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Immunoglobulin G4-Related Kidney Disease: A Comprehensive Pictorial Review of the Imaging Spectrum, Mimickers, and Clinicopathological Characteristics.

Seo N, Kim JH, Byun JH, Lee SS, Kim HJ, Lee MG - Korean J Radiol (2015)

Bottom Line: Immunoglobulin G4 (IgG4)-related kidney disease (IgG4-KD) has recently been demonstrated to be an important part of IgG4-related sclerosing disease (IgG4-SD).However, since IgG4-KD is still relatively unfamiliar to radiologists and physicians as compared to IgG4-SD involving other organs, it could, therefore, be easily missed.In this article, we present a comprehensive pictorial review of IgG4-KD with regards to the imaging spectrum, mimickers, and clinicopathologic characteristics, based on our clinical experience with 48 patients during the past 13 years, as well as a literature review.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul 05505, Korea.

ABSTRACT
Immunoglobulin G4 (IgG4)-related kidney disease (IgG4-KD) has recently been demonstrated to be an important part of IgG4-related sclerosing disease (IgG4-SD). However, since IgG4-KD is still relatively unfamiliar to radiologists and physicians as compared to IgG4-SD involving other organs, it could, therefore, be easily missed. In this article, we present a comprehensive pictorial review of IgG4-KD with regards to the imaging spectrum, mimickers, and clinicopathologic characteristics, based on our clinical experience with 48 patients during the past 13 years, as well as a literature review. Awareness of the broad imaging spectrum of IgG4-KD and differential diagnosis from its mimickers will thus facilitate its early diagnosis and treatment.

No MeSH data available.


Related in: MedlinePlus

72-year-old man who underwent left nephrectomy due to IgG4-KD mimicking urothelial carcinoma.A. Contrast-enhanced CT image shows ill-defined, homogeneous, soft-tissue mass (arrow) which encases left renal pelvis causing hydronephrosis. B. He underwent left nephrectomy with diagnosis of having urothelial carcinoma. Photograph of surgical specimen demonstrates lobulated, yellowish, firm mass (arrows) around renal pelvis. C, D. Lower-power (× 100) (C) and higher-power (× 400) (D) photomicrographs of histological specimen (hematoxylin and eosin staining) show dense plasma cell infiltration (arrows) with fibrous background (Fi) and obliterative phlebitis (arrowheads). E. IgG4 immunostaining (× 100) of histologic specimen shows numerous, brown, IgG4-positive plasma cells. IgG4-KD = immunoglobulin G4-related kidney disease
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Figure 1: 72-year-old man who underwent left nephrectomy due to IgG4-KD mimicking urothelial carcinoma.A. Contrast-enhanced CT image shows ill-defined, homogeneous, soft-tissue mass (arrow) which encases left renal pelvis causing hydronephrosis. B. He underwent left nephrectomy with diagnosis of having urothelial carcinoma. Photograph of surgical specimen demonstrates lobulated, yellowish, firm mass (arrows) around renal pelvis. C, D. Lower-power (× 100) (C) and higher-power (× 400) (D) photomicrographs of histological specimen (hematoxylin and eosin staining) show dense plasma cell infiltration (arrows) with fibrous background (Fi) and obliterative phlebitis (arrowheads). E. IgG4 immunostaining (× 100) of histologic specimen shows numerous, brown, IgG4-positive plasma cells. IgG4-KD = immunoglobulin G4-related kidney disease

Mentions: Immunoglobulin G4-related kidney disease manifests most commonly as tubulointerstitial nephritis, followed by glomerular disease such as membranous glomerulonephritis (1). Other less frequent conditions, including IgG4-related chronic sclerosing pyelitis, IgG4-related plasma-cell arteritis, and IgG4-related inflammatory pseudotumors of the ureter, have also been reported (1141516). The key histologic findings of IgG4-KD are dense lymphoplasmacytic infiltration with increased IgG4-positive plasma cells, and storiform fibrosis, both of which are identical to the findings of IgG4-SD involving other organs (Fig. 1) (310). In addition, the characteristic microscopic features of IgG4-related tubulointerstitial nephritis are nests of inflammatory cells with irregular fibers surrounding them, and immune complex deposition in the tubular basement membrane (51718).


