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Published on: November 10, 2021
IgG4-Related Tubulointerstitial Nephritis
Pingchuan Zhang1, Lynn D Cornell1
1Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.
Immunoglobulin G4 (IgG4)-related disease can affect any organ, with IgG4-related tubulointerstitial nephritis (IgG4-TIN) being the most common kidney manifestation. While often responsive to steroids, IgG4-TIN has a high relapse rate after stopping treatment.
Area of Science:
- Nephrology
- Immunology
- Rheumatology
Background:
- Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a recognized fibroinflammatory disorder affecting multiple organs.
- IgG4-related tubulointerstitial nephritis (IgG4-TIN) is the predominant renal manifestation of IgG4-RD.
- The kidney can be the initial or sole site of IgG4-RD involvement.
Purpose of the Study:
- To summarize the clinical presentation, diagnostic features, and treatment response of IgG4-related tubulointerstitial nephritis.
- To highlight the kidney as a potential primary site for IgG4-related disease.
- To underscore the characteristic therapeutic response and relapse patterns in IgG4-TIN.
Main Methods:
- Review of clinical data and biopsy findings in patients with IgG4-related tubulointerstitial nephritis.
- Analysis of presenting symptoms, including kidney insufficiency and mass lesions.
- Evaluation of histopathological hallmarks: lymphoplasmacytic infiltrate, IgG4+ plasma cells, storiform fibrosis, and immune deposits.
Main Results:
- Common clinical presentations include kidney insufficiency and/or renal mass lesions.
- Histopathology reveals dense lymphoplasmacytic infiltrates, increased IgG4+ plasma cells, storiform fibrosis, and immune deposits.
- Elevated serum IgG4 levels are supportive but not diagnostic.
- IgG4-TIN typically shows a prompt response to steroid therapy.
Conclusions:
- IgG4-related tubulointerstitial nephritis is a key renal manifestation of IgG4-RD.
- Early recognition and treatment are crucial, despite a high rate of relapse upon immunosuppression withdrawal.
- Further research into the pathogenesis of IgG4-RD is warranted.
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