Nakao Yuki, Mori Yutaro, Kunieda Junko, Taguchi Towako, Mori Katsuo, Yamamura Ayumi, Naito Shotaro, Ando Fumiaki, Mandai Shintaro, Iimori Soichiro, Susa Koichiro, Mori Takayasu, Ohashi Kenichi, Sohara Eisei, Uchida Shinichi
Department of Nephrology, Graduate School of Medical and Dental Sciences, Institute of Science Tokyo, Japan.
Department of Comprehensive Pathology, Graduate School of Medical and Dental Sciences, Institute of Science Tokyo, Japan.
Intern Med. 2025 Jul 10. doi: 10.2169/internalmedicine.5526-25.
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.
引言
免疫球蛋白A肾病(IgAN)是一种常见的肾小球肾脏疾病,表现为系膜细胞增殖和系膜基质增生,通常采用口服糖皮质激素治疗。然而,某些口服糖皮质激素抵抗型IgA肾病病例的临床表现和治疗策略尚未确立。
临床表现
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样病变。一名42岁女性22年前因健康检查时发现尿潜血就诊于我院,经肾活检诊断为IgA肾病。尽管口服糖皮质激素治疗取得了暂时缓解,但病情反复复发。八年前,她接受了第三次肾活检,结果显示为II级(C),牛津分类为M1E0S1T0。扁桃体切除和类固醇脉冲治疗后取得了暂时缓解。随后,她出现肾病综合征,尿蛋白水平迅速升高。第四次肾活检显示出以前未观察到的膜增生性肾小球肾炎(MPGN)样病变。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样病变可能是其病因。在这些病例中,皮质类固醇和额外的环孢素可能都有效。