Calciphylaxis: no therapeutic concepts for a poorly understood syndrome?
Calciphylaxis is a very uncommon and severe disease which mainly appears in patients with chronic renal insufficiency. It presents with ischemia and necrosis of the skin, subcutaneous adipose tissue, muscles and rarely viscera. The pathogenetic mechanisms inducing calciphylaxis are for the most part unknown. The mortality rate of 80% in the first year is very high. Patients experience marked pain, recurrent infections and the constant risk of secondary sepsis. Even multidisciplinary therapeutic strategies are limited, although there are recent case reports providing promising new therapeutic options including sodium thiosulfate and cinacalcet. This review summarizes the important aspects of diagnosis, pathogenesis, prevention and the possible therapeutic strategies of this intriguing, rare and often fatal disease.
Meissner
Markus
M
Dept. of Dermatology, Johann Wolfgang Goethe University, Frankfurt am Main, Germany. meissner.markus@gmx.de
Gille
Jens
J
Kaufmann
Roland
R
D000367:Age Factors; D002115:Calciphylaxis / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology / Q000401:mortality / Q000473:pathology / Q000517:prevention & control / Q000601:surgery / Q000628:therapy; D002118:Calcium / Q000097:blood; D000069449:Cinacalcet; D003924:Diabetes Mellitus, Type 2 / Q000150:complications; D005260:Female; D006801:Humans; D006931:Hyperbaric Oxygenation; D006961:Hyperparathyroidism / Q000150:complications; D015994:Incidence; D007676:Kidney Failure, Chronic / Q000150:complications; D008297:Male; D008875:Middle Aged; D009281:Naphthalenes / Q000627:therapeutic use; D009765:Obesity / Q000150:complications; D016105:Parathyroidectomy; D010710:Phosphates / Q000097:blood; D006435:Renal Dialysis / Q000009:adverse effects; D012307:Risk Factors; D012737:Sex Factors; D013885:Thiosulfates / Q000627:therapeutic use; D013997:Time Factors
D009281:Naphthalenes; D010710:Phosphates; D013885:Thiosulfates; C017717:sodium thiosulfate; D002118:Calcium; D000069449:Cinacalcet