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Calciphylaxis

Fatima M. Kazi, MD, Edgar V. Lerma, MD, FACP, FASN, FAHA, FASH, FNLA, FNKF, FASDIN, FPSN (Hon) and Amar R. Chadaga, MD Reviewed 06/2018
 


BASICS

DESCRIPTION

  • Rare, highly morbid, obliterative vasculopathy, characterized by ischemic or necrotic skin lesions

  • Affects patients with end-stage renal disease (ESRD)—calcific uremic arteriolopathy ...

DIAGNOSIS

  • Diagnosis is primarily clinical by characteristic skin findings in ESRD patients with risk factors.

  • Lab studies are not useful for diagnostic purposes.

  • Imaging studies demonstrating tissue cal...

TREATMENT

  • No FDA medications specifically approved for calciphylaxis

  • Aggressive control of Ca, P, and PTH (2)[C]

  • Pharmacologic mobilization/solubilization of Ca from involved tissue

  • Chelation of Ca and P...

ONGOING CARE

PROGNOSIS

  • High morbidity and mortality

    • Infectious morbidity within weeks of diagnosis and death within months are common.

    • 1-Year mortality 45–80%

    • Higher mortality with ulcerative lesions (65–...

REFERENCES

1
Fine A, Zacharias J. Calciphylaxis is usually non-ulcerating: risk factors, outcome and therapy. Kidney Int.  2002;61(6):2210–2217. {L-End} [View Abstract on OvidMedline]
2
Block GA. P...

ADDITIONAL READING

  • Araya CE, Fennell RS, Neiberger RE, et al. Sodium thiosulfate treatment for calcific uremic arteriolopathy in children and young adults. Clin J Am Soc Nephrol.  2006;1(6):1161–...

CODES

ICD10

E83.59 Other disorders of calcium metabolism 

ICD9

275.49 Other disorders of calcium metabolism 

SNOMED

237900002 Calciphylaxis (disorder) 

CLINICAL PEARLS

  • Calciphylaxis is a highly morbid systemic condition of uncertain pathogenesis, primarily affecting the skin that is almost unique to the ESRD population, often confused with ulcerative...

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