IgG4-related kidney disease--A review.
IgG4-related disease (IgG4-RD) is a recently recognized systemic autoimmune disorder characterized by high levels of serum IgG4 and dense infiltration of IgG4-positive plasma cells in multiple organs. The condition was first described as a disease of the pancreas, and has since been recognized in various organ systems including the kidneys. IgG4 related kidney disease (IgG4-RKD) signifies any form of renal involvement by IgG4-RD. The most common renal involvement by IgG4-RD is tubulointerstitial nephritis. Glomerular disease, in particular membranous glomerulonephritis, may also be seen. Other co-existent glomerular diseases such as IgA nephropathy, membranoproliferative glomerulonephritis, and mesangioproliferative immune complex glomerulonephritis may be identified. IgG4-related plasma cell arteritis has also been noted in the kidney. As with IgG4-RD in general, IgG4 related kidney disease (IgG4-RKD) usually occurs in middle-aged to elderly men. Common findings in IgG4-RKD are plasma cell-rich interstitial inflammatory infiltrate either in a focal or diffuse pattern with increased IgG4+ plasma cells, expansile swirling interstitial fibrosis, high levels of serum IgG and IgG4, hypocomplementemia, high serum IgE levels and/or peripheral blood eosinophilia. By immunofluorescence, most of the cases show IgG4 dominant tubular basement membrane immune complex deposits. Similar to IgG4-RD, IgG4-RKD often shows a rapid response to steroid therapy. In this review, we discuss the current knowledge on IgG4-RKD and its clinical relevance.
University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
Dinesh
D
Pradhan
Kalinga Hospital, Bhubaneswar, India.
Niharika
N
Pattnaik
University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
Russell
R
Silowash
CORE Diagnostics, Gurgaon, India. Electronic address: sambit04@gmail.com.
Sambit Kumar
SK
Mohanty
D000818:Animals; D001327:Autoimmune Diseases / Q000276:immunology*; D005455:Fluorescent Antibody Technique / Q000379:methods; D015433:Glomerulonephritis, Membranous; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood* / Q000276:immunology*; D007668:Kidney / Q000276:immunology*; D007674:Kidney Diseases / Q000276:immunology*
D007074:Immunoglobulin G
IgG4; Kidney disease