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Calciphylaxis: a review.

Cynthia M Magro1, Richard Simman2, Sarah Jackson3

  • 1Department of Pathology and Laboratory Medicine, Weil Medical College of Cornell University, New York, NY 10065, USA.

The Journal of the American College of Certified Wound Specialists
|February 15, 2014
PubMed
Summary
This summary is machine-generated.

Human calciphylaxis is a severe vascular disease causing tissue damage, often seen in kidney failure. Research is exploring its varied causes and new treatments like sodium thiosulfate.

Keywords:
CalciphylaxisCalcium depositionMicroangiopathySkin necrosis

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Area of Science:

  • Vascular Biology
  • Nephrology
  • Dermatology

Background:

  • Calciphylaxis is a severe microangiopathy causing tissue compromise.
  • It presents with vascular thrombotic occlusion and endoluminal calcification.
  • While common in renal failure, it occurs in multiple myeloma, POEMS syndrome, cirrhosis, and rheumatoid arthritis.

Purpose of the Study:

  • To elucidate the pathophysiology of human calciphylaxis.
  • To describe the histomorphologic spectrum of the disease.
  • To identify factors influencing outcomes and potential therapeutic targets.

Main Methods:

  • Review of clinical and histopathological findings in calciphylaxis.
  • Analysis of contributing factors such as procoagulant states and ectopic calcification.
  • Evaluation of emerging therapeutic strategies.

Main Results:

  • Calciphylaxis involves occlusive microangiopathy with calcification, but can present with thrombosis alone or pseudoangiosarcomatous changes.
  • Pathophysiology involves procoagulant states and aberrant neo-osteogenesis (e.g., increased osteopontin, reduced fetuin).
  • Truncal/genital involvement and systemic forms are associated with adverse outcomes.

Conclusions:

  • Calciphylaxis is a complex microangiopathy with a diverse presentation.
  • Understanding its pathogenesis is key to developing effective treatments.
  • Emerging therapies targeting oxidative stress and inflammation show promise.