First author, year Study description Author recommendations/conclusions Level of evidence
Kramann, 2013[@137838] Primary study of skin specimens from 7 patients with calciphylaxis that focused on pathogenesis and histology Calciphylaxis pathogenesis involves upregulation of BMP-2 signaling, hydroxyapatite deposition, and matrix remodeling in the subcutaneous tissue. VI
Lal, 2009[@137803] Retrospective analysis of cases of 26 patients with biopsy-proven calciphylaxis, evaluating risk factors, parathyroidectomy, and surgical debridement Surgical debridement showed statistically significant improvement in survival; parathyroidectomy did not. The authors suggested that a multidisciplinary approach to treatment with early diagnosis, aggressive medical management, surgical debridement, and parathyroidectomy is warranted for these patients. V
McCarthy, 2016[@137797] Retrospective analysis of 101 patients with calciphylaxis seen at Mayo Clinic in Rochester, Minnesota, between January 1, 1999, and September 30, 2014, that evaluated risk factors, comorbid conditions, and treatment response Results showed that surgical wound debridement and parathyroidectomy in patients with hyperparathyroidism improved survival. Tissue plasminogen activator, sodium thiosulfate, and hyperbaric oxygen therapy may also be considered for wound healing and seem to be well tolerated with no adverse effects. V
Robert, 2020[@137839] Retrospective analysis of 8 patients with calciphylaxis treated by Rheopheresis after sodium thiosulfate and discontinuation of vitamin K antagonists failed to resolve symptoms Of the 8 patients, 5 had complete remission of disease after Rheopheresis. The authors recommended a prospective study with a larger sample to confirm benefits of the Rheopheresis approach. VI
Torregrosa, 2015[@137808] Combined retrospective analysis of cases of 12 patients and a prospective analysis of cases of 11 patients, all of whom received bisphosphonate therapy for calciphylaxis Intravenous bisphosphonate therapy may be useful for treatment of calciphylaxis wounds despite potential for exacerbation of bone disease in renal failure patients. V
Weenig, 2007[@137798] Retrospective analysis including 64 patients with calciphylaxis that evaluated risk factors and prognosis Calciphylaxis is a multifactorial disease with high mortality that is associated with obesity, systemic corticosteroid use, liver disease, increased serum aluminum, and increased erythrocyte sedimentation rate. Of note, warfarin use and increased calcium-phosphate product were not reliably associated with calciphylaxis development. Surgical debridement was shown to improve survival but not parathyroidectomy. The authors recommended modification or avoidance of identified risk factors as a means for primary or secondary prevention of the disease. V
Zakher, 2021[@137804] Retrospective analysis of 10 patients with calciphylaxis that evaluated treatment outcomes involving sodium thiosulfate and intensified hemodialysis therapy Mortality rates improved and wounds resolved after multimodal treatment that included sodium thiosulfate and intensified hemodialysis (>20 h/wk). VI