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Abstract: SA-PO316

Calciphylaxis in a Patient with Polymyositis and Absence of Renal Disease

Session Information

Category: Bone and Mineral Metabolism

  • 502 Bone and Mineral Metabolism: Clinical

Authors

  • Morison, Doree, Tulane University School of Medicine, New Orleans, Louisiana, United States
  • Lewis, Sarah J., Tulane University School of Medicine, New Orleans, Louisiana, United States
  • Radosta, Stella, Tulane University School of Medicine, New Orleans, Louisiana, United States
  • Boh, Erin E., Tulane University School of Medicine, New Orleans, Louisiana, United States
  • Alvarado, Flor, Tulane University School of Medicine, New Orleans, Louisiana, United States
Introduction

Calciphylaxis is a rare and life-threatening disease in which calcium accumulates in small arteries and can cause tissue necrosis, thrombosis, and painful, non-healing skin ulcers. It is most often seen in patients with advanced choric kidney disease (CKD) but may occur when risk factors such as autoimmune or inflammatory conditions are present. We describe a case of calciphylaxis in a patient with polymyositis and acute kidney injury (AKI) without CKD.

Case Description

A 61-year-old man with polymyositis presented to the hospital with worsening dyspnea, and painful, lower leg wounds. He first noticed the leg wounds following a muscle biopsy for his polymyositis about 6 months prior to presentation; he reported the wounds increased in size since the biopsy.
Medical history was significant for polymyositis on prolonged prednisone course, bronchiolitis obliterans organizing pneumonia, atrial fibrillation, hypertension, diabetes, and systolic heart failure.
Physical exam revealed sinus tachycardia, coarse breath sounds with bilateral crackles. On his left leg, there were two tender-to-touch ulcers (~2x2 cm, clean-based, with dry yellow fibrinous exudate and surrounding hyperpigmentation).
Laboratory data revealed a white blood cell count of 19.6 (81% neutrophils), blood urea nitrogen 52 mg/dl, creatinine 1.8 mg/dl (baseline 1 mg/dl), calcium 10 mg/dl, phosphorus 3.6 mg/dl, and lactic acid 4.1 mmol/L.
He was admitted for further work-up of sepsis and AKI. Dermatology was consulted and took a biopsy of the wound which showed calcinosis with thrombotic vessels and fat necrosis consistent with calciphylaxis. Over the course of his hospitalization, the patient improved with antibiotics and fluids, and his AKI resolved. Blood cultures remained negative throughout his hospital stay, and antibiotics were adjusted accordingly by discharge. He was given clinic follow-ups with dermatology and rheumatology.

Discussion

To our knowledge, this is the first case of calciphylaxis reported in a patient with polymyositis and no underlying CKD. Clear treatment guidelines for calciphylaxis are lacking and many physicians are not familiar with nonuremic presentations of calciphylaxis. We present this case to further advance awareness and knowledge of calciphylaxis occurring in the absence of advanced CKD.