IgG4-related kidney disease
ORPHA:449395· ICD-10 N11.8
Definition
A rare renal disease occurring in the setting of a systemic IgG4 related disease (IgG4-RD). The disorder is characterized by a fibrosing tubulointerstitial nephritis consisting of predominantly IgG4+ plasma cells with/without glomerulonephritis, retroperitoneal fibrosis and hydronephrosis.
- Prevalence
- Unknown
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly