Clin Nephrol. 2022 Dec;98(6):280-287. doi: 10.5414/CN110851.
To study the clinical profile of anti-glomerular basement membrane (anti-GBM) disease and its outcome with two different treatment regimens comprising either cyclophosphamide (CYC) or rituximab (RTX).
A retrospective analysis of anti-GBM crescentic glomerulonephritis patients admitted to our hospital over 5 years.
14 patients were diagnosed with anti-GBM crescentic glomerulonephritis. The mean duration of symptoms was 3.6 ± 1.9 weeks. All patients presented with rapidly progressive glomerulonephritis (RPGN). Five (35.7%) patients had concomitant urinary tract infection (UTI), 2 (14.3% had underlying type 2 diabetes mellitus, 5 (35.7%) patients also had positive anti-neutrophil cytoplasmic antibodies (ANCA), and 9 (64.3%) were dialysis-dependent at presentation. Four (28.6%) patients developed diffuse alveolar hemorrhage (DAH). All patients received baseline corticosteroids, and 7 (50%) patients also received plasmapheresis. Nine (64.3%) patients were treated with CYC, and 3 (21.4%) patients received RTX. In the CYC arm, 2 (28.6%) patients died, 3 had end-stage kidney disease (ESKD) at 3 months, and 2 had chronic kidney disease (CKD) stage III at 3 months of follow-up. Two patients were lost to follow-up. In the RTX arm, all 3 patients survived with no incidence of DAH, 1 patient each had ESKD and CKD stage III, and 1 dialysis-dependent patient achieved normal kidney function at the end of 3 months.
Most patients presented late with dialysis-dependent renal failure, and many had concomitant UTI. Concomitant infection causes diagnostic confusion with RPGN and DAH, which delays diagnosis and treatment. RTX as an alternative to CYC in addition to baseline corticosteroids and/or plasmapheresis and is associated with favorable outcomes.
研究两种不同治疗方案（环磷酰胺[CYC]或利妥昔单抗[RTX]）治疗抗肾小球基底膜（anti-GBM）疾病的临床特征及其结果。
对我院 5 年内收治的抗 GBM 新月体肾炎患者进行回顾性分析。
共诊断出 14 例抗 GBM 新月体肾炎患者，症状平均持续 3.6±1.9 周。所有患者均表现为急进性肾小球肾炎（RPGN）。5（35.7%）例患者合并尿路感染（UTI），2（14.3%）例患者合并 2 型糖尿病，5（35.7%）例患者抗中性粒细胞胞浆抗体（ANCA）阳性，9（64.3%）例患者就诊时依赖透析。4（28.6%）例患者发生弥漫性肺泡出血（DAH）。所有患者均接受基础皮质类固醇治疗，7（50%）例患者接受血浆置换。9（64.3%）例患者接受 CYC 治疗，3（21.4%）例患者接受 RTX 治疗。在 CYC 组中，2（28.6%）例患者死亡，3 例患者在 3 个月时进展为终末期肾病（ESKD），2 例患者在 3 个月随访时进展为慢性肾脏病（CKD）Ⅲ期。2 例患者失访。在 RTX 组中，所有 3 例患者均存活，无 DAH 发生，1 例患者进展为 ESKD，1 例患者进展为 CKD Ⅲ期，1 例依赖透析的患者在 3 个月时肾功能恢复正常。
大多数患者就诊时已出现肾衰竭依赖透析，且多数合并 UTI。合并感染会导致 RPGN 和 DAH 的诊断出现混淆，从而延误诊断和治疗。在基础皮质类固醇和/或血浆置换的基础上，RTX 可替代 CYC，治疗效果较好。
