Abstract
BACKGROUND: Normal-renal calciphylaxis (NRC) is a devastating calcific vasculopathy characterised by refractory wounds. It is more likely to be misdiagnosed because of the absence of renal insufficiency. Failure to effectively control may lead to rapid deterioration and ultimately death. However, current knowledge of it is still inadequate. OBJECTIVES: To provide a relatively comprehensive review of NRC. METHODS: Nine electronic medical databases including PubMed, Web of Science, Embase, the Cochrane Library and so on were searched from inception to April 1, 2022. Articles in which calciphylaxis was diagnosed in patients with normal renal function were included. RESULTS: Totally 140 articles were retrieved with 187 patients (median age, 62 years [IQR, 49.63 ~ 75.70 years]; 83.42% female; 84.5% Caucasian). Vitamin K antagonism (43.32%) and diabetes (39.57%) accounted for most of the concomitant factors. Cutaneous presentations were the most common. Pathological confirmation was made in 97.86% of patients with an accuracy of 99.45%. Multidisciplinary therapeutic strategies were usually implemented while the final prognosis was not ideal, with a 6-month mortality rate of 21.10% and a 1-year mortality rate of 27.52%. CONCLUSION: NRC is an under-recognised disease caused by a variety of factors; meanwhile, multidisciplinary efforts are required to inform diagnostic and therapeutic decisions.