Yamada Takehisa, Kashiwagi Tetsuya, Shimizu Akira, Tsuruoka Shuichi
Department of Nephrology, Nippon Medical School Chiba Hokusoh Hospital.
Department of Analytic Human Pathology, Nippon Medical School.
J Nippon Med Sch. 2018;85(5):259-264. doi: 10.1272/jnms.JNMS.2018_85-41.
The physical condition of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated rapidly progressive glomerulonephritis (RPGN) is at times too critical for physicians to undertake a renal biopsy. In such cases, physicians need to start treatments without determining the pathological features of the disease. However, the prevalent clinical practice guidelines for ANCA-associated RPGN in Japan do not necessitate the pathological findings in a renal biopsy for determining first-line treatments. The aim of this study was to assess the correlation between the pathological findings and the clinical guideline-based treatments of ANCA-associated RPGN.
We investigated clinical and pathological features of patients who were admitted to our hospital to undergo a percutaneous renal biopsy for the diagnosis of ANCA-associated RPGN and were treated per clinical practice guidelines formulated by the Japanese Society of Nephrology. We divided patients into the following three groups according to their treatments: (a) group A, oral intake of prednisolone (PSL) only; (b) group B, methylprednisolone (mPSL) pulse therapy followed by oral intake of PSL; and (c) group C, mPSL pulse therapy followed by oral intake of PSL and the administration of immunosuppressive agents. We compared the crescent formation ratio (CFR) in glomeruli in each treatment group.
The median CFR (%; ±standard deviation [SD]) in group A (8.7%±7.7%) was significantly lower than that in groups B (30.2%±15.7%) and C (71.3%±25.5%); group A vs. group B, P<0.05; group A vs. group C, P<0.0001. In addition, the median CFR in group B was significantly lower than that in group C (P<0.01). The median serum creatinine levels (mg/dL; ±SD) in both groups A (2.03±0.96 mg/dL) and B (1.90±0.86 mg/dL) were significantly lower than that in group C (4.30±1.63 mg/dL); group A vs. group C, P<0.01; group B vs. group C, P<0.01. No significant difference was observed in the serum levels of C-reactive protein and ANCA in each treatment group. Although the pathological features of renal biopsy are not included in the factors in the clinical scores described in the guidelines, guideline-based treatments reflected the severity of the crescent formation in each treatment group.
This study suggests that the clinical practice guidelines for ANCA-associated RPGN used in Japan facilitate physicians to determine disease management in patients presenting difficulties in undergoing a renal biopsy.
抗中性粒细胞胞浆抗体（ANCA）相关的急进性肾小球肾炎（RPGN）患者的身体状况有时对医生来说过于危急，无法进行肾活检。在这种情况下，医生需要在不确定疾病病理特征的情况下开始治疗。然而，日本ANCA相关RPGN的现行临床实践指南并不要求肾活检的病理结果来确定一线治疗方案。本研究的目的是评估ANCA相关RPGN的病理结果与基于临床指南的治疗之间的相关性。
我们调查了因诊断ANCA相关RPGN入院接受经皮肾活检并按照日本肾脏病学会制定的临床实践指南进行治疗的患者的临床和病理特征。我们根据治疗方法将患者分为以下三组：（a）A组，仅口服泼尼松龙（PSL）；（b）B组，甲泼尼龙（mPSL）冲击治疗后口服PSL；（c）C组，mPSL冲击治疗后口服PSL并给予免疫抑制剂。我们比较了各治疗组肾小球中的新月体形成率（CFR）。
A组的CFR中位数（%；±标准差[SD]）（8.7%±7.7%）显著低于B组（30.2%±15.7%）和C组（71.3%±25.5%）；A组与B组比较，P<0.05；A组与C组比较，P<0.0001。此外，B组的CFR中位数显著低于C组（P<0.01）。A组（2.03±0.96mg/dL）和B组（1.90±0.86mg/dL）的血清肌酐水平中位数均显著低于C组（4.30±1.63mg/dL）；A组与C组比较，P<0.01；B组与C组比较，P<0.01。各治疗组的C反应蛋白和ANCA血清水平未观察到显著差异。虽然肾活检的病理特征未包含在指南中描述的临床评分因素中，但基于指南的治疗反映了各治疗组新月体形成的严重程度。
本研究表明，日本使用的ANCA相关RPGN临床实践指南有助于医生确定在进行肾活检时存在困难的患者疾病管理方案。
