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Abstract: PO1489

Crescentic Pauci-Immune Glomerulonephritis in a Patient with Sickle Cell Anemia and Cocaine Abuse

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation

Author

  • Contreras Nieves, Marimar, Stanford Medicine, Stanford, California, United States
Introduction

Levamisole is an anthelminthic agent and a common contaminant found in cocaine. It has been linked to ANCA-associated vasculitis with cutaneous, and more rarely, renal and pulmonary manifestations. This is the case of a patient with sickle cell anemia and cocaine abuse presenting with acute kidney injury (AKI) and nephrotic-range proteinuria, initially attributed to sickle cell nephropathy, but with kidney biopsy revealing pauci-immune glomerulonephritis, demonstrating the importance of having a high level of suspicion in patients with known cocaine use.

Case Description

A 58-year-old male with history of sickle cell anemia, CKD Stage 3, hypertension, and cocaine abuse, was admitted for epididymitis and found to have AKI. He presented with testicular pain, as well as right knee and back pain. Initial work up revealed a creatinine of 2.45 mg/dL, from baseline of 1.8 mg/dL. Urine toxicology was positive for cocaine. His urine studies were consistent with intrinsic renal disease. He received red blood cell exchange transfusion, but he continued with worsening renal function following the procedure. His 24-hr urine collection revealed 6.2 g proteinuria. Extensive workup included normal C3 and C4, positive rheumatoid factor, negative UPEP, free kappa/lambda light chain ratio 1.8, negative HIV and anti-HCV, hepatitis B immunity, negative c-ANCA, but positive p-ANCA and anti-MPO. Patient chronically smoked cocaine, sometimes cut with levamisole, which is associated with vasculitis. A kidney biopsy was performed, with pathology showing a pauci-immune necrotizing and crescentic glomerulonephritis, transmural arteritis, and sickle cell nephropathy. He was treated with pulse dose steroids and rituximab, followed by maintenance prednisone and additional doses of rituximab after discharge. His renal function improved, but did not return to baseline, which could have been due to his degree of kidney injury and ongoing cocaine use after discharge.

Discussion

This case demonstrates the importance of keeping a broad differential diagnosis in the evaluation of AKI and nephrotic-range proteinuria in sickle cell anemia, particularly in the setting of cocaine use and its known association with levamisole. Although sickle cell nephropathy could have explained this patient’s presentation, a broader workup was key to arrive to the correct diagnosis, and therefore prompt treatment.