Abstract
IgG4-related disease is a recently established systemic condition. Tubulointerstitial nephritis is the most common renal manifestation. Glomerular lesions, particularly membranous glomerulonephritis, can develop simultaneously. Some patients present with serological renal dysfunction associated with elevated IgG or IgE levels and hypocomplementemia, while others are incidentally found to have abnormalities in kidneys on imaging. A majority of patients with IgG4-related kidney disease have similar lesions at other anatomical sites, which help us to suspect this condition. Serum IgG4 elevation (>135 mg/dL) is the most, although not entirely, specific marker for the diagnosis. Imaging findings varies from small nodules to bilateral diffuse abnormalities. In addition to the renal parenchyma, the renal pelvis and perirenal adipose tissue can be affected. Histological features include dense lymphoplasmacytic infiltration, storiform or "bird's eye" fibrosis (highlighted by PAM stain), and IgG4-positive plasma cell infiltration (>10 cells/high-power field and IgG4/IgG-positive cell ratio >40%). Immune complex deposition is detectable in the tubular basement membrane by immunofluorescence and/or electron microscopy. Patients usually respond well to corticosteroids, but highly active diseases may require other immunosuppressive therapies. Further investigations will be required to fully understand pathophysiology underlying this emerging condition.
| Original language | English |
|---|---|
| Pages (from-to) | 6379-6385 |
| Number of pages | 7 |
| Journal | International Journal of Clinical and Experimental Pathology |
| Volume | 7 |
| Issue number | 9 |
| Publication status | Published - 2014 |
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Keywords
- IgG4-related disease
- Kidney
- Review
ASJC Scopus subject areas
- Pathology and Forensic Medicine
- Histology
Cite this
IgG4-related renal disease : Clinical and pathological characteristics. / Kuroda, Naoto; Nao, Tomoya; Fukuhara, Hideo; Karashima, Takashi; Inoue, Keiji; Taniguchi, Yoshinori; Takeuchi, Mai; Zen, Yoh; Sato, Yasuharu; Notohara, Kenji; Yoshino, Tadashi.
In: International Journal of Clinical and Experimental Pathology, Vol. 7, No. 9, 2014, p. 6379-6385.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - IgG4-related renal disease
T2 - Clinical and pathological characteristics
AU - Kuroda, Naoto
AU - Nao, Tomoya
AU - Fukuhara, Hideo
AU - Karashima, Takashi
AU - Inoue, Keiji
AU - Taniguchi, Yoshinori
AU - Takeuchi, Mai
AU - Zen, Yoh
AU - Sato, Yasuharu
AU - Notohara, Kenji
AU - Yoshino, Tadashi
PY - 2014
Y1 - 2014
N2 - IgG4-related disease is a recently established systemic condition. Tubulointerstitial nephritis is the most common renal manifestation. Glomerular lesions, particularly membranous glomerulonephritis, can develop simultaneously. Some patients present with serological renal dysfunction associated with elevated IgG or IgE levels and hypocomplementemia, while others are incidentally found to have abnormalities in kidneys on imaging. A majority of patients with IgG4-related kidney disease have similar lesions at other anatomical sites, which help us to suspect this condition. Serum IgG4 elevation (>135 mg/dL) is the most, although not entirely, specific marker for the diagnosis. Imaging findings varies from small nodules to bilateral diffuse abnormalities. In addition to the renal parenchyma, the renal pelvis and perirenal adipose tissue can be affected. Histological features include dense lymphoplasmacytic infiltration, storiform or "bird's eye" fibrosis (highlighted by PAM stain), and IgG4-positive plasma cell infiltration (>10 cells/high-power field and IgG4/IgG-positive cell ratio >40%). Immune complex deposition is detectable in the tubular basement membrane by immunofluorescence and/or electron microscopy. Patients usually respond well to corticosteroids, but highly active diseases may require other immunosuppressive therapies. Further investigations will be required to fully understand pathophysiology underlying this emerging condition.
AB - IgG4-related disease is a recently established systemic condition. Tubulointerstitial nephritis is the most common renal manifestation. Glomerular lesions, particularly membranous glomerulonephritis, can develop simultaneously. Some patients present with serological renal dysfunction associated with elevated IgG or IgE levels and hypocomplementemia, while others are incidentally found to have abnormalities in kidneys on imaging. A majority of patients with IgG4-related kidney disease have similar lesions at other anatomical sites, which help us to suspect this condition. Serum IgG4 elevation (>135 mg/dL) is the most, although not entirely, specific marker for the diagnosis. Imaging findings varies from small nodules to bilateral diffuse abnormalities. In addition to the renal parenchyma, the renal pelvis and perirenal adipose tissue can be affected. Histological features include dense lymphoplasmacytic infiltration, storiform or "bird's eye" fibrosis (highlighted by PAM stain), and IgG4-positive plasma cell infiltration (>10 cells/high-power field and IgG4/IgG-positive cell ratio >40%). Immune complex deposition is detectable in the tubular basement membrane by immunofluorescence and/or electron microscopy. Patients usually respond well to corticosteroids, but highly active diseases may require other immunosuppressive therapies. Further investigations will be required to fully understand pathophysiology underlying this emerging condition.
KW - IgG4-related disease
KW - Kidney
KW - Review
UR - http://www.scopus.com/inward/record.url?scp=84911928166&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84911928166&partnerID=8YFLogxK
M3 - Article
C2 - 25337295
AN - SCOPUS:84911928166
VL - 7
SP - 6379
EP - 6385
JO - International Journal of Clinical and Experimental Pathology
JF - International Journal of Clinical and Experimental Pathology
SN - 1936-2625
IS - 9
ER -