Kidney Research Institute, Division of Nephrology, West China Hospital, Sichuan University, Chengdu, 610041, China.
Department of Pathology, West China Hospital, Sichuan University, Chengdu, 610041, China.
BMC Nephrol. 2021 Feb 5;22(1):53. doi: 10.1186/s12882-021-02232-1.
Anti-glomerular basement membrane (anti-GBM) disease is an organ-specific autoimmune disease that involves the lung and kidneys and leads to rapid glomerulonephritis progression, with or without diffuse alveolar hemorrhage, and even respiratory failure. Classic cases of anti-GBM disease are diagnosed based on the presence of the anti-GBM antibody in serum samples and kidney or lung biopsy tissue samples. However, atypical cases of anti-GBM disease are also seen in clinical practice.
We herein report the rare case of a patient with atypical anti-GBM disease whose serum was negative for the anti-GBM antibody but positive for the myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (p-ANCA) and another atypical ANCA. Laboratory test results showed severe renal insufficiency with a creatinine level of 385 μmol/L. Renal biopsy specimen analysis revealed 100% glomeruli with crescents; immunofluorescence showed immunoglobulin G (IgG) linearly deposited alongside the GBM. Finally, the patient was discharged successfully after treatment with plasmapheresis, methylprednisolone and prednisone.
This patient, whose serum was negative for the anti-GBM antibody but positive for p-ANCA and another atypical ANCA, had a rare case of anti-GBM disease. Insights from this unusual case might help physicians diagnose rare forms of glomerulonephritis and treat affected patients in a timely manner.
抗肾小球基底膜 (anti-GBM) 病是一种器官特异性自身免疫性疾病，涉及肺和肾脏，导致快速肾小球肾炎进展，伴有或不伴有弥漫性肺泡出血，甚至呼吸衰竭。经典的抗 GBM 病病例是基于血清样本和肾脏或肺活检组织样本中存在抗 GBM 抗体来诊断的。然而，临床上也可见到不典型的抗 GBM 病病例。
我们在此报告一例罕见的不典型抗 GBM 病患者，其血清抗 GBM 抗体阴性，但髓过氧化物酶 (MPO) 抗中性粒细胞胞质抗体 (p-ANCA) 和另一种非典型 ANCA 阳性。实验室检查结果显示严重的肾功能不全，肌酐水平为 385μmol/L。肾活检标本分析显示 100%的肾小球有新月体；免疫荧光显示 IgG 沿 GBM 线性沉积。最后，患者在接受血浆置换、甲基强的松龙和泼尼松治疗后成功出院。
该患者血清抗 GBM 抗体阴性，但 p-ANCA 和另一种非典型 ANCA 阳性，患有罕见的抗 GBM 病。从这个不寻常的病例中获得的见解可能有助于医生及时诊断罕见形式的肾小球肾炎并治疗受影响的患者。
