ICD-10 Diagnosis Code E85.1

Neuropathic heredofamilial amyloidosis

Diagnosis Code E85.1

ICD-10: E85.1
Short Description: Neuropathic heredofamilial amyloidosis
Long Description: Neuropathic heredofamilial amyloidosis
This is the 2017 version of the ICD-10-CM diagnosis code E85.1

Code Classification
  • Endocrine, nutritional and metabolic diseases
    • Metabolic disorders (E70-E88)
      • Amyloidosis (E85)

Information for Medical Professionals

Diagnostic Related Groups
The diagnosis code E85.1 is grouped in the following Diagnostic Related Group(s) (MS-DRG v33.0)


Convert to ICD-9 Additional informationCallout TooltipGeneral Equivalence Map
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.

  • Amyloid nephropathy
  • Amyloid polyneuropathy type I
  • Cerebral amyloid angiopathy
  • Cerebral amyloid angiopathy
  • Cerebrovascular amyloidosis
  • Familial amyloid nephropathy with urticaria AND deafness
  • Familial amyloid neuropathy, Finnish type
  • Familial amyloid polyneuropathy
  • Familial amyloid polyneuropathy with cutaneous amyloidosis
  • Familial amyloid polyneuropathy, Iowa type
  • Familial amyloid polyneuropathy, Jewish type
  • Familial amyloid polyneuropathy, type II
  • Familial amyloid polyneuropathy, type VI
  • Hereditary cerebral amyloid angiopathy, Icelandic type
  • Hereditary cystatin C amyloid angiopathy
  • Heredofamilial systemic amyloidosis affecting skin
  • Localized hereditary amyloidosis
  • Neuropathy associated with dysproteinemias
  • Neuropathy in secondary amyloidosis
  • Polyneuropathy in amyloidosis
  • Systemic amyloidosis affecting skin

Index of Diseases and Injuries
References found for the code E85.1 in the Index of Diseases and Injuries:

Information for Patients


Amyloidosis occurs when abnormal proteins called amyloids build up and form deposits. The deposits can collect in organs such as the kidney and heart. This can cause the organs to become stiff and unable to work the way they should.

There are three main types of amyloidosis:

  • Primary - with no known cause
  • Secondary - caused by another disease, including some types of cancer
  • Familial - passed down through genes

Symptoms can vary, depending upon which organs are affected. Treatment depends on the type of amyloidosis you have. The goal is to help with symptoms and limit the production of proteins. If another disease is the cause, it needs to be treated.

  • Cardiac amyloidosis
  • Hereditary amyloidosis
  • Primary amyloidosis
  • Secondary systemic amyloidosis

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Transthyretin amyloidosis Transthyretin amyloidosis is a slowly progressive condition characterized by the buildup of abnormal deposits of a protein called amyloid (amyloidosis) in the body's organs and tissues. These protein deposits most frequently occur in the peripheral nervous system, which is made up of nerves connecting the brain and spinal cord to muscles and sensory cells that detect sensations such as touch, pain, heat, and sound. Protein deposits in these nerves result in a loss of sensation in the extremities (peripheral neuropathy). The autonomic nervous system, which controls involuntary body functions such as blood pressure, heart rate, and digestion, may also be affected by amyloidosis. In some cases, the brain and spinal cord (central nervous system) are affected. Other areas of amyloidosis include the heart, kidneys, eyes, and gastrointestinal tract. The age at which symptoms begin to develop varies widely among individuals with this condition, and is typically between ages 20 and 70.There are three major forms of transthyretin amyloidosis, which are distinguished by their symptoms and the body systems they affect.The neuropathic form of transthyretin amyloidosis primarily affects the peripheral and autonomic nervous systems, resulting in peripheral neuropathy and difficulty controlling bodily functions. Impairments in bodily functions can include sexual impotence, diarrhea, constipation, problems with urination, and a sharp drop in blood pressure upon standing (orthostatic hypotension). Some people experience heart and kidney problems as well. Various eye problems may occur, such as cloudiness of the clear gel that fills the eyeball (vitreous opacity), dry eyes, increased pressure in the eyes (glaucoma), or pupils with an irregular or "scalloped" appearance. Some people with this form of transthyretin amyloidosis develop carpal tunnel syndrome, which is characterized by numbness, tingling, and weakness in the hands and fingers.The leptomeningeal form of transthyretin amyloidosis primarily affects the central nervous system. In people with this form, amyloidosis occurs in the leptomeninges, which are two thin layers of tissue that cover the brain and spinal cord. A buildup of protein in this tissue can cause stroke and bleeding in the brain, an accumulation of fluid in the brain (hydrocephalus), difficulty coordinating movements (ataxia), muscle stiffness and weakness (spastic paralysis), seizures, and loss of intellectual function (dementia). Eye problems similar to those in the neuropathic form may also occur. When people with leptomeningeal transthyretin amyloidosis have associated eye problems, they are said to have the oculoleptomeningeal form.The cardiac form of transthyretin amyloidosis affects the heart. People with cardiac amyloidosis may have an abnormal heartbeat (arrhythmia), an enlarged heart (cardiomegaly), or orthostatic hypertension. These abnormalities can lead to progressive heart failure and death. Occasionally, people with the cardiac form of transthyretin amyloidosis have mild peripheral neuropathy.
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