A Rare Case of Calciphylaxis in an Orthotopic Liver Transplant Recipient with Acute Kidney Injury

Abstract

Calciphylaxis is a rare disease characterized by calcification of small- to medium-sized blood vessels in the dermis and subcutaneous fat, resulting in cutaneous necrosis. Although most commonly shown in patients with end-stage kidney disease, it has also been reported in patients with other diseases, including alcoholic cirrhosis and malignancies. Here, we report an unusual case of calciphylaxis in an orthotopic liver transplant recipient with acute kidney injury. The patient, a 43-year-old white female with a history of type 2 diabetes mellitus, alcoholic cirrhosis, and normal kidney function, presented with decompensated liver disease and hepatorenal syndrome; she no longer responded to medical treatment and required treatment with dialysis. Ten days after admission, she underwent liver transplant, resulting in improved liver function tests. She had acute tubular necrosis (creatinine peak: 325 μmol/L) from sustained hypotension during and after surgery, which required 4 sessions of dialysis over 2weeks. Six weeks after her transplant, she developed painful, nonulcerating, erythematous plaques over her shins and thighs. Skin biopsy of the lesions showed calciphylaxis, calcium deposits, and thrombotic vasculopathy. She also developed severe hypercalcemia (calcium level of 2.75 mmol/L) from immobility, which required treatment with a bisphosphonate and hemodialysis. The lesions improved 6 weeks later, and her renal function returned to normal. Calciphylaxis diagnosed in an orthotopic liver transplant recipient with acute kidney injury has not been previously reported. We hypothesize that her chronic inflammatory state caused down-regulation and low levels of fetuin A and protein C. She also had other risk factors, including hypoalbuminemia, obesity, systemic glucocorticoids, and alcoholic liver disease. Calciphylaxis can occur in patients with alcoholic cirrhosis and acute renal failure even after liver transplant. Further studies into the pathogenesis of this disease may help us understand why it develops in these patients and not others with the same risk factors.

Calciphylaxis and Kidney Disease: A Review

“…1 While uremic calciphylaxis has predominantly been linked to ESKD, more case reports suggest that this rare depositional vasculopathy may appear in the context of early kidney injury including acute kidney injury (AKI). [11][12][13][14][15][16] A case report described the appearance of calciphylaxis in the bilateral thighs of a woman three months after developing painless hematuria and nephrotic-range proteinuria in the setting of anti-GBM disease. 17 Furthermore, a population of non-uremic (nontraditional) patients may develop calciphylaxis despite normal kidney and parathyroid function.…”

Untitled

“…Calciphylaxis, also referred to as calcific uremic arteriolopathy, is a rare life-threatening vasculopathy, characterized by deposition of calcium in the arteriolar microvasculature of the deep dermis and subcutaneous adipose tissue, resulting in intensely painful, ischemic skin lesions [1]. Although uremic calciphylaxis typically affects patients with end-stage chronic kidney disease, it can also occur in patients with earlier stages of chronic kidney disease (CKD) [2], acute kidney injury [3][4][5], prior receipt of a kidney transplant [6] and even with normal kidney function. The largest nationwide study to date estimates an incidence rate of 3.49 per 1,000 patient-years among patients receiving maintenance hemodialysis [7].…”

"Exploring Novel Therapeutic Strategies for Calciphylaxis: A Focus on Vitamin K2 Supplementation"

“…Calciphylaxis, also referred to as calcific uremic arteriolopathy, is a rare life-threatening vasculopathy, characterized by deposition of calcium in the arteriolar microvasculature of the deep dermis and subcutaneous adipose tissue, resulting in intensely painful, ischemic skin lesions [1]. Although uremic calciphylaxis typically affects patients with end-stage chronic kidney disease, it can also occur in patients with earlier stages of chronic kidney disease (CKD) [2], acute kidney injury [3][4][5], prior receipt of a kidney transplant [6] and even with normal kidney function. The largest nationwide study to date estimates an incidence rate of 3.49 per 1,000 patient-years among patients receiving maintenance hemodialysis [7].…”