Abstract
IgG4-related kidney disease is a term that refers to any form of renal involvement by IgG4-related disease (IgG4-RD), a recently recognized systemic immune-mediated disease. The most common renal manifestation is IgG4-related tubulointerstitial nephritis (IgG4-TIN), which presents as acute or chronic renal insufficiency, renal mass lesions, or both. On biopsy, IgG4-TIN shows a plasma cell-rich interstitial inflammatory infiltrate with increased IgG4+ plasma cells, along with expansile interstitial fibrosis; tubular basement membrane immune complex deposits are common. IgG4-TIN usually shows a brisk response to immunosuppressive therapy. Glomeruli may be affected by IgG4-RD, usually in the form of membranous glomerulonephritis. Other patterns of glomerular disease include IgA nephropathy, membranoproliferative glomerulonephritis, and endocapillary or mesangioproliferative immune complex glomerulonephritis. IgG4-related plasma cell arteritis has also been observed in the kidney. This review describes the histopathologic and immunophenotypic patterns of renal involvement by IgG4-RD, with associated clinical, radiographic, and serologic features.
Original language | English (US) |
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Pages (from-to) | 245-250 |
Number of pages | 6 |
Journal | Seminars in Diagnostic Pathology |
Volume | 29 |
Issue number | 4 |
DOIs | |
State | Published - Nov 2012 |
Keywords
- Glomerulonephritis;
- IgG4-related disease;
- Immune complex;
- Interstitial nephritis;
- Membranous
- Nephropathy
ASJC Scopus subject areas
- Pathology and Forensic Medicine