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Updated: Jul 19, 2026
14:18
Dioscin Mediated IgA Nephropathy Alleviation by Inhibiting B Cell Activation In Vivo and Decreasing Galactose-Deficient IgA1 Production In Vitro
Published on: October 13, 2023
Nephropathy in IgG4-related systemic disease.
Simon J W Watson1, David A S Jenkins, Christopher O S Bellamy
1Nephrology, Renal Unit, Queen Margaret Hospital, Whitefield Road, Dunfermline KY12 0SU, UK. swatson@imap4all.com
The American Journal of Surgical Pathology
|October 26, 2006
Summary
This study details a case of autoimmune pancreatitis leading to kidney disease. Treatment with steroids improved kidney function and normalized immunoglobulin G4 (IgG4) levels, suggesting IgG4
Area of Science:
- Nephrology
- Immunology
- Gastroenterology
Background:
- Autoimmune pancreatitis (AIP) is a systemic fibrosclerotic disease characterized by elevated immunoglobulin G4 (IgG4) levels.
- While pancreatic and biliary involvement are common, extranodal manifestations of AIP are less frequently reported.
Observation:
- A patient with initially unrecognized AIP developed focal sclerosing lymphoplasmacytic tubulointerstitial nephritis and membranous nephropathy 5 years later.
- The patient presented with hypertension, elevated serum creatinine, proteinuria, eosinophilia, and high serum IgG4 levels.
Findings:
- Renal biopsy revealed numerous IgG4-positive plasma cells with IgG4 deposition in the glomeruli and peritubular areas.
- Steroid therapy led to normalization of serum IgG4, resolution of eosinophilia, and improvement in renal function and other symptoms.
Implications:
- This case highlights a distinct, treatable form of tubulointerstitial nephritis associated with systemic IgG4-related disease.
- The findings suggest that dysregulated IgG4 may contribute to the pathogenesis of membranous nephropathy.
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