Sarcoidosis-Associated Hypercalcemia and Calciphylaxis in a Patient on Dialysis with Left Ventricular Assist Device - A Tale of Caution

Introduction Sarcoidosis is a systemic disease that is difficult to diagnose in the absence of biopsy-proven non-caseating granulomas. We report a rare case of sarcoidosis-associated hypercalcemia and calciphylaxis in a patient on hemodialysis (HD) with left ventricular assist device (LVAD). Case A 53-year-old man with non-ischemic cardiomyopathy, poorly controlled diabetes, and chronic kidney disease (CKD) presented with decompensated heart failure and ventricular tachycardia. Positron emission tomography-computed tomography (PET-CT) of the heart suggested myocardial inflammation and sarcoidosis. He developed cardiogenic shock despite steroids and received an LVAD. Apical core biopsy showed normal myocardium. His CKD progressed to end-stage renal disease (ESRD) requiring HD six months later. He developed bilateral lower extremity pain thought to be diabetic neuropathy. He had hypercalcemia with normal parathyroid and thyroid hormones, but high angiotensin-converting enzyme level. Whole body PET-CT showed increased fluorodeoxyglucose (FDG) uptake in the spleen, liver, and small lung nodules. Biopsy of supraclavicular node with increased FDG uptake was negative for granulomas or malignancy. Serum protein electrophoresis did not yield monoclonal protein, and bone marrow biopsy showed no lymphoma. Bilateral calves and glans penis developed painful plaques with sharp borders and thick eschar (Figure A). Punch biopsy showed vascular calcifications in subcutaneous tissue, consistent with calciphylaxis (Figure B-D). Sodium thiosulfate was started with resultant shrinkage of lesions. Hypercalcemia resolved with prednisone and hydroxychloroquine. Unfortunately, the patient developed sepsis from the skin lesions and suffered a hemorrhagic stroke. His family decided to withdraw care. Discussion In this case, the diagnosis of sarcoidosis was made based on indirect evidence, and supported by resolution of hypercalcemia with prednisone. Hypercalcemia in sarcoidosis stems from calcitriol overproduction by monocytes, leading to increased intestinal calcium absorption. High calcium and phosphate levels, often found in patients with ESRD, have been implicated in the pathogenesis of calciphylaxis. For his LVAD, the patient was on warfarin, a known risk factor for calciphylaxis. Previous studies reported