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Kidney Week

Abstract: PO1905

Systemic Amyloidosis Presenting as Progressive Dysphagia, Hypercalcemia, and Proteinuria

Session Information

Category: Onco-Nephrology

  • 1500 Onco-Nephrology

Authors

  • Patrick, Kennerly Clinton, Virginia Commonwealth University Health System, Richmond, Virginia, United States
  • Tripathi, Shreyank D., Virginia Commonwealth University Health System, Richmond, Virginia, United States
  • Patel, Nilang G., Virginia Commonwealth University Health System, Richmond, Virginia, United States
Introduction

Systemic amyloidosis consists of several disorders whereby amyloid fibrils deposit in the extracellular tissue of multiple organs and as such, is associated with a wide spectrum of disease leading to significant morbidity and mortality. The severity and clinical manifestations of systemic amyloidosis is highly dependent on the site of amyloid fibril deposition.

Case Description

We present a 75-year-old male with no medical history who presented with dysphagia and epigastric abdominal pain. Lab work revealed moderate hypercalcemia and acute kidney injury (AKI) with urinalysis significant for >500mg/dL of protein. He received intravenous fluid resuscitation with improvement in renal function and temporary resolution of hypercalcemia. His AKI and hypercalcemia were attributed to volume depletion and possible milk-alkali syndrome due to consumption of calcium carbonate. After discharge however, he continued to have persistent sub-nephrotic range proteinuria, mild hypercalcemia and progressive renal insufficiency. UPEP and serum free light chain analysis revealed elevated kappa light chains. A kidney biopsy showed glomeruli with mesangial expansion as well as Congo red positive staining of glomeruli, interstitium, and vessels. Electron microscopy showed mesangial deposition of fibrillary material consistent with AL kappa light chain renal amyloidosis. Prior to follow up with Hematology, he was re-hospitalized for AKI, acute liver injury concerning for hepatic amyloidosis and progressive dysphagia likely due to gastrointestinal involvement. Therapy was initiated with bortezomib and dexamethasone; however, no significant kidney recovery was observed, and he remained dependent on dialysis. Due to rapid clinical decline, additional chemotherapy was not offered, and he was transitioned to comfort care.

Discussion

This patient presented with dysphagia, persistent hypercalcemia, renal insufficiency and proteinuria highlighting the clinical variability of systemic amyloidosis. As such, systemic amyloidosis, a rare infiltrative disorder, requires a high level of clinical suspicion in order to reach an early diagnosis and prevent long-term complications and mortality associated with advanced, multi-organ involvement. In addition, it is crucial to exclude coexisting multiple myeloma in patients presenting with hypercalcemia, renal insufficiency and AL amyloidosis.