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免疫触须样肾小球病病例,尽管具有严重的病理特征,但对类固醇治疗显示出高度反应性。

Case of immunotactoid glomerulopathy showing high responsiveness to steroids therapy despite severe pathological features.

作者信息

Ohashi Atsuki, Kumagai Jiro, Nagahama Kiyotaka, Fujisawa Hajime

机构信息

Nephrology, Yokohama City Minato Red Cross Hospital, Yokohama, Japan.

Pathology, Yokohama City Minato Red Cross Hospital, Yokohama, Japan.

出版信息

BMJ Case Rep. 2019 Jul 26;12(7):e229751. doi: 10.1136/bcr-2019-229751.

DOI:10.1136/bcr-2019-229751
PMID:31350228
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6663160/
Abstract

A 72-year-old woman presented with nephrotic proteinuria and moderate haematuria. Renal pathology was compatible with immunotactoid glomerulopathy (ITG), for which there is no consensus for appropriate therapy. We, therefore, postponed immunosuppressive therapy. After 4 years, the patient's renal function started to decline and renal pathology was re-evaluated, revealing a pathological change from mesangial proliferative glomerulonephritis to endocapillary proliferative glomerulonephritis. Treatment with oral prednisolone (30 mg/day) was initiated. Within 5 weeks, complete remission of proteinuria was obtained (proteinuria 6.02 g/gCr to 0.12 g/gCr), and the patient's renal function stabilised. Generally, responsiveness to immunosuppressive therapy is poor in patients with ITG, and the present case represented a very rare clinical course. Some previous cases have indicated susceptibility to the therapy, regardless of the severity of renal damage. As a possible distinct entity that determines susceptibility to immunosuppressive therapy, we suggest the presence of a latent lymphoproliferative disease with no significant haematological symptoms.

摘要

一名72岁女性出现肾病性蛋白尿和中度血尿。肾脏病理检查结果符合免疫触须样肾小球病(ITG),对于该病尚无合适治疗方案的共识。因此,我们推迟了免疫抑制治疗。4年后,患者肾功能开始下降,对肾脏病理进行重新评估,结果显示病理变化从系膜增生性肾小球肾炎转变为毛细血管内增生性肾小球肾炎。开始口服泼尼松龙治疗(30毫克/天)。在5周内,蛋白尿完全缓解(蛋白尿从6.02克/克肌酐降至0.12克/克肌酐),患者肾功能稳定。一般来说,ITG患者对免疫抑制治疗的反应较差,本病例呈现出非常罕见的临床病程。一些既往病例表明,无论肾损害的严重程度如何,患者对该治疗敏感。作为一种可能决定对免疫抑制治疗敏感性的独特实体,我们认为存在一种无明显血液学症状的潜在淋巴增殖性疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/91ec/6663160/fd9722ebee93/bcr-2019-229751f04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/91ec/6663160/a741fdebd256/bcr-2019-229751f01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/91ec/6663160/80aaaa234108/bcr-2019-229751f02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/91ec/6663160/0fd97b4c8a6c/bcr-2019-229751f03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/91ec/6663160/fd9722ebee93/bcr-2019-229751f04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/91ec/6663160/a741fdebd256/bcr-2019-229751f01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/91ec/6663160/80aaaa234108/bcr-2019-229751f02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/91ec/6663160/0fd97b4c8a6c/bcr-2019-229751f03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/91ec/6663160/fd9722ebee93/bcr-2019-229751f04.jpg

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Immunotactoid glomerulopathy: clinicopathologic and proteomic study.免疫触须样肾小球病:临床病理和蛋白质组学研究。
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A case of immunotactoid glomerulopathy exhibiting nephrotic syndrome successfully treated with corticosteroids and antihypertensive therapy.
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