环孢素和口服皮质类固醇治疗IgA肾病伴膜增生性肾小球肾炎型病变所致肾病综合征的成功缓解。

Yuki Nakao, Yutaro Mori, Junko Kunieda, Towako Taguchi, Katsuo Mori, Ayumi Yamamura, Shotaro Naito, Fumiaki Ando, Shintaro Mandai, Soichiro Iimori, Koichiro Susa, Takayasu Mori, Kenichi Ohashi, Eisei Sohara, Shinichi Uchida
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摘要

免疫球蛋白A肾病(IgAN)是一种常见的肾小球肾病,表现为系膜细胞增生和系膜基质增生,通常用口服糖皮质激素治疗。然而,某些口服糖皮质激素抵抗性IgA肾病的临床表现和治疗策略尚未确定。我们在此报告一例复发性IgA肾病,表现为肾病综合征和膜增生性肾小球肾炎型病变。22年前,一名42岁女性在健康检查中发现尿隐血,经肾活检诊断为IgA肾病。虽然口服糖皮质激素治疗暂时缓解,但复发反复发生。八年前,她接受了第三次肾活检,结果显示II级(C)和牛津分级M1E0S1T0。扁桃体切除术和类固醇脉冲治疗后暂时缓解。随后,她出现肾病综合征,尿蛋白水平迅速升高。第四次肾活检显示膜增生性肾小球肾炎(MPGN)型病变,以前未观察到。在类固醇脉冲治疗后给予环孢素和皮质类固醇导致完全缓解。结论在复发性IgA肾病合并肾病综合征的病例中,mpgn型病变可能是这种情况的基础。在这些情况下,皮质类固醇和额外的环孢素都可能有效。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Successful Remission of Nephrotic Syndrome Caused by IgA Nephropathy with Membranoproliferative Glomerulonephritis-pattern Lesion Treated by Cyclosporine and Oral Corticosteroids.

Introduction Immunoglobulin A nephropathy (IgAN) is a common glomerular renal disease presenting with mesangial cell proliferation and mesangial matrix hyperplasia and is typically treated with oral glucocorticoids. However, the clinical presentations and treatment strategies for certain cases of oral glucocorticoid-resistant IgA nephropathy have not been established. Clinical Presentation We herein report a case of recurrent IgA nephropathy presenting with nephrotic syndrome and membranoproliferative glomerulonephritis-pattern lesions. A 42-year-old woman presented to our clinic 22 years ago after urinary occult blood was detected during a health examination, leading to a diagnosis of IgA nephropathy based on a renal biopsy. Although temporary remission was achieved with oral glucocorticoid therapy, the relapse occurred repeatedly. Eight years ago, she underwent a third renal biopsy, which revealed grade II (C) and Oxford classification M1E0S1T0 findings. Temporary remission was achieved following tonsillectomy and steroid pulse therapy. Subsequently, she presented with nephrotic syndrome and rapidly increasing urinary protein levels. A fourth renal biopsy revealed membranoproliferative glomerulonephritis (MPGN) pattern lesions that were not previously observed. The administration of cyclosporine and corticosteroids after steroid pulse therapy led to complete remission. Conclusion In cases of recurrent IgA nephropathy complicated by nephrotic syndrome, MPGN-pattern lesions may underlie this condition. In these cases, both corticosteroids and additional cyclosporine may be effective.

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