Proposal of daignostic criteria for calciphylaxis based on nationwide surveillance in Japan

Abstract

Calciphylaxis is a rare and often fatal complication of end-stage renal disease, and is characterized by painful skin ulceration. The main pathological findings are medial calcification and edematous intimal proliferation of small arteries. There had been little information regarding the prevalence and clinical features of calciphylaxis in Japan before our group conducted a nationwide survey in a Japanese dialysis population in 2009 as the study of “Research on Intractable Disease of Health and Labour Sciences Research Grants” from the Ministry of Health and Labour. The prevalence of calciphylaxis was speculated to be much lower than that in Western countries, and the lack of knowledge on the disease was suggested be related to this low prevalence. Therefore, to facilitate the diagnosis of calciphylaxis and spread knowledge of it, we proposed diagnostic criteria based on nationwide surveillance. These criteria must be reviewed by the accumulation of cases, while this attempt should promote caution regarding calciphylaxis.