■抗肾小球基底膜(抗GBM)疾病是一种罕见的器官特异性自身免疫性疾病。患者的总体和肾脏结局大多在小型队列中报道。我们的目的是研究临床概况,总生存率,我们中心抗肾小球基底膜病患者的肾脏存活率。
我们对2019年10月至2022年3月诊断为抗GBM疾病的患者的临床特征和肾脏生存率数据进行了回顾性分析,最低随访时间为12个月。
■研究中有15名患者,平均年龄为51.6±13.7岁。肾病学家意见的症状发作持续时间中位数为15(10-23)天。肾外表现见于呼吸道,耳鼻喉科,和神经系统。平均血清抗GBM滴度为154.5(14.9-263.5)U/mL。血清抗GBM滴度在13/15(86.6%)患者中存在,12/13(92.3%)患者高于参考范围。在12/15(80%)患者中评估了抗中性粒细胞胞浆抗体(ANCA)水平,9/12(75%)的水平更高。14例月牙超过50%的患者可进行肾活检。随着新月,坏死性病变,鲍曼胶囊破裂,还可见肉芽肿性病变。在最初的治疗中,对13名(86.6%)患者进行了类固醇脉冲,而8例(53.3%)患者接受了膜血浆置换。Inj.环磷酰胺和inj.利妥昔单抗分别给予8例(53.3%)和4例(26.6%)患者,分别。在临床特征上没有发现差异,肾活检特征,接受治疗,以及ANCA阳性的结果,除了年龄,与ANCA阴性患者相比,ANCA阳性患者年龄较大.在4例(26.6%)和6例(40%)患者中观察到一年的肾脏和患者生存率,分别。
■大多数抗GBM病患者都有活性沉积物,肌酐升高,和非特异性症状学。由于大多数患者患有晚期肾衰竭,因此肾脏和患者预后较差。
UNASSIGNED: Anti-glomerular basement membrane (anti-GBM) disease is a rare organ-specific autoimmune disease. The overall and renal outcomes of patients have mostly been reported in small-sized cohorts. We aimed to study the clinical profile, overall survival, and renal survival of anti-glomerular basement membrane disease patients at our center.
UNASSIGNED: We conducted a retrospective analysis of the data regarding the clinical profile and renal survival of patients diagnosed with anti-GBM disease from October 2019 to March 2022, having a minimum follow-up of 12 months.
UNASSIGNED: There were 15 patients in the study, with the mean age of presentation being 51.6 ± 13.7 years. The median duration of symptoms onset to the nephrologist opinion was 15 (10-23) days. The extrarenal manifestations were seen in the respiratory, otorhinolaryngological, and neurological systems. The mean serum anti-GBM titers were 154.5 (14.9-263.5) U/mL. Serum anti-GBM titers were present in 13/15 (86.6%) patients, and 12/13 (92.3%) patients had above the reference range. Anti-neutrophil cytoplasm antibody (ANCA) levels were assessed in 12/15 (80%) patients, and 9/12 (75%) had higher levels. Renal biopsy was available in 14 patients with more than 50% crescents. Along with crescents, necrotizing lesions, rupture of the Bowman\'s capsule, and granulomatous lesions were also seen. Among the initial therapies, the steroid pulse was given to 13 (86.6%) patients, whereas membrane plasmapheresis was given to 8 (53.3%) patients. Inj. cyclophosphamide and inj. rituximab were given to 8 (53.3%) and 4 (26.6%) patients, respectively. No difference was seen in clinical characteristics, renal biopsy features, treatment received, and outcomes with ANCA positivity except for age, where patients who were ANCA positive were older compared to patients who were ANCA negative. One-year renal and patient survival was seen in 4 (26.6%) and 6 (40%) patients, respectively.
UNASSIGNED: Most patients of anti-GBM disease have active sediments, raised creatinine, and non-specific symptomatology. There is poor renal and patient outcome as most patients present with advanced renal failure.