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Start free with EleplanIgG4-related kidney disease
ORPHA:449395Clinical subtype
What it is
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.
Key facts
- Age of onset
- Adult, Elderly
- Inheritance
- Not applicable
- Classified as
- Clinical subtype
Signs and symptoms
Very common80–99%
3Common30–79%
13- Acute kidney injury
- Antinuclear antibody positivity
- Chronic kidney disease
- Complement deficiency
- Decreased circulating complement C3 concentration
- Decreased circulating complement C4 concentration
- Elevated circulating creatinine concentration
- Hematuria
- Increased circulating IgG1 level
- Proteinuria
- Renal insufficiency
- Renal interstitial immunoglobulin deposits
- Rheumatoid factor positive
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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