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Abstract: FR-PO949

Calcific Non-Uremic Arteriolopathy: An Uncommon Disease Presenting Uncommonly

Session Information

Category: CKD (Non-Dialysis)

  • 2203 CKD (Non-Dialysis): Mechanisms

Authors

  • Floyd, Stephanie M., Kent Hospital, Warwick, Rhode Island, United States
  • Tillquist, Kristen N., Kent Hospital, Warwick, Rhode Island, United States
  • Shah, Ankur, Rhode Island Hospital, Providence, Rhode Island, United States
Introduction

Calcific uremic arteriolopathy (CUA) is a rare and devastating disease presenting with painful necrotic ulcers and eschars on extremities and areas of high adiposity, typically in patients with ESKD. It is commonly thought to be a manifestation of dysregulation of parathyroid hormone, calcium and phosphorus metabolism commonly seen in dialysis patients. However, studies have shown a subset of patients without pre-existing kidney disease who develop ‘non-uremic CUA’. We present a rare case of CUA in a patient without CKD in the setting of decompensated alcohol cirrhosis and warfarin use.

Case Description

A 39-year-old woman with alcoholic cirrhosis decompensated by encephalopathy, portal vein thrombosis transiently on warfarin, hyponatremia, and paracentesis dependent ascites presented with 2 weeks of bilateral leg pain and rash. Pain predated the lesions which blistered and progressed to a dark purple scabbed area. She had no history of kidney disease or hyperparathyroidism.

Presenting exam was notable for exquisitely tender purpuric plaques on bilateral thighs with right thigh erythema, induration and ulceration. Lab work showed Na 123 mmol/L, sCr 0.99 mg/dL, Ca 9.4 mg/dL, Po4 4.3 mg/dL and iPTH 65 pg/mL. Punch biopsy of the lesions revealed deposition of calcifications around small vessels and adipocyte lobules with fibrin thrombi and ischemic necrosis of the epidermis consistent with calciphylaxis. Sodium thiosulfate (STS) was started, with dose adjustment due to acidemia. Lesions remained stable for much of the hospitalization. Hospital course was complicated by hepatorenal syndrome, hyponatremia, and gastrointestinal bleeding. She developed worsening hemodynamic compromise and expired after transition to hospice.

Discussion

This case of CUA in the absence of kidney disease highlights risk factors that should increase clinical suspicion and multidisciplinary approach required to manage this disease. Pain often precedes the development of visible lesions as it did for our patient. Given the severity of the disease there should be a high clinical suspicion prompting early investigation of suspicious lesions. Management includes STS with close monitoring for acidemia. Second line therapies include bisphosphonates and hyperbaric O2. This case highlights underappreciated risk factors, the need for multidisciplinary care, early diagnosis, and difficulty in management.