Pauci-immune glomerulonephritis without ANCA

Orpha code: 97564OMIM code:

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
-

No additional description.

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