Nonuremic Calciphylaxis Secondary to Decompensated Alcoholic Liver Disease with Subsequent Renal Failure

Abstract

Calciphylaxis is rare condition associated with chronic end-stage renal disease (ESRD). However, it has known non-uremic etiologies including hyperparathyroidism, malignancy, and alcoholic liver disease, though these are only documented in less than 15 cases. A 39-year-old male with alcoholic liver cirrhosis was transferred to our institution with bilateral groin and thigh skin ulcerations that persisted despite antibiotics for cellulitis. The concern was for a rapidly progressive, toxic dermatologic syndrome requiring tertiary level care. Upon arrival he was disoriented, tachypneic, and unable to compensate for worsening metabolic acidosis. He was emergently intubated and further tests revealed a creatinine of 5.5mg/dL (from 0.6mg/dL 2 months prior), an elevated bilirubin to 3.9mg/dL, AST/ALT of 50/19 IU/L, and INR of 2.29. CT head was unremarkable. Lactulose was started for hepatic encephalopathy. Dermatology was consulted for a skin biopsy and indicated a high suspicion for calciphylaxis. Empirically a chelating agent, sodium thiosulfate, was started. Midodrine, octreotide and albumin were also began for possible hepatorenal syndrome (HRS). He subsequently became anuric and received continuous renal replacement therapy. His MELD score was 34-40. The hospital course also featured disseminated intravascular coagulation and an acute portal vein thrombosis. He was eventually stabilized and transferred to a liver transplant center.Figure 1Calciphylaxis is typically associated with ESRD. Early diagnosis is paramount in this usually fatal disease. Only 20% of patients who suffer from non-uremic calciphylaxis are believed to have alcoholic liver disease as a cause. Proposed risk factors are decreased protein C and S levels and repeated albumin infusions. Interestingly, our patient developed the further complication of kidney injury from some combination of the calciphylaxis and HRS. While originally believed that calciphylaxis triggered vessel calcifications created endothelial dysfunction related pathology, hypercoagulability is becoming more widely recognized as problematic in non-uremic calciphylaxis. Our patient also developed disseminated intravascular coagulation and portal vein thrombosis, neither of which has been documented in the literature in the setting of calciphylaxis. As our patient demonstrates, in patients with underlying liver disease, coupled with the calcification depositions inherent in calciphylaxis, thrombotic complications must be anticipated.