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Abstract: SA-PO0886

ANCA-Negative, Pauci-Immune Crescentic Glomerulonephritis Associated with Hepatitis C: A Diagnostic and Therapeutic Challenge

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Paswan, Rishi, ECU Nephrology & Hypertension, Greenville, North Carolina, United States
  • Qadir, Neela, ECU Nephrology & Hypertension, Greenville, North Carolina, United States
  • Rodriguez Lopez, Armando Francisco, ECU Nephrology & Hypertension, Greenville, North Carolina, United States
  • Myers, Iskra, ECU Nephrology & Hypertension, Greenville, North Carolina, United States
  • Obi, Reginald Ifeanyi, ECU Nephrology & Hypertension, Greenville, North Carolina, United States
Introduction

Pauci-immune glomerulonephritis, most often associated with anti-neutrophil cytoplasmic antibodies (ANCA), represents a significant proportion of diagnosed rapidly progressive glomerulonephritidies (RPGNs). However, ANCA-negative pauci-immune RPGN is a rare and diagnostically challenging entity, often requiring a high index of suspicion and histopathologic confirmation to properly direct management of these patients.

Case Description

We describe the case of a 63-year-old with a past medical history that includes papillary renal cell carcinoma status post left partial nephrectomy in 2016, atrial fibrillation on rivaroxaban, COPD, hypertension, and previous history of polysubstance abuse who presented due to worsening generalized abdominal pain and diffuse body rash. Initial laboratory workup revealed acute kidney injury with a creatinine of 3.71 mg/dl from a baseline of 0.7-1.0 mg/dl, hyperkalemia, and urinalysis notable for numerous red blood cells, 3+ hemoglobin, and 2+ protein. Urine protein creatinine ratio was elevated at 2.9 mg/dl, concerning for a possible rapidly progressive glomerulonephritis (RPGN). Preliminary serologic testing, including ANA, ANCA, myeloperoxidase antibody, proteinase-3 antibody, cryoglobulin levels, and hepatitis panel were collected. Hepatitis C antibody serology returned positive, with the remainder of serology without significant positive findings.
An initial renal biopsy was completed that was nondiagnostic, with the patient subsequently suffering a left middle cerebral artery stroke, necessitating ICU admission. A repeat biopsy later confirmed the diagnosis of focal, ANCA negative pauci-immune, necrotizing crescentic glomerulonephritis. He was initiated on immunosuppressive therapy, including high-dose corticosteroids and rituximab, with subsequent clinical improvement and eventual discharge.

Discussion

This case demonstrated the diagnostic and therapeutic challenges of RPGN due to ANCA negative pauci-immune glomerulonephritis. The association with hepatitis C virus, although normally more associated with cryoglobulinemia, would suggests an atypical inflammatory response and would warrant further study. The cerebrovascular event highlights the systemic nature and severity of this rare subtype of RPGN that is often associated with significant morbidity.

Digital Object Identifier (DOI)