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Calciphylaxis: a still unmet challenge.
J Nephrol 2011 Mar-Apr; 24(2):142-8JN

Abstract

INTRODUCTION

Calcific uremic arteriolopathy (CUA), also known as calciphylaxis, is a rare disease most frequently occurring in patients with advanced chronic kidney disease (CKD). The clinical picture is typically characterized by very painful skin lesions and ulcerations following calcification and occlusion of small cutaneous arterioles. CUA is life-threatening due to infections and concomitant cardiovascular diseases.

METHODS

We performed a literature search for the terms calciphylaxis and calcific uremic arteriolopathy and summarized current state-of-the-art knowledge about pathophysiology, clinical picture, course of the disease, as well as treatment options. We have filled out the literature data with our personal treatment experiences.

RESULTS

A combination of various local and systemic risk factors are necessary to cause the development of calciphylaxis. This pathophysiological cascade is still incompletely understood. Patients with advanced CKD and dialysis patients are especially at risk to develop CUA. Regarding therapy, no randomized prospective trials are available, and treatment is rather based on pathophysiological considerations as well as on evidence derived from case reports or case series. Therapy focuses on optimized dialysis treatment, control of chronic kidney disease-mineral and bone disorder parameters, experimental anticalcification strategies and wound care.

CONCLUSION

Facing the still deleterious outcome of patients with calciphylaxis, further studies on prophylaxis as well as treatment are urgently needed. Current treatment strategies may help ameliorate the course of the disease in some patients. However, it is still unclear if they are able to decrease mortality.

Authors+Show Affiliations

Department of Cardiology, University Hospital Aachen, Aachen, Germany. Vincent.Brandenburg@post.rwth-aachen.deNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

21337312

Citation

Brandenburg, Vincent M., et al. "Calciphylaxis: a Still Unmet Challenge." Journal of Nephrology, vol. 24, no. 2, 2011, pp. 142-8.
Brandenburg VM, Cozzolino M, Ketteler M. Calciphylaxis: a still unmet challenge. J Nephrol. 2011;24(2):142-8.
Brandenburg, V. M., Cozzolino, M., & Ketteler, M. (2011). Calciphylaxis: a still unmet challenge. Journal of Nephrology, 24(2), pp. 142-8.
Brandenburg VM, Cozzolino M, Ketteler M. Calciphylaxis: a Still Unmet Challenge. J Nephrol. 2011;24(2):142-8. PubMed PMID: 21337312.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Calciphylaxis: a still unmet challenge. AU - Brandenburg,Vincent M, AU - Cozzolino,Mario, AU - Ketteler,Markus, PY - 2010/01/05/accepted PY - 2011/2/22/entrez PY - 2011/2/22/pubmed PY - 2011/7/20/medline SP - 142 EP - 8 JF - Journal of nephrology JO - J. Nephrol. VL - 24 IS - 2 N2 - INTRODUCTION: Calcific uremic arteriolopathy (CUA), also known as calciphylaxis, is a rare disease most frequently occurring in patients with advanced chronic kidney disease (CKD). The clinical picture is typically characterized by very painful skin lesions and ulcerations following calcification and occlusion of small cutaneous arterioles. CUA is life-threatening due to infections and concomitant cardiovascular diseases. METHODS: We performed a literature search for the terms calciphylaxis and calcific uremic arteriolopathy and summarized current state-of-the-art knowledge about pathophysiology, clinical picture, course of the disease, as well as treatment options. We have filled out the literature data with our personal treatment experiences. RESULTS: A combination of various local and systemic risk factors are necessary to cause the development of calciphylaxis. This pathophysiological cascade is still incompletely understood. Patients with advanced CKD and dialysis patients are especially at risk to develop CUA. Regarding therapy, no randomized prospective trials are available, and treatment is rather based on pathophysiological considerations as well as on evidence derived from case reports or case series. Therapy focuses on optimized dialysis treatment, control of chronic kidney disease-mineral and bone disorder parameters, experimental anticalcification strategies and wound care. CONCLUSION: Facing the still deleterious outcome of patients with calciphylaxis, further studies on prophylaxis as well as treatment are urgently needed. Current treatment strategies may help ameliorate the course of the disease in some patients. However, it is still unclear if they are able to decrease mortality. SN - 1724-6059 UR - https://www.unboundmedicine.com/medline/citation/21337312/Calciphylaxis:_a_still_unmet_challenge_ L2 - http://ovidsp.ovid.com/ovidweb.cgi?T=JS&PAGE=linkout&SEARCH=21337312.ui DB - PRIME DP - Unbound Medicine ER -