Liu Ya-Ru, Ning Wei, Qi Yuan-Yuan, Zhao Zhan-Zheng
Department of Nephrology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China.
The First Clinical Medical College, Zhengzhou University, Zhengzhou, Henan, China.
Ren Fail. 2025 Dec;47(1):2502607. doi: 10.1080/0886022X.2025.2502607. Epub 2025 May 29.
Anti-glomerular basement membrane disease (anti-GBM disease) is a rare autoimmune disease, traditionally considered a monophasic autoimmune disorder, but more and more cases of recurrence have been reported. Through a review of the published literature and analysis of recurrent cases from our institution, we identified key predisposing factors including smoking (21.7%), hydrocarbon exposure (7.2%), prodromal infections (12.0%), and comorbid autoimmune conditions (12.0%). Notably, 43.5% (20/46) of recurrences occurred without identifiable triggers. There are not a few antibody-negative recurrences, challenging the reliability of serological monitoring alone for disease activity, and such recurrences may have an underlying unrecognized pathogenesis. Clinical presentations at recurrence varied: 38.6% of the patients had both lung and kidney involvement, 33.3% had lung involvement only, and 28.1% had kidney involvement only. Patients with concurrent systemic autoimmune diseases exhibited universally poor renal outcomes.
抗肾小球基底膜病(anti-GBM病)是一种罕见的自身免疫性疾病,传统上被认为是单相自身免疫性疾病,但越来越多的复发病例被报道。通过回顾已发表的文献并分析我们机构的复发病例,我们确定了关键的诱发因素,包括吸烟(21.7%)、接触碳氢化合物(7.2%)、前驱感染(12.0%)和合并自身免疫性疾病(12.0%)。值得注意的是,43.5%(20/46)的复发没有可识别的触发因素。抗体阴性复发并不少见,这对仅通过血清学监测疾病活动的可靠性提出了挑战,并且此类复发可能存在未被认识的潜在发病机制。复发时的临床表现各不相同:38.6%的患者同时有肺和肾受累,33.3%仅肺部受累,28.1%仅肾脏受累。并发全身性自身免疫性疾病的患者普遍肾脏预后较差。