Unraveling the Enigmatic Mysteries of a Rare Renal Disorder ANCA - Negative Pauci-Immune Glomerulonephritis

Dr. Anbalagan Suyambulingam | Dr. Sankar Arumugam | Dr. R.Umashankar

Abstract: Background: ANCA-negative pauci-immune glomerulonephritis is a rare and intriguing form of glomerular inflammation that presents diagnostic and therapeutic challenges. This condition, characterized by rapidly progressive glomerular injury and crescent formation, occurs in approximately 10% of patients with systemic vasculitis. Despite the absence of detectable anti-neutrophil cytoplasmic antibodies (ANCA), patients exhibit histological features consistent with pauci-immune GN. The pathogenesis and optimal management of ANCA-negative pauci-immune glomerulonephritis remain largely unexplored. Methods: In this study, we present a comprehensive analysis of a case of ANCA-negative pauci-immune glomerulonephritis encountered in a 55-year-old normotensive, euglycemic woman. The patient's clinical presentation, laboratory findings, imaging results, treatment protocol, and response to therapy were thoroughly assessed. Additionally, we conducted a literature review to provide a broader perspective on the current state of knowledge regarding this rare condition. Results: The patient exhibited a myriad of symptoms, including fever, cough, weight loss, loss of appetite, bilateral pedal edema, decreased urine output, and breathlessness. Laboratory investigations revealed normocytic normochromic anemia, elevated creatinine and urea levels, hypokalemia, and an elevated total white blood cell count. Imaging studies demonstrated multiple lung cavities, and urine analysis showed numerous red blood cells and pus cells with klebsiella growth. Notably, all autoantibody tests, including C-ANCA, P-ANCA, ANA PROFILE, COMPLEMENTS C3, C4, and ANTI GBM ANTIBODY, were negative. The patient received an initial course of IV steroids, followed by oral prednisolone and cyclophosphamide therapy. Hemodialysis and PRBC transfusion were administered, and renal biopsy confirmed the presence of cellular crescents, consistent with pauci-immune glomerulonephritis. After six weeks of intensive treatment, the patient experienced complete renal recovery and a reduction in the size of lung cavities. Conclusion: ANCA-negative pauci-immune glomerulonephritis presents a unique and challenging clinical scenario due to its rarity and diagnostic complexities. Early recognition and initiation of immunosuppressive therapy, including steroids and cyclophosphamide, are critical in achieving favorable outcomes. This case report underscores the need for increased awareness and research to improve our understanding of the pathogenesis and management of ANCA-negative pauci-immune glomerulonephritis. Further investigations and case series are warranted to enhance the management of this distinct subset of glomerulonephritis and optimize patient outcomes.

Keywords: ANCA-negative, pauci-immune glomerulonephritis, glomerular inflammation, systemic vasculitis, crescent formation, immunosuppressive therapy, lung cavities

Edition: Volume 12 Issue 8, August 2023,

Pages: 2478 - 2481