Anca-positive vasculitis with full-house nephropathy, an unusual association: a case report and review of literature

Abstract

Rapidly progressive glomerulonephritis is a medical emergency, with mortality around 20%. It is characterized by crescent glomerulonephritis and progressive loss of kidney function, hematuria, and proteinuria. Its classification is given by immunofluorescence detection of antibodies against glomerular basement membrane (Anti-MBG), immunocomplexes, or pauci-immune pattern. Its etiology should be based on clinical findings, immunological profile, age, sex, and histopathological characteristics. We present a case of a 27-year-old woman with symptoms consistent with rapidly progressive glomerulonephritis and biopsy findings of a full-house kidney nephropathy, with an early fatal outcome. An association of low incidence, as it is a case with a full-house pattern, and an autoimmune profile for negative systemic lupus erythematosus makes this a rare case. ANCA-associated vasculitis with full-house kidney disease was diagnosed, an unusual condition with up to 3% presentation and few reports in the literature, highlighting the importance of its reporting and contribution to the literature.