Rare Case of Renal Limited Dual Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: Double Trouble!

  • Elenjickal Elias John Department of Nephrology, Christian Medical College, Vellore, Tamil Nadu, India
  • Sanjeet Roy Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India
  • Selvin Sundar Raj Mani Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India
  • Jeethu Joseph Eapen Department of Nephrology, Christian Medical College, Vellore, Tamil Nadu, India
  • Utkarash Mishra Department of Nephrology, Christian Medical College, Vellore, Tamil Nadu, India
  • Santosh Varughese Department of Nephrology, Christian Medical College, Vellore, Tamil Nadu, India

Keywords

ANCA-associated vasculitis, anti-proteinase-3, anti-myeloperoxidase, hypocomplementemia, crescents

Abstract

Dual anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) characterized by the presence of both anti-proteinase-3 (PR3-ANCA) and anti-myeloperoxidase (MPO-ANCA) antibodies is a rare clinical entity. Only few cases have been reported previously, most of which were associated with infections, drugs, autoimmune diseases and malignancies. Herein, we describe a young woman who presented with rapidly progressive glomerulonephritis with hypocomplementemia and markedly elevated anti-PR3 and anti-MPO titres. Meticulous work-up ruled out all possible secondary causes. Renal biopsy showed the presence of focal fibrocellular crescents with focal mesangial hypercellularity. Immunofluorescence and electron microscopy showed pauci-immune deposits. The patient was treated with an induction regimen comprising oral prednisolone and cyclophosphamide. She attained both clinical and serological remission at 3 months and is currently on an azathioprine-based maintenance regimen. We have extensively reviewed all previous cases of dual AAV and have formulated an approach to diagnose and treat this rare entity.

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Published: 2022-05-10

Issue: 2022: Vol 9 No 5 (view)

Section: Articles

How to cite:
1.
Elias John E, Roy S, Sundar Raj Mani S, Joseph Eapen J, Mishra U, Varughese S. Rare Case of Renal Limited Dual Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: Double Trouble!. EJCRIM 2022;9 doi:10.12890/2022_003365.