抗肾小球基底膜病(GBM)是一种快速进展的肾小球肾炎,在某些情况下,与其他疾病同时发生,例如抗中性粒细胞胞浆抗体(ANCA)相关的血管炎。很少,它也发生在膜性肾病(MN)。我们报道了一系列这样的病人,表征他们的长期随访。
案例系列。
2001年至2019年期间,12名患者转诊到1个医疗中心,患有抗GBM疾病和MN,其临床特征和病程进行了描述。
患者年龄从20岁到81岁,所有患者均出现严重急性肾损伤,需要在就诊时或之后不久进行透析。只有1例患者在就诊时出现肺部检查结果。主要病理为新月体性和坏死性肾小球肾炎,沿GBM对免疫球蛋白G进行线性染色,并伴有外膜电子致密免疫型沉积物。所有11名接受测试的患者均具有显着的抗GBM抗体滴度,但活检中5例磷脂酶A2受体(PLA2R)检测中只有1例阳性.八名患者接受了环磷酰胺治疗,泼尼松,和血浆置换;2例患者使用泼尼松和血浆置换;2例患者使用以利妥昔单抗为基础的方案。所有12例患者都出现了肾脏替代疗法需求的进展,但2例患者后来恢复了肾功能。研究的任何患者均未出现抗GBM疾病的复发。
活检和血清中PLA2R检测不完全,样本量有限,缺乏统一的治疗方案。
在这种情况下,同时出现抗GBM疾病和MN的特点是快速进展性肾小球肾炎和不良的肾脏结局.这些发现表明,早期诊断可能具有价值,并且需要确定更有效的治疗方案。
Anti-glomerular basement membrane (GBM) disease is a rapidly progressive glomerulonephritis which, in some instances, occurs concurrently with other diseases such as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Rarely, it also occurs with membranous nephropathy (MN). We report a series of such patients, characterizing their long-term follow up.
Case series.
Twelve patients referred to 1 medical center between 2001 and 2019 with anti-GBM disease and MN whose clinical characteristics and course were described.
Patients\' ages ranged from 20 to 81 years old, and all presented with severe acute kidney injury requiring dialysis on presentation or shortly thereafter. Only 1 patient had pulmonary findings on presentation. The predominant pathology was crescentic and necrotizing glomerulonephritis with linear staining for immunoglobulin G along the GBM associated with epimembranous electron-dense immune-type deposits. All 11 patients who were tested had significant titers of anti-GBM antibodies, but only 1 of the 5 tested for phospholipase A2 receptor (PLA2R) on biopsy was positive. Eight patients received therapy with cyclophosphamide, prednisone, and plasmapheresis; 2 patients with prednisone and plasmapheresis; and 2 with rituximab-based regimens. Progression to a requirement for kidney replacement therapy occurred in all 12 patients, but 2 patients later recovered kidney function. Recurrence of anti-GBM disease did not occur for any of the patients studied.
Incomplete testing for PLA2R in biopsy and serum, limited sample size, and lack of uniform treatment regimen.
In this
case series, the presentation of concurrent anti-GBM disease and MN was characterized by rapidly progressive glomerulonephritis and poor kidney outcomes. These findings suggest possible value from earlier diagnosis and the need for identification of more effective treatment regimens.