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IgG4-related kidney disease--an update
Mitsuhiro Kawano1, Takako Saeki
1aDivision of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan bDepartment of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Japan.
Current Opinion in Nephrology and Hypertension
|January 17, 2015
Summary
IgG4-related kidney disease (IgG4-RKD) often presents as tubulointerstitial nephritis (TIN) or membranous glomerulonephritis (MGN). While corticosteroid therapy can improve kidney function, severe cases may only achieve partial recovery.
Area of Science:
- Nephrology
- Immunology
- Rheumatology
Background:
- IgG4-related disease (IgG4-RD) is a systemic inflammatory condition affecting multiple organs.
- The kidneys are frequently involved, manifesting as tubulointerstitial nephritis (TIN), a key lesion of IgG4-RD.
Purpose of the Study:
- This review synthesizes current knowledge on IgG4-related kidney disease (IgG4-RKD).
- It focuses on the latest diagnostic and therapeutic insights into IgG4-RKD.
Main Methods:
- Review of recent literature on IgG4-related kidney disease.
- Analysis of clinical presentations, imaging findings, and treatment responses.
Main Results:
- IgG4-RKD encompasses TIN, membranous glomerulonephritis (MGN), and pyelitis.
- Characteristic imaging includes multiple low-density lesions or thickened renal pelvic walls.
- Rapid response to corticosteroids is typical for IgG4-TIN, but renal function recovery may be incomplete in advanced cases.
Conclusions:
- TIN and MGN are the primary renal manifestations of IgG4-RD.
- The role of IgG4 in IgG4-RD pathogenesis is questioned by the emergence of IgG4-negative cases.
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