Title:



Declaration Page

The results presented in this paper have not been published previously in whole or part except in abstract form.

The authors have no conflicts of interest to disclose.

Title:

Calciphylaxis in a Patient with Acute Kidney Injury and Alcoholic Cirrhosis

Authors:

Paula Wichienkuer, MD,

Resident of Internal Medicine

Department of Medicine, Oregon Health and Science University

Mail Code: OP-30

3181 SW Sam Jackson Park Road

Portland, OR 97239

Wilscott Naugler, MD

Assistant Professor of Medicine

Division of Gastroenterology

Department of Medicine, Oregon Health and Science University

Mail Code: L462

3181 SW Sam Jackson Park Road

Portland, OR 97239

Raghav Wusirika, MD

Assistant Professor of Medicine

Division of Nephrology and Hypertension

Department of Medicine, Oregon Health and Science University, Portland, OR

Mail Code: PP262

3314 S.W. U.S. Veterans Hospital Rd.

Portland, Oregon 97239-2940

Address Correspondence to :

Raghav Wusirika, MD

Division of Nephrology and Hypertension

Department of Medicine, Oregon Health and Science University, Portland, OR

Mail Code: PP262

3314 S.W. U.S. Veterans Hospital Rd.

Portland, Oregon 97239-2940

Email wusirika@ohsu.edu

Phone 502-494-8284

Facsimile 503-494-5330

Abstract

Calciphylaxis is a rare disorder characterized by painful skin necrosis and calcification of small vessels which is seen mainly in the dialysis population. We describe a case of a patient with alcoholic cirrhosis developing calciphylaxis during an episode of acute kidney injury. We also review the literature in prior cases and postulate that unrecognized renal dysfunction in cirrhotic patients may have played a role in the development of this uncommon disorder. Our patient had complete recovery of the lesions with conservative treatment which coincided with recovery of her renal function. This case serves to highlight how small changes in creatinine levels in patients with cirrhosis may represent larger and clinically relevant changes in actual renal function that predispose these patients to calciphylaxis.

Key Words

Calciphylaxis – Cirrhosis – Acute Kidney Injury

Introduction

Calciphylaxis is a rare disorder characterized by painful necrotic non-healing ulcers. It is most frequently seen in patients with end stage renal disease in which the prevalence has been reported to be as high as 4% [1]. In patients with renal disease it is believed to be related to abnormal mineral metabolism including elevation of phosphorus, secondary hyperparathyroidism and vitamin D therapy although the exact etiology causing some patients to contract the disorder while other patients have similar laboratory findings but no lesions remains largely unknown.

Calciphylaxis has also been very rarely described in patients with normal renal function. The other disorders associated with calciphylaxis include primary hyperparathyroidism, malignancy, alcoholic liver disease, connective tissue disease, diabetes, protein C and S deficiency, Crohn’s disease, vitamin D deficiency and weight loss [2-8]. We report a case of a patient with calciphylaxis and alcoholic cirrhosis in the setting of acute kidney injury which resolved after the renal function normalized. Although the absolute change in serum creatinine was only 0.4 mg/dL, we postulate that the level of clinically important renal dysfunction was underestimated by the creatinine changes.

Case Report:

A 41-year-old woman with a history of newly diagnosed alcoholic cirrhosis presented with two months of violaceous purpuric painful plaques on both thighs. Two months prior to the development of these lesions, she had been hospitalized for abdominal pain and distension and was found to have new onset ascites and acute alcoholic hepatitis. She was treated with a course of pentoxifylline 400 mg twice daily and prednisone 40 mg daily at the time for her alcoholic hepatitis. She had no other medical or surgical history.

Examination of the rash demonstrated bilateral indurated plaques on the proximal thighs with central purpuric necrotic changes [Figure 1]. The plaques ranged from 5 to 15 cm in diameter. Laboratory tests revealed: white blood cell count 10.4x103 /mcL, hemoglobin 9.8 g/dL, blood urea nitrogen 18 mg/dL, creatinine 1.21 mg/dL [baseline 0.8 mg/dL], serum calcium 10.4 mg/dL, ionized calcium of 11.1 mg/dL, serum phosphorus 5.8 mg/dL, albumin 3.3 g/dL, intact PTH 10 mg/dl. Of particular note the serum creatinine was greater than 1.0 mg/dl in the five cases in which it was reported.

Serum creatinine is known to be a poor indicator of renal function in the cirrhotic population. Although serum creatinine is easily measured, the discrepancy between actual renal function and the creatinine is accentuated in liver failure patients due to malnutrition, decreased muscle mass and increased tubular secretion of creatinine. Thus most creatinine based equations for estimation of renal function in this population would tend to overestimate the actual glomerular filtration rate [16]. In a study of liver transplant candidates whose average serum creatinine was 1.0 mg/dL, their estimated glomerular filtration rate by the Modification of Diet in Renal Disease [MDRD] equation was 76.9 ml/min while direct measurement by iothalamate clearance was 58.2 ml/min representing a 32% difference in the estimated versus measured values [17]. In addition it is well known that creatinine is a poor indicator of renal function during acute kidney injury in all populations as all estimations are based on the creatinine being in a steady state and it takes time for the creatinine to accumulate in cases of acute kidney injury. This may make serum creatinine an even worse marker for actual renal function in acute kidney injury patients with cirrhosis for many of the same reasons mentioned above and it is possible that creatinine values greater than 1.0 mg/dL in the cirrhotic patients with reports of calciphylaxis may represent significant reduction in renal function for these patients [18].

Calciphylaxis has been reported in non-dialysis patients with chronic kidney disease in the past [19]. In a review of 64 cases of calciphylaxis at the Mayo clinic, 15 patients were non-dialysis patients. However, only one of these patients had an estimated GFR above 60 ml/min based on Cockcroft-Gault. Eleven of the 15 had eGFR ................
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