Department of Nephrology and Rheumatology, Kyorin University School of Medicine, 6-20-2, Mitaka-shi, Tokyo, 181-8611, Japan.
Department of Pathology, Kyorin University Hospital, Tokyo, Japan.
BMC Nephrol. 2020 Jul 17;21(1):283. doi: 10.1186/s12882-020-01947-x.
The prognosis for renal function in anti-GBM glomerulonephritis (anti-GBM GN) is extremely poor, and when renal impairment progresses severely, it is difficult to expect improvement. In addition, it is also known that once the disease activity can be controlled by aggressive treatment, its recurrence is rare. We experienced an anti-GBM GN that improved from severe renal dysfunction and relapsed. A possible cause was the superimpose of nephrotic syndrome due to minimal change disease (MCD).
A 30-year-old man was admitted to our hospital because of general malaise, fever, oliguria and renal dysfunction. The patient's laboratory data showed serum creatinine as high as 6.6 mg/dl, and severe inflammation (C-reactive protein 20.6 mg/dl). Anti-glomerular basement membrane antibody (anti-GBM Ab) was detected in his serum, which led to the diagnosis of anti-GBM GN. Treatment was initiated with high-dose glucocorticoid (GC) and plasma exchange therapy (PE), and the patient's renal function and oliguria improved rapidly and he was discharged 40 days after admission. Renal biopsy findings showed cellular crescents associated with linear IgG depositions along the glomerular tufts compatible with anti-GBM GN, but only about one-third of the glomeruli was involved, suggesting that it still remains an early stage of the disease. However, 2 months after discharge, he had a relapse and was readmitted due to severe proteinuria with positive anti-GBM Ab. On the second admission, after high-dose GC and PE combined with intravenous cyclophosphamide, and remission was achieved. Despite the relatively minor renal biopsy findings, the patient showed rapid renal dysfunction and relatively rapid improvement with our treatment. Electron microscopy of the renal biopsy tissue showed significant foot process effacement on podocytes in the apparently normal glomeruli, without electron dense deposits.
On the basis of clinical course and renal pathology, it is suggested that the present case was a rare complication of an early stage of anti-GBM GN and minimal change nephrotic syndrome. Although the simultaneous development of anti-GBM GN and MCD with anti-GBM antibody is unclear, it might have been precipitated by influenza infection or some unknown factor.
抗肾小球基底膜（anti-GBM）肾小球肾炎（anti-GBM GN）的肾功能预后极差，当肾功能严重受损时，难以期待改善。此外，已知一旦通过积极治疗控制疾病活动，其复发很少见。我们遇到了一例从严重肾功能障碍中改善并复发的抗-GBM GN。可能的原因是微小病变病（MCD）引起的肾病综合征重叠。
一名 30 岁男性因全身不适、发热、少尿和肾功能障碍而入院。患者的实验室数据显示血清肌酐高达 6.6mg/dl，且炎症严重（C 反应蛋白 20.6mg/dl）。他的血清中检测到抗肾小球基底膜抗体（anti-GBM Ab），导致抗-GBM GN 的诊断。治疗开始时使用高剂量糖皮质激素（GC）和血浆置换疗法（PE），患者的肾功能和少尿迅速改善，入院后 40 天出院。肾活检结果显示细胞性新月体，伴线性 IgG 沿肾小球足细胞沉积，符合抗-GBM GN，但只有大约三分之一的肾小球受累，表明仍处于疾病的早期阶段。然而，出院后 2 个月，他因大量蛋白尿和阳性抗-GBM Ab 而复发并再次入院。第二次入院后，联合使用高剂量 GC 和 PE 以及静脉环磷酰胺治疗，缓解病情。尽管肾活检结果相对较轻，但患者的肾功能迅速恶化，经我们治疗后迅速改善。肾活检组织的电子显微镜显示明显正常的肾小球中足细胞的显著足突消失，无电子致密沉积物。
根据临床过程和肾脏病理学，提示本例为抗-GBM GN 和微小病变肾病综合征早期罕见并发症。虽然抗-GBM GN 和 MCD 同时发生且与抗-GBM 抗体有关尚不清楚，但可能是由流感感染或一些未知因素引发。
