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Kidney Week

Abstract: PO0558

Calciphylaxis: Clinical Features, Therapeutic Options, and Outcomes

Session Information

Category: CKD (Non-Dialysis)

  • 2102 CKD (Non-Dialysis): Clinical, Outcomes, and Trials

Authors

  • Agapito Fonseca, José, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisboa, Lisboa, Portugal
  • Sanches, Maria, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisboa, Lisboa, Portugal
  • Duarte, Inês Clemente, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisboa, Lisboa, Portugal
  • Gameiro, Joana, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisboa, Lisboa, Portugal
  • Outerelo, Cristina Beato Henriques, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisboa, Lisboa, Portugal
  • Silva, Hugo, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisboa, Lisboa, Portugal
  • Jorge, Sofia C.a., Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisboa, Lisboa, Portugal
  • Lopes, Jose António, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisboa, Lisboa, Portugal
  • Nogueira, Estela, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisboa, Lisboa, Portugal
Background

Calciphylaxis is characterized by microvascular disease with calcification of the middle layer of the arterioles, intimal hyperplasia and thrombotic occlusion, conditioning areas of ischemia and skin necrosis. Given the rarity of this pathology, there is a lack in literature regarding clinical presentation, diagnostic approach and therapeutic management. We performed a descriptive analysis of clinical, epidemiologic, laboratory characteristics, treatment options and outcomes in a population of patients with calciphylaxis.

Methods

Retrospective analysis of all calciphylaxis diagnosed in a single-center between January 2003 and December 2019.

Results

The diagnosis of calciphylaxis was made in 9 patients, 7 of whom were female, with a mean age of 63.4±10.9 years. Eight patients were on renal replacement therapy (all hemodialysis) at the time of diagnosis, with a dialysis vintage of 66.4±82.4 months and one patient had no chronic kidney disease. Six patients were taking warfarin, with an average of 46 months on anticoagulation. The mean pre-diagnostic serum calcium value was 9.4 mg/dL, with an average phosphorus level of 4.5 mg/dL, phosphocalcic product was 42 mg2/dL2 and the average PTH was 1078 pg/mL. The onset or increase in calcium-containing phosphorus binders was recorded in 6 patients, with cinacalcet being used in only 4 patients. Five patients underwent bisphosphonate therapy, 1 undewent sodium thiosulfate, 3 were submitted to hyperbaric chamber sessions, 2 underwent dialysis intensification and 3 patients were submitted parathyroidectomy. Three patients died within 12 months of diagnosis. Mean follow-up time was 28.8±24.5 months.

Conclusion

More than 60% of patients were under warfarin, reinforcing the role of vitamin K antagonists in the pathogenesis. Mean time on dialysis was highly variable, from the 1st to the 216th month since the beginning of the technique. The standard of treatment varied according to the drugs available and the clinical evidence that supported its use at the time of diagnosis. The registered deaths corresponded to patients diagnosed later in the course of the disease, reinforcing the importance of a high clinical suspicion regarding the appearance of trophic skin lesions in this population as a form of early diagnosis to prevent mortality.