| Item type |
文献 / Documents(1) |
| 公開日 |
2020-03-26 |
| アクセス権 |
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アクセス権 |
open access |
| 資源タイプ |
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資源タイプ識別子 |
http://purl.org/coar/resource_type/c_6501 |
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資源タイプ |
journal article |
| 出版社版DOI |
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|
関連識別子 |
https://doi.org/10.1186/s12882-017-0676-5 |
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関連名称 |
10.1186/s12882-017-0676-5 |
| 出版タイプ |
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出版タイプ |
VoR |
|
出版タイプResource |
http://purl.org/coar/version/c_970fb48d4fbd8a85 |
| タイトル |
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|
タイトル |
Successful treatment of highly advanced immunoglobulin G4-related kidney disease presenting renal mass-like regions with end-stage kidney failure : a case study |
| 著者 |
小野, 広幸
村上, 太一
美馬, 晶
柴田, 恵理子
田蒔, 昌憲
吉本, 咲耶
上田, 紗代
岸, 史
岸, 誠司
川中, 崇
松浦, 元一
長井, 幸二郎
安部, 秀斉
ハラダ, マサシ
土井, 俊夫
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| 抄録 |
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内容記述 |
Background: Immunoglobulin G4-related kidney disease characterized by immunoglobulin G4-positive plasma cell-rich tubulointerstitial nephritis has distinctive serological and radiological findings. Renal prognosis is good because of a good response to glucocorticoids. Here we report a case of successful treatment of highly advanced immunoglobulin G4-related kidney disease presenting renal mass-like regions with end-stage kidney failure.
Case Presentation: A 59-year-old Japanese man was referred to our hospital because of uremia with a creatinine level of 12.36 mg/dL. Urinalysis revealed mild proteinuria and hyperβ2microglobulinuria, and blood tests showed hyperglobulinemia with an IgG level of 3243 mg/dL and an IgG4 level of 621 mg/dL. Non-contrast computed tomography revealed renal mass-like regions. Based on the findings, immunoglobulin G4-related kidney disease was suspected, however, further radiological examination showed unexpected results. Ga-67 scintigraphy showed no kidney uptake. T2-weighted magnetic resonance imaging revealed high-intensity signals which corresponded to mass-like regions and multiple patchy low-intensity signals in kidney cortex. Finally, the patient was diagnosed with immunoglobulin G4-related kidney disease by renal pathology of severe immunoglobulin G4-positive plasma cellrich tubulointerstitial nephritis and characteristic fibrosis. He received 50 mg oral prednisolone, which was tapered with a subsequent decrease of serum creatinine and IgG4 levels. One year after initiation of treatment, he achieved normalization of serum IgG4 level and proteinuria, and remained off dialysis with a creatinine level of 3.50 mg/dL. After treatment with steroids, repeat imaging suggested bilateral severe focal atrophy. However, mass-like regions did not show atrophic change although renal atrophy was evident in patchy low-intensity lesions on T2-weighted magnetic resonance imaging. These findings suggest that multiple patchy low-intensity signals and high-intensity mass-like regions were mildly atrophic lesions of immunoglobulin G4-related kidney disease due to severe fibrosis and normal parts of kidney, respectively.
Conclusions: In immunoglobulin G4-related kidney disease with severe kidney failure, radiological findings should be carefully examined. In addition, renal prognosis may be good despite highly advanced tubulointerstitial nephritis and fibrosis. |
| キーワード |
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|
主題 |
Immunoglobulin G4 |
| キーワード |
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|
主題 |
End-stage kidney disease |
| キーワード |
|
|
主題 |
Tubulointerstitial nephritis |
| キーワード |
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|
主題 |
Fibrosis |
| 書誌情報 |
en : BMC Nephrology
巻 18,
p. 261,
発行日 2017-08-03
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| 収録物ID |
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収録物識別子タイプ |
ISSN |
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収録物識別子 |
14712369 |
| 収録物ID |
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収録物識別子タイプ |
NCID |
|
収録物識別子 |
AA12035314 |
| 出版者 |
|
|
出版者 |
Springer Nature |
| 出版者 |
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|
出版者 |
BioMed Central |
| 権利情報 |
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|
権利情報 |
© The Author(s). 2017 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
| EID |
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|
識別子 |
350340 |
| 言語 |
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|
言語 |
eng |
BMCNephr_18_261.pdf (1.1 MB)
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