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Kidney Week

Abstract: FR-PO638

Calciphylaxis Patient Characteristics and Outcomes: Case Series of 7 Patients

Session Information

  • Trainee Case Reports - IV
    October 26, 2018 | Location: Exhibit Hall, San Diego Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Reports

  • 701 Dialysis: Hemodialysis and Frequent Dialysis

Authors

  • Zakher, Mariam, Albany Medical College, Albany, New York, United States
  • Salman, Loay H., Albany Medical College, Albany, New York, United States
  • Mehta, Swati, Albany Medical College, Albany, New York, United States
  • Daoui, Sabrina, 363, Saratoga Springs, New York, United States
  • Daoui, Rachid, Saratoga Hospital , Saratoga, New York, United States
  • Chaudhry, Rafia I., Albany Medical College, Albany, New York, United States
Introduction

Calcific uremic arteriolopathy (CUA) also known as calciphylaxis is a rare, life-threatening ischemic small-vessel vasculopathy, primarily a complication of advanced CKD or ESRD. The pathogenesis involves intimal hypertrophy and medial calcification, resulting in endovascular fibrosis and thrombosis of small arteries and arterioles. We reviewed historical cases to assess patient characteristics, treatment modalities employed and clinical outcomes.

Case Description

We present 7 cases of calciphylaxis diagnosed between 2013 and 2017. Median age at time of diagnosis was 57.7 (42-76), 57% were male, 57% were Caucasian, 71% smokers, and 100% obese. Patients had been on dialysis for an average of 5.2 years prior to diagnosis. Only 1 patient was on peritoneal dialysis; the rest were on in-center hemodialysis (3 days/week). Comorbid conditions included DM (4/7), HTN (4/7), concomitant anticoagulation with warfarin (2/7), vitamin D analogue (4/7), oral calcium supplements (2/7), and calcitriol (2/7). Calciphylaxis was confirmed by skin biopsy in 7 cases. Mean calcium, phosphorus and PTH levels were 9.15, 5.6, and 322.9, respectively.
All patients received sodium thiosulfate 25 mg 3 days/ week, along with increased daily dialysis (average of 5.2 treatments/ week for 4.3 hours over 7.2 months). 5/7 patients were reported to have complete resolution after an average of 7.64 months. One patient continues to receive treatment. Two patients died, including one patient who chose to discontinue dialysis 3.8 years after diagnosis, and the second patient did not tolerate sodium thiosulfate (metabolic acidosis and hypotension) and died from sepsis 8 days after diagnosis. Complications of sodium thiosulfate included metabolic acidosis (6/7), hypotension (4/7), and hypocalcemia (2/7).

Discussion

Previously reported risk factors include high BMI, female sex, DM, PD, higher serum calcium, phosphorus, and parathyroid hormone, and combined therapy with calcium salts and vitamin D, cinacalcet, warfarin and calcitriol.
Mortality rate is reported at 27% at 6 months after CUA diagnosis, and 45% at 12 months. Data on the dose and duration of increased intensity dialysis as well as treatment are lacking, and while CUA is associated with a high rates of mortality, we have a 71% survival rate with emphasis on maximizing dialysis (daily).