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CASE REPORT
Year : 2021  |  Volume : 10  |  Issue : 2  |  Page : 83-86

“Triple-positive” renal limited vasculitis presenting with rapidly progressive glomerulonephritis: A case report


1 Department of Medicine, Jawaharlal Nehru Medical College, DMIMS (Deemed to be University), India
2 Department of Pathology, Jawaharlal Nehru Medical College, DMIMS (Deemed to be University), India

Correspondence Address:
Amol Andhale
Department of Medicine, Jawaharlal Nehru Medical College, DMIMS (Deemed to be University)
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2221-6189.312215

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Rationale: Coexistence of anti-glomerular basement membrane (anti-GBM) disease with anti-neutrophil cytoplasmic antibody (ANCA) in a case of glomerulonephritis is often identified as a “double-positive” disease. Interestingly, the majority of “double positive” ANCA is myeloperoxidase (MPO)-ANCA and some of the MPO-ANCA positive cases showed intrarenal arteritis, suggesting an ANCA-associated kidney lesion. Proteinase 3-ANCA positive diseases are also rarely reported. Patients positive for all three antibodies, i.e., triple-positive patients, are extremely rare. Patient’s Concern: A 53 year-old female presented with anasarca and oliguria of 2 months’ duration. Diagnosis: Pauci-immune type renal limited crescentic glomerulonephritis positive for MPO-ANCA, proteinase 3-ANCA, and anti-GBM antibody (triple-positive). Interventions: Intravenous high dose cyclophosphamide, oral azathioprine, intravenous methylprednisolone, and plasma exchange as per British Health Professionals in Rheumatology Guidelines. Outcomes: After one-month follow-up, anasarca and proteinuria were lessened, serum creatinine was normalized, titers of MPO-ANCA levels were decreased, and anti-GBM antibody levels were normalized. Lessons: Triple-positive renal limited vasculitis is rare and response to combined immunosuppressive therapy and plasma exchange can contribute to successful remission.


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