ALTERNATE NAMES

Amyloidosis Associated Kidney Disease; Kidney Amyloidosis; Kidney Amyloidosis AL Type; Renal AL Amyloidosis; Renal Amyloidosis

DESCRIPTION

Amyloidosis is a rare disease that occurs when amyloid proteins are deposited in tissues and organs. Amyloid proteins are abnormal proteins that the body cannot break down and recycle, as it does with normal proteins. When amyloid proteins clump together, they form amyloid deposits. The buildup of these deposits damages a person’s organs and tissues. The kidneys are the organs most commonly affected by primary amyloidosis.

In Renal amyloidosis - AL type, amyloid deposits damage the kidneys and make it harder for them to filter wastes and break down proteins. When the kidneys become too damaged, they may no longer be able to function well enough to maintain health, resulting in kidney failure. Kidney failure can lead to problems such as high blood pressure, bone disease, and anemia.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9/ICD-10-CM CODING

Diagnostic testing: The diagnosis of renal amyloidosis is made by:

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  Medical and family history;

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  Physical exam;

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  Urinalysis;

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  Blood tests; and

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  Kidney biopsy.

Physical findings: The physical findings include:

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  Albuminuria (increased amount of albumin, a protein, in the urine);

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  Hyperlipidemia (blood has more-than-normal amounts of fats and cholesterol);

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  Edema (swelling of the legs, feet, or ankles and less often the hands or face); and

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  Hypoalbuminemia (less-than-normal amounts of albumin).

Other signs and symptoms of primary amyloidosis may include:

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  Fatigue or feeling tired;

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  Shortness of breath;

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  Low blood pressure;

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  Numbness, tingling, or a burning sensation in the hands or feet; and

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  Unintentional weight loss.

ICD-9: 277.3; 277.39

ICD-10: E85.4

PROGRESSION

Amyloidosis that affects the kidneys leads to life-threatening kidney failure and end-stage renal disease. Renal amyloidosis - AL type has very poor prognosis with most individuals dying in 2-3 years from diagnosis.

TREATMENT

Renal amyloidosis - AL type is difficult to treat, and treatment is generally only marginally effective. Treatment may consist of medication therapy, chemotherapy, and stem cell transplant. Chemotherapy and bone marrow or stem cell transplant tend to be the most effective therapies.

Suggested MER for Evaluation:

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  Clinical history and examination that describes the diagnostic features of renal involvement;

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  Neuroimaging; and

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  Tissue biopsy.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

6.05 A

Only the conditions of listing 6.05 A must be met.

14.03

14.07 B

Use listing 14.07 B when a stem cell or bone marrow transplant has been completed.