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Kidney Week

Abstract: TH-PO0635

Sarcoidosis as a Driver of Nonuremic Calciphylaxis

Session Information

Category: CKD (Non-Dialysis)

  • 2202 CKD (Non-Dialysis): Clinical, Outcomes, and Trials

Authors

  • Wortman, Kevin O'Neal, Tulane University School of Medicine, Department of Nephrology and Hypertension, New Orleans, Louisiana, United States
  • Pittappilly, Matthew, Tulane University School of Medicine, Department of Nephrology and Hypertension, New Orleans, Louisiana, United States
Introduction

Although classically associated with end-stage renal disease, non-uremic calciphylaxis has rarely been reported in patients with CKD. We report a case of suspected non-uremic calciphylaxis associated with sarcoidosis-related hypercalcemia.

Case Description

A 56-year-old woman with stage 4 CKD, diabetes mellitus, hypertension, and MGUS presented with two weeks of painful rash on her thighs. Hydrochlorothiazide was increased one month prior. Exam showed hyperpigmented nodules with focal ulceration. Labs showed calcium 12.4 mg/dL, phosphorus 3.4 mg/dL, creatinine 2.41 mg/dL, PTH 8 pg/mL, 1,25-dihydroxy vitamin D 111 pg/mL, ACE 94 U/L, and elevated lysozyme. CXR showed a right perihilar opacity. Autoimmune, infectious, and malignancy evaluation was negative. Skin biopsy showed thrombosis without vascular calcification on Von Kossa stain. Hydrochlorothiazide and vitamin D supplementation were discontinued. Fluids, furosemide, sodium thiosulfate, and pamidronate was started.
She later developed a cough. Bronchoscopy with biopsy showed granulomatous inflammation. Corticosteroids improved hypercalcemia, pain, renal function, and lesions.

Discussion

Non-uremic calciphylaxis is an uncommon but severe disorder that may occur in patients with or without end-stage renal disease. Risk factors in this patient included CKD, diabetes, obesity, female sex, vitamin D supplementation, and recent thiazide escalation. Although calciphylaxis is frequently associated with ESRD, CKD and hypercalcemia may also contribute to vascular calcification and thrombosis.
Sarcoidosis-associated calciphylaxis is rarely reported. Diagnosing calciphylaxis can be difficult becuase biopsy findings can be nonspecific in early disease. This case highlights the importance of maintaining suspicion for calciphylaxis in patients with characteristic lesions and risk factors, even without ESRD or confirmatory biopsy findings.

Lesion.