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伴有新月体的膜性肾病的临床病理及长期预后特征:一项日本单中心经验

Clinicopathological and long-term prognostic features of membranous nephropathy with crescents: a Japanese single-center experience.

作者信息

Saito Masaya, Komatsuda Atsushi, Sato Ryuta, Saito Ayano, Kaga Hajime, Abe Fumito, Sawamura Masato, Nara Mizuho, Togashi Masaru, Okuyama Shin, Wakui Hideki, Takahashi Naoto

机构信息

Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita, Akita, 010-8543, Japan.

Department of Life Science, Akita University Graduate School of Engineering Science, Akita, Japan.

出版信息

Clin Exp Nephrol. 2018 Apr;22(2):365-376. doi: 10.1007/s10157-017-1465-y. Epub 2017 Aug 29.

Abstract

BACKGROUND

Three recent studies from the United States and China reported the clinicopathological features and short-term prognosis in patients with membranous nephropathy (MN) and crescents in the absence of secondary MN, anti-glomerular basement membrane (GBM) antibodies, and anti-neutrophil cytoplasmic antibodies (ANCA).

METHODS

We compared clinicopathological and prognostic features in 16 MN patients with crescents (crescent group) and 38 MN patients without crescents (control group), in the absence of secondary MN, anti-GBM antibodies, and ANCA. Median follow-up periods in the crescent and control groups were 79 and 50 months, respectively.

RESULTS

Decreased estimated glomerular filtration rates (<50 mL/min/1.73 m), glomerulosclerosis, and moderate-to-severe interstitial fibrosis were more frequently observed in the crescent group than in the control group (P = 0.043, P = 0.004, and P = 0.035, respectively). Positive staining rates for glomerular IgG2 and IgG4 were significantly different between the 2 groups (P = 0.032, P = 0.006, respectively). Doubling of serum creatinine during follow-up was more frequently observed in the crescent group than in the control group (P = 0.002), although approximately two-thirds of patients in the crescent group were treated with immunosuppressive therapy. Crescent formation and interstitial fibrosis were risks for doubling of serum creatinine [hazard ratio (HR) = 10.506, P = 0.012; HR = 1.140, P = 0.009, respectively].

CONCLUSIONS

This is the first Japanese study demonstrating significant differences in clinicopathological and prognostic features between the 2 groups. Most patients in the crescent group may develop a long-term decline in renal function despite immunosuppressive therapy.

摘要

背景

美国和中国最近的三项研究报告了在无继发性膜性肾病(MN)、抗肾小球基底膜(GBM)抗体和抗中性粒细胞胞浆抗体(ANCA)的情况下,伴有新月体的MN患者的临床病理特征和短期预后。

方法

我们比较了16例伴有新月体的MN患者(新月体组)和38例无新月体的MN患者(对照组)在无继发性MN、抗GBM抗体和ANCA情况下的临床病理和预后特征。新月体组和对照组的中位随访期分别为79个月和50个月。

结果

与对照组相比,新月体组更常观察到估计肾小球滤过率降低(<50 mL/min/1.73 m²)、肾小球硬化和中度至重度间质纤维化(分别为P = 0.043、P = 0.004和P = 0.035)。两组间肾小球IgG2和IgG4的阳性染色率有显著差异(分别为P = 0.032、P = 0.006)。尽管新月体组约三分之二的患者接受了免疫抑制治疗,但随访期间血清肌酐翻倍在新月体组比对照组更常见(P = 0.002)。新月体形成和间质纤维化是血清肌酐翻倍的危险因素[风险比(HR)= 10.506,P = 0.012;HR = 1.140,P = 0.009]。

结论

这是第一项表明两组临床病理和预后特征存在显著差异的日本研究。尽管进行了免疫抑制治疗,新月体组的大多数患者可能仍会出现肾功能的长期下降。

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