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Calciphylaxis

Fatima M. Kazi, MD, Amar R. Chadaga, MD and Edgar V. Lerma, MD, FACP, FASN, FPSN (Hon) Reviewed 06/2019
 


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 based on clinical suspicion with characteristic skin findings in ESRD patients with risk factors.

  • Based on expert opinion, an interdisciplinary approach is recommended ...

TREATMENT

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

  • Pharmacologic mobilization/solubilization of Ca from involved tissue

  • Chelation of Ca and P by IV thiosulfate (emerging as a standard of care based o...

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 (6...

REFERENCES

1
Fine A, Zacharias J. Calciphylaxis is usually non-ulcerating: risk factors, outcome and therapy. Kidney Int.  2002;61(6):2210–2217. [View Abstract on OvidMedline]
2
Nigwekar SU, Thadhan...

ADDITIONAL READING

  • el-Azhary RA, Arthur AK, Davis MD, et al. Retrospective analysis of tissue plasminogen activator as an adjuvant treatment for calciphylaxis. JAMA Dermatol.  2013;149(1):63–67. ...

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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