Adapa Sreedhar, Konala Venu Madhav, Hou Jean, Naramala Srikanth, Agrawal Nikhil, Dhingra Hemant, Aronow Wilbert S
Division of Nephrology, The Nephrology Group, Fresno, CA, USA.
Department of Internal Medicine/Division of Medical Oncology, Ashland Bellefonte Cancer Center, Ashland, KY, USA.
Ann Transl Med. 2019 Jun;7(11):246. doi: 10.21037/atm.2019.04.60.
A 46-year-old female presented with a chief complaint of fatigue and intermittent painless gross hematuria for one month. The patient was fluid overloaded on physical examination and noted to be in acute renal failure with a serum creatinine of 10.8 mg/dL. The patient was emergently started on hemodialysis. Serologies were negative for antinuclear antibody (ANA), anti-neutrophilic cytoplasmic antibody (ANCA), and anti-glomerular basement membrane (anti-GBM) antibody. However, renal biopsy revealed 90% glomerular involvement by temporally heterogeneous crescents ranging from cellular to fibrous. Immunofluorescence studies revealed strong, linear glomerular capillary wall staining for immunoglobulin G (IgG). Although the patient was treated with pulse dose steroids and cyclophosphamide, the patient ultimately developed infectious complications from immunosuppression, and treatment was terminated. This case highlights the atypical presentation of anti-GBM disease diagnosed based on renal biopsy with negative serologies. Although rare, the possibility of atypical anti-GBM antibodies which are not detected by standard commercial assays should be considered in such cases.
一名46岁女性，主要症状为疲劳和间歇性无痛肉眼血尿1个月。体格检查发现患者体液超负荷，血清肌酐为10.8mg/dL，处于急性肾衰竭状态。患者紧急开始血液透析。抗核抗体（ANA）、抗中性粒细胞胞浆抗体（ANCA）和抗肾小球基底膜（抗GBM）抗体血清学检查均为阴性。然而，肾活检显示90%的肾小球有不同时期的异质性新月体累及，从细胞性到纤维性。免疫荧光研究显示免疫球蛋白G（IgG）在肾小球毛细血管壁呈强线性染色。尽管患者接受了大剂量脉冲类固醇和环磷酰胺治疗，但最终因免疫抑制出现感染并发症，治疗终止。该病例突出了血清学阴性但经肾活检诊断的抗GBM病的非典型表现。虽然罕见，但在此类病例中应考虑存在标准商业检测无法检测到的非典型抗GBM抗体的可能性。
