has a specific exclusion.
• Ensure genetic testing results or a confirmed family history of non-neuropathic amyloidosis are present in the medical record.