Garo Florian, Aglae Cédric, Perrochia Hélène, Ahmadpoor Pedram, Daniel Laurent, Moranne Olivier
Nephrology-Dialysis-Apheresis Unit, Nimes University Hospital, Nimes, France.
IDESP Inserm-UM University Montpellier-Nimes, Montpellier, France.
Case Rep Nephrol Dial. 2022 Apr 28;12(1):73-80. doi: 10.1159/000521862. eCollection 2022 Jan-Apr.
Kidney biopsy is the gold standard for diagnosing glomerular kidney disease. Some authors debate the necessity of systematically performing kidney biopsies in ANCA-associated vasculitis (AAV) to confirm the diagnosis and assess the severity of renal damage. Nevertheless, kidney involvement is considered an organ-threatening disease requiring an aggressive immunosuppressive regimen. We present a series of 4 cases with a high clinical suspicion of ANCA-associated crescentic glomerulonephritis based on rising serum creatinine, presence of proteinuria and/or hematuria, and presence of ANCA with specificity against PR-3 or MPO. The main diagnosis, however, was arterionephrosclerosis without renal AAV. Certain comorbidities, such as diabetes and/or high blood pressure, can quickly mimic progressive glomerulonephritis. In addition, some patients with AAV do not have high creatinine, proteinuria, or hematuria levels. ANCA alone is not specific to AAV and has a poor positive predictive value. The main concern is to prevent the unnecessary, inappropriate complications of heavy immunosuppression, i.e., serious infections or risk of future malignancies. Kidney pathological confirmation is important in patients with no compatible extra-renal manifestations of AAV or any other possible renal diagnosis such as may be found in polyvascular disease or diabetic patients.
肾活检是诊断肾小球疾病的金标准。一些作者对在抗中性粒细胞胞浆抗体相关性血管炎(AAV)中系统地进行肾活检以确诊并评估肾损伤严重程度的必要性存在争议。然而,肾脏受累被认为是一种威胁器官的疾病,需要积极的免疫抑制方案。我们报告了一系列4例高度怀疑抗中性粒细胞胞浆抗体相关性新月体性肾小球肾炎的病例,这些病例基于血清肌酐升高、蛋白尿和/或血尿的存在以及针对蛋白酶3(PR