Title |
Successful treatment of highly advanced immunoglobulin G4-related kidney disease presenting renal mass-like regions with end-stage kidney failure: a case study
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Published in |
BMC Nephrology, August 2017
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DOI | 10.1186/s12882-017-0676-5 |
Pubmed ID | |
Authors |
Hiroyuki Ono, Taichi Murakami, Akira Mima, Eriko Shibata, Masanori Tamaki, Sakiya Yoshimoto, Sayo Ueda, Fumi Kishi, Seiji Kishi, Takashi Kawanaka, Motokazu Matsuura, Kojiro Nagai, Hideharu Abe, Masashi Harada, Toshio Doi |
Abstract |
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. 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 cell-rich 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. 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. |
X Demographics
Geographical breakdown
Country | Count | As % |
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India | 1 | 33% |
Unknown | 2 | 67% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 2 | 67% |
Practitioners (doctors, other healthcare professionals) | 1 | 33% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
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Unknown | 9 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
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Other | 3 | 33% |
Student > Master | 2 | 22% |
Student > Bachelor | 2 | 22% |
Student > Ph. D. Student | 1 | 11% |
Unknown | 1 | 11% |
Readers by discipline | Count | As % |
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Biochemistry, Genetics and Molecular Biology | 2 | 22% |
Agricultural and Biological Sciences | 2 | 22% |
Medicine and Dentistry | 2 | 22% |
Psychology | 1 | 11% |
Nursing and Health Professions | 1 | 11% |
Other | 0 | 0% |
Unknown | 1 | 11% |