TY - JOUR
T1 - IgG4-Related Tubulointerstitial Nephritis
AU - Zhang, Pingchuan
AU - Cornell, Lynn D.
N1 - Publisher Copyright:
© 2016 National Kidney Foundation, Inc.
PY - 2017/3/1
Y1 - 2017/3/1
N2 - Immunoglobulin G4 (IgG4)–related disease (IgG4-RD) is a fibroinflammatory disorder that can involve nearly any organ. The disorder has increasingly become known as a distinct clinical entity during the last decade. IgG4-related tubulointerstitial nephritis (IgG4-TIN) is the most common manifestation of IgG4-RD in the kidney. Many patients with IgG4-TIN are diagnosed after IgG4-RD has been recognized in other organ systems, but the kidney may also be the first or only site involved. The presenting clinical features of IgG4-TIN are most commonly kidney insufficiency, kidney mass lesion(s), or both. On biopsy, IgG4-TIN shows a dense lymphoplasmacytic infiltrate, increased IgG4+ plasma cells, storiform fibrosis, and often tubular basement membrane immune complex deposits. Elevation of serum IgG4 often accompanies IgG4-RD; however, it is not specific in reaching the diagnosis. Like IgG4-RD in other organs, IgG4-TIN characteristically responds promptly to steroids, although there is a high relapse rate on discontinuation of immunosuppression. The pathogenesis of IgG4-RD is not understood.
AB - Immunoglobulin G4 (IgG4)–related disease (IgG4-RD) is a fibroinflammatory disorder that can involve nearly any organ. The disorder has increasingly become known as a distinct clinical entity during the last decade. IgG4-related tubulointerstitial nephritis (IgG4-TIN) is the most common manifestation of IgG4-RD in the kidney. Many patients with IgG4-TIN are diagnosed after IgG4-RD has been recognized in other organ systems, but the kidney may also be the first or only site involved. The presenting clinical features of IgG4-TIN are most commonly kidney insufficiency, kidney mass lesion(s), or both. On biopsy, IgG4-TIN shows a dense lymphoplasmacytic infiltrate, increased IgG4+ plasma cells, storiform fibrosis, and often tubular basement membrane immune complex deposits. Elevation of serum IgG4 often accompanies IgG4-RD; however, it is not specific in reaching the diagnosis. Like IgG4-RD in other organs, IgG4-TIN characteristically responds promptly to steroids, although there is a high relapse rate on discontinuation of immunosuppression. The pathogenesis of IgG4-RD is not understood.
KW - IgG4-related disease
KW - Immune complex
KW - Interstitial nephritis
KW - Plasma cell
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U2 - 10.1053/j.ackd.2016.12.001
DO - 10.1053/j.ackd.2016.12.001
M3 - Review article
C2 - 28284385
AN - SCOPUS:85014818750
SN - 1548-5595
VL - 24
SP - 94
EP - 100
JO - Advances in Chronic Kidney Disease
JF - Advances in Chronic Kidney Disease
IS - 2
ER -