Immunoglobulin G4-Related Kidney Disease: A Comprehensive Pictorial Review of the Imaging Spectrum, Mimickers, and Clinicopathological Characteristics.

Seo N, Kim JH, Byun JH, Lee SS, Kim HJ, Lee MG - Korean J Radiol (2015)

72-year-old man who underwent left nephrectomy due to IgG4-KD mimicking urothelial carcinoma.A. Contrast-enhanced CT image shows ill-defined, homogeneous, soft-tissue mass (arrow) which encases left renal pelvis causing hydronephrosis. B. He underwent left nephrectomy with diagnosis of having urothelial carcinoma. Photograph of surgical specimen demonstrates lobulated, yellowish, firm mass (arrows) around renal pelvis. C, D. Lower-power (× 100) (C) and higher-power (× 400) (D) photomicrographs of histological specimen (hematoxylin and eosin staining) show dense plasma cell infiltration (arrows) with fibrous background (Fi) and obliterative phlebitis (arrowheads). E. IgG4 immunostaining (× 100) of histologic specimen shows numerous, brown, IgG4-positive plasma cells. IgG4-KD = immunoglobulin G4-related kidney disease
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4559777&req=5

Figure 1: 72-year-old man who underwent left nephrectomy due to IgG4-KD mimicking urothelial carcinoma.A. Contrast-enhanced CT image shows ill-defined, homogeneous, soft-tissue mass (arrow) which encases left renal pelvis causing hydronephrosis. B. He underwent left nephrectomy with diagnosis of having urothelial carcinoma. Photograph of surgical specimen demonstrates lobulated, yellowish, firm mass (arrows) around renal pelvis. C, D. Lower-power (× 100) (C) and higher-power (× 400) (D) photomicrographs of histological specimen (hematoxylin and eosin staining) show dense plasma cell infiltration (arrows) with fibrous background (Fi) and obliterative phlebitis (arrowheads). E. IgG4 immunostaining (× 100) of histologic specimen shows numerous, brown, IgG4-positive plasma cells. IgG4-KD = immunoglobulin G4-related kidney disease
Mentions: Immunoglobulin G4-related kidney disease manifests most commonly as tubulointerstitial nephritis, followed by glomerular disease such as membranous glomerulonephritis (1). Other less frequent conditions, including IgG4-related chronic sclerosing pyelitis, IgG4-related plasma-cell arteritis, and IgG4-related inflammatory pseudotumors of the ureter, have also been reported (1141516). The key histologic findings of IgG4-KD are dense lymphoplasmacytic infiltration with increased IgG4-positive plasma cells, and storiform fibrosis, both of which are identical to the findings of IgG4-SD involving other organs (Fig. 1) (310). In addition, the characteristic microscopic features of IgG4-related tubulointerstitial nephritis are nests of inflammatory cells with irregular fibers surrounding them, and immune complex deposition in the tubular basement membrane (51718).

Bottom Line: Immunoglobulin G4 (IgG4)-related kidney disease (IgG4-KD) has recently been demonstrated to be an important part of IgG4-related sclerosing disease (IgG4-SD).However, since IgG4-KD is still relatively unfamiliar to radiologists and physicians as compared to IgG4-SD involving other organs, it could, therefore, be easily missed.In this article, we present a comprehensive pictorial review of IgG4-KD with regards to the imaging spectrum, mimickers, and clinicopathologic characteristics, based on our clinical experience with 48 patients during the past 13 years, as well as a literature review.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul 05505, Korea.

ABSTRACT
Immunoglobulin G4 (IgG4)-related kidney disease (IgG4-KD) has recently been demonstrated to be an important part of IgG4-related sclerosing disease (IgG4-SD). However, since IgG4-KD is still relatively unfamiliar to radiologists and physicians as compared to IgG4-SD involving other organs, it could, therefore, be easily missed. In this article, we present a comprehensive pictorial review of IgG4-KD with regards to the imaging spectrum, mimickers, and clinicopathologic characteristics, based on our clinical experience with 48 patients during the past 13 years, as well as a literature review. Awareness of the broad imaging spectrum of IgG4-KD and differential diagnosis from its mimickers will thus facilitate its early diagnosis and treatment.

No MeSH data available.


Related in: MedlinePlus