Program Operations Manual System (POMS)
TN 57 (08-23)
COMPASSIONATE ALLOWANCE
INFORMATION
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CALCIPHYLAXIS
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ALTERNATE NAMES
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Calcific Uremic Arteriolopathy; Uremic Gangrene Syndrome
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DESCRIPTION
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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.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM
CODING
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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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Skin lesions and sores; and
ICD-9: 275.49
ICD-10: E83.59
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PROGRESSION
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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.
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TREATMENT
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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.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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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.
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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6.03
6.04
6.09
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Equals
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6.09
8.04
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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.
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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