ALTERNATE NAMES

Calcific Uremic Arteriolopathy; Uremic Gangrene Syndrome

DESCRIPTION

Calciphylaxis is a rare metabolic disease in which calcium builds up in the walls of veins and arteries, blocking blood flow and resulting in damage to multiple organ systems. The denial of blood to tissues causes painful, infected sores on the skin that cannot heal without external intervention.

Although the exact cause is unknown, calciphylaxis is usually observed in people with end-stage renal disease (ESRD) who are on dialysis or have undergone a kidney transplant. When seen in people with normal kidney function, associated comorbidities tend to include inflammatory bowel disease, autoimmune disorders, or cancer.

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

Diagnostic testing: A diagnosis of calciphylaxis can be confirmed through skin biopsy, function tests of the kidneys and liver, and tests for concentration of calcium and other minerals in the blood. In some advanced cases involving ESRD, diagnosis is possible through visual inspection of wounds.

Physical signs and symptoms of calciphylaxis may include:

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  Fatigue;

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  Pain;

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  Muscle weakness;

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  Aches and cramps;

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  Vision problems;

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  Skin lesions and sores; and

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  Skin necrosis.

ICD-9: 275.49

ICD-10: E83.59

PROGRESSION

The prognosis for people with calciphylaxis is extremely poor. The condition tends to advance rapidly, causing intense pain and organ failure. The disease is usually fatal.

TREATMENT

There is no cure for calciphylaxis. Treatment is supportive and focused on pain relief and wound care.

Intravenous administration of sodium thiosulfate (STS) has shown some promise in dissolving calcium in the blood, improving blood flow, and promoting wound healing. However, this is an experimental, off-label use of STS; more study is required.

Suggested MER for Evaluation:

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  Clinical history and examination that describes the diagnostic features of the impairment; and

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  Laboratory tests confirming impaired liver and kidney function, and threshold levels of calcium on fat and blood.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

6.03

6.04

6.09

Equals

6.09

8.04

Calciphylaxis can equal listing 8.04 if the skin lesions produced by non-infectious conditions persist for at least 3 months despite continuing treatment as prescribed.