Division of Cardiology, Nephrology, Pulmonology and Neurology, Department of Internal Medicine, Asahikawa Medical University, 2-1-1-1 Midorigaoka-higashi, Asahikawa, Japan.
Reverse Translational Research Project, Center for Rare Disease Research, National Institutes of Biomedical Innovation, Health and Nutrition (NIBIOHN), Ibaraki, Japan.
Sci Rep. 2023 Sep 7;13(1):14771. doi: 10.1038/s41598-023-41909-5.
The nationwide clinical features of Japanese patients with primary nephrotic syndrome (NS), including minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), or membranous nephropathy (MN), have not yet been reported. We collected the clinical personal records of patients with primary NS between 2015 and 2018 from the national registry organized by the Japanese Ministry of Health, Labour, and Welfare. Overall, the demographics, chronic kidney disease classification based on glomerular filtration rate and albuminuria, and treatment of 6036 patients were collected: 3394 (56.2%) with MCD, 677 (11.2%) with FSGS, 1455 (24.1%) with MN, and 510 (8.5%) with others. MN patients were older than MCD and FSGS patients (67 vs. 42 and 47 years, respectively). Steroid-dependent NS or frequently relapsing NS was found in 70.2%, 40.5%, and 24.6%, whereas steroid-resistant NS was found in 6.4%, 36.0%, and 37.9% of patients in the MCD, FSGS, and MN, respectively. The present oral prednisolone use (mean dose, mg/day) was 87.2% (21.2), 80.9% (20.0), and 77.5% (18.8) of patients in the MCD, FSGS, and MN, respectively. The national registry of clinical personal records of primary NS could provide an informative insight into the characteristics, clinical features, and treatment approaches for patients with primary NS in Japan.
日本原发性肾病综合征(NS)患者的全国临床特征,包括微小病变病(MCD)、局灶节段性肾小球硬化(FSGS)或膜性肾病(MN),尚未有报道。我们从日本厚生劳动省组织的全国登记处收集了 2015 年至 2018 年间原发性 NS 患者的临床个人记录。总体而言,共收集了 6036 例患者的人口统计学资料、基于肾小球滤过率和蛋白尿的慢性肾脏病分类以及治疗情况:3394 例(56.2%)为 MCD,677 例(11.2%)为 FSGS,1455 例(24.1%)为 MN,510 例(8.5%)为其他类型。MN 患者比 MCD 和 FSGS 患者年龄大(分别为 67 岁、42 岁和 47 岁)。70.2%、40.5%和 24.6%的患者分别存在激素依赖性 NS 或频繁复发 NS,而 MCD、FSGS 和 MN 患者中激素抵抗性 NS 的比例分别为 6.4%、36.0%和 37.9%。目前 MCD、FSGS 和 MN 患者口服泼尼松的使用率(平均剂量,mg/天)分别为 87.2%(21.2)、80.9%(20.0)和 77.5%(18.8)。原发性 NS 的全国临床个人记录登记处可以为了解日本原发性 NS 患者的特征、临床特征和治疗方法提供有价值的信息。