Pauci-immune glomerulonephritis without ANCA

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Synonyms: Antineutrophil cytoplasmic antibody-negative pauci-immune glomerulonephritis | Pauci-immune glomerulonephritis without antineutrophil cytoplasmic antibody

A form of pauci-immune glomerulonephritis characterized by rapidly progressive glomerulonephritis and the absence of antineutrophilic cytoplasmic antibodies (ANCA). In comparison with pauci-immune GN with ANCA patients lacking ANCA may be younger at onset of the disease have fewer extra renal manifestations (e.g. involvement of lung eye ear nose and throat) fewer constitutional symptoms (e.g. fever weight loss muscle pain and arthralgia) and a high prevalence of nephrotic syndrome and chronic renal lesions. The prognosis is generally poorer.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on Data version July 2024

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