Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition 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. Disease data Classification Clinical subtype Synonyms Antineutrophil cytoplasmic antibody-negative pauci-immune glomerulonephritis Pauci-immune glomerulonephritis without antineutrophil cytoplasmic antibody Antineutrophil cytoplasmic antibody-negative pauci-immune glomerulonephritis Pauci-immune glomerulonephritis without antineutrophil cytoplasmic antibody ORPHA code 97564 OMIM code - ICD10 code N05.7 ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl