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Pauci-імунний гломерулонефрит без ANCA

ORPHA:97564· ICD-10 N05.7· Pauci-immune glomerulonephritis without ANCA

Визначення(English summary)

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.

Поширеність
Unknown
Успадкування
Not applicable
Вік початку
Adolescent, Adult, Childhood