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Abstract: FR-PO618

ANCA-Associated Vasculitis With Hypocomplementemia

Session Information

Category: Glomerular Diseases

  • 1302 Glomerular Diseases: Immunology and Inflammation

Authors

  • Diaz Garcia, Juan Daniel, Centro Medico Nacional 20 de Noviembre, Mexico City, Mexico City, Mexico
  • Alamilla Sanchez, Mario, Centro Medico Nacional 20 de Noviembre, Mexico City, Mexico City, Mexico
  • Yanez Salguero, Valeria, Centro Medico Nacional 20 de Noviembre, Mexico City, Mexico City, Mexico
  • Yama Estrella, Martin Benjamin, Centro Medico Nacional 20 de Noviembre, Mexico City, Mexico City, Mexico
  • Ortega, Jose Luis, Centro Medico Nacional 20 de Noviembre, Mexico City, Mexico City, Mexico
  • Morales Lopez, Enrique Fleuvier, Centro Medico Nacional 20 de Noviembre, Mexico City, Mexico City, Mexico
  • Nieto, Julio Cesar, Centro Medico Nacional 20 de Noviembre, Mexico City, Mexico City, Mexico
  • López, Claudia Bethzabé, Centro Medico Nacional 20 de Noviembre, Mexico City, Mexico City, Mexico
  • Ramirez, Irving Gaston, Centro Medico Nacional 20 de Noviembre, Mexico City, Mexico City, Mexico
  • Velasco Garcia Lascurain, Francisco, Centro Medico Nacional 20 de Noviembre, Mexico City, Mexico City, Mexico
Introduction

It has been suggested that the role of complement activation, mainly of the alternate pathway, in the pathogenesis of ANCA vasculitis is significant. Although ANCA-related necrotizing and crescentic glomerulonephritis is often referred to as a “pauciimmune” process, in certain cases there is deposition of immune complexes and complement in the glomeruli detected by immunofluorescence. Complement deposits in the kidney of patients with ANCA vasculitis correlate with greater kidney damage, more significant proteinuria, and general disease activity.

Case Description

3 patients are reported, 2 men and 1 woman, with histopathological diagnosis of pauciimmune glomerulonephritis and C3 deposits detected by immunofluorescence, positive ANCAS PR3, negative ANAS, profoundly decreased C3 with values less than 20 mg/dl in all 3 patients and C4 decreased by less than 10 mg/dl. Active urinary sediment with dysmorphic erythrocytes 70%, proteinuria in 24 hours between 2 g to 2.5 g, progressive deterioration of renal function, requiring 2 of them renal replacement therapy. Management was started with methylprednisolone 1000 mg every 24 hours for 3 days and Rituximab 375 mg/m2 every week for 4 weeks as induction therapy. 2 patients with a progressive decrease in serum creatinine reaching baseline renal function and one patient who required hemodialysis sessions without recovery of baseline renal function, being diagnosed with chronic kidney disease.

Discussion

According to reports, 25% of patients with ANCA vasculitis have low C3 levels at the time of diagnosis, which is associated with more severe kidney disease and worse renal outcomes, with complement deposits being reported in biopsies. in 25-54% of patients with ANCA vasculitis. C3 deposition was associated with proteinuria, higher creatinine, lower C3, and certain parameters of renal histopathology, such as a higher percentage of crescent formation. In our cases, the three patients presented decreased complement in both C3 and C4 with severe kidney disease; however, 2 patients with a favorable outcome thanks to timely management and another patient who ended up with chronic kidney disease on hemodialysis. This should be taken into account in therapeutic and follow-up strategies.