Pauci-immune necrotizing glomerulonephritis in a 24-year-old female with negative ANCA antibodies: A Rare Case Report. Key Clinical Message:ANCA-associated vasculitis (AAV) disorders include eosinophilic granulomatous polyangiitis (EGPA, formerly known as "Churg-Strauss syndrome"), granulomatous polyangiitis (GPA, formerly known as "Wegener's granulomatosis"), and microscopic polyangiitis.However, a clinically negative ANCA test can occur in as many as 10% of individuals with small vascular vasculitis.On the other hand, false positive findings can happen in the general population as well as in the context of autoimmune renal and gastrointestinal disorders, infections, and cancers.A 24-year-old female presented to the emergency department with polyarthralgia, a fever measuring 38 degrees , dysfagya, and dyspnea.The clinical examination revealed pallor, pharyngitis, Synovial thickening ,Crackles.Urine analysis revealed 70 white blood cells, 800 red blood cell counts, and protein.Coronal computer tomography (CT) images show multifocal patchy centrilobular consolidative opacities in the bilateral middle lobes and lower lobes, consistent with acute hemorrhage or infection.Pulmonary nephrotic syndrome was suspected and P ANCA C ANCA was negative.The kidney biopsy was consistent with Pauci-immune crescentic glomerulonephritis type that is usually found with ANCAassociated vasculitis.Pauci-immune necrotizing glomerulonephritis (PING) is a small vessel renal vasculitis usually associated with the presence of antineutrophil cytoplasmic antibodies (ANCA) against myeloperoxidase or proteinase.A small proportion of PING patients do not have ANCA antibodies.
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