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Diseases of the Parathyroid Glands pp 113–146Cite as

Calcific Uremic Arteriolopathy (Calciphylaxis)

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Abstract

Calcific uremic arteriolopathy (CUA), also referred to as calciphylaxis, is a poorly understood syndrome of vascular calcification and cutaneous necrosis first described in 1898 when its presence was associated with uremia. At present, it is believed to be caused by arteriolar calcification and cutaneous plaque formation which causes vascular stenosis which predisposes to thrombotic occlusion causing tissue ischemia and violaceous skin lesions that may progress to nonhealing ulceration and gangrene which may lead to amputation, sepsis, and death. Although there are many reports of calciphylaxis associated with nonuremic causes, the majority of cases occur in patients with end-stage renal disease on dialysis. It is likely that uremia creates a favorable milieu for extraskeletal deposition of hydroxyapatite in soft tissues including small vessels, predisposing to thrombus formation, and the development of calciphylaxis. In this chapter, the authors present a discussion of the basic and clinical aspects of the disease process as well as novel therapeutic options.

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Abbreviations

25-(OH)D3 :

25-Hydroxyvitamin D3 (also known as: 25-hydroxycholecalciferol, calcifediol, or calcidiol)

1,25-(OH2)D3 :

1,25-Dihydroxyvitamin D3 (also known as: 1,25-dihydroxycholecalciferol or calcitriol)

ApoE:

Apolipoprotein E

BMP:

Bone morphogenic protein

CKD:

Chronic kidney disease

CKD-MBD:

Chronic kidney disease-mineral and bone disorder (also known as: renal osteodystrophy)

EGFRs:

Epidermal growth factor receptors

ESRD:

End-stage renal disease

FGF-23:

Fibroblast growth factor-23

HPT:

Hyperparathyroidism

IL-1, IL-6:

Interleukin-1 and -6

LDL:

Low-density lipoprotein

MGP:

Matrix Gla protein

OPG:

Osteoprotegerin

OPN:

Osteopontin

PTH:

Parathyroid hormone (also known as: parathormone or parathyrin)

TGF-α:

Transforming growth factor-alpha

TGF-β:

Transforming growth factor-beta

TNF-α:

Tumor necrosis factor-alpha

ucMGP:

Uncarboxylated matrix Gla protein

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  1. Department of Nephrology and Hypertension, Georgetown University Medical Center, 3800 Reservoir Road NW—6 PHC, Washington, DC, 20007, USA

    Earl H. Rudolph DO

  2. Department of Medicine, Section of Nephrology, University of Illinois at Chicago College of Medicine, 3543 Wisconsin Ave, Berwyn, IL, 60402, USA

    Edgar V. Lerma MD

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  1. , DEPTS BIOMED ENG & ENDOCRIN-METABOL, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, 44195-5036, Ohio, USA

    Angelo A. Licata

  2. Section of Nephrology, University of Illinois at, Department of Medicine, Associates in Nephrology, SC, Chicago, 60402, Illinois, USA

    Edgar V. Lerma

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Rudolph, E.H., Lerma, E.V. (2012). Calcific Uremic Arteriolopathy (Calciphylaxis). In: Licata, A., Lerma, E. (eds) Diseases of the Parathyroid Glands. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-5550-0_6

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