Pauci-immune glomerulonephritis with ANCA

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Synonyms: Pauci-immune glomerulonephritis with antineutrophil cytoplasmic antibody

A form of pauci-immune glomerulonephritis characterized by a rapidly progressive glomerulonephritis in association with the presence of circulating antineutrophilic cytoplasmic antibodies (ANCA) mostly directed against proteinase-3 (PR3) and myeloperoxidase (MPO). Patients usually present with urinary abnormalities and rapidly declining renal function often leading to dialysis within weeks without treatment. Cutaneous pulmonary musculoskeletal and nervous involvement may be observed in case of systemic disease and the correlation between ANCA titer and disease activity has been demonstrated.

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 http://www.orphadata.org. Data version July 2024

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