Renal Division, Peking University First Hospital, Beijing, China.
Renal Division, Shanxi Medical University Second Hospital, Shanxi Kidney Disease Institute, Taiyuan, China.
Ren Fail. 2022 Dec;44(1):1123-1129. doi: 10.1080/0886022X.2022.2097405.
Anti-glomerular basement membrane (anti-GBM) disease is a rare but severe autoantibody-mediated immune disorder. The typical clinical presentation includes rapidly progressive glomerulonephritis and often concurrent pulmonary hemorrhage. The present study is aimed to investigate the therapeutic effects of rituximab either used alone or with other immunosuppressants.
Eight patients diagnosed with anti-GBM disease and treated with rituximab from 2014 to 2020 were retrospectively reviewed.
Eight patients included 5 males and 3 females with a median age of 58.5 years. They all presented severe kidney injuries and 1 patient had lung hemorrhage. At diagnosis, the median of serum creatinine was 246 µmol/L (ranging from 91 to 850 µmol/L), with 3 patients requiring dialysis. All of them received corticosteroids and plasmapheresis. Rituximab was given as either standard four weekly doses or one pulse ranging from 100 to 600 mg. After a median follow-up of 34.5 months, kidney function was partially recovered or stabilized in 5/8 (62.5%) patients, free of dialysis. Anti-GBM antibodies remained undetected in all patients during follow-up. No severe adverse effect associated with rituximab was observed.
Rituximab may be an alternative therapy in the treatment of patient with severe or refractory anti-GBM disease.
抗肾小球基底膜(anti-GBM)病是一种罕见但严重的自身抗体介导的免疫性疾病。典型的临床表现包括急进性肾小球肾炎,常并发肺出血。本研究旨在探讨利妥昔单抗单独或与其他免疫抑制剂联合应用的治疗效果。
回顾性分析 2014 年至 2020 年收治的 8 例诊断为抗 GBM 病并接受利妥昔单抗治疗的患者。
8 例患者中男 5 例,女 3 例,中位年龄 58.5 岁。所有患者均表现为严重的肾脏损伤,1 例有肺出血。诊断时,血清肌酐中位数为 246μmol/L(范围 91-850μmol/L),3 例患者需要透析。所有患者均接受了皮质类固醇和血浆置换治疗。利妥昔单抗的使用剂量为标准的每周 4 次或 1 次 100-600mg 的脉冲剂量。中位随访 34.5 个月后,5/8(62.5%)患者肾功能部分恢复或稳定,无需透析。所有患者在随访期间均未检测到抗 GBM 抗体。未观察到与利妥昔单抗相关的严重不良反应。
利妥昔单抗可能是治疗重症或难治性抗 GBM 病的一种替代治疗方法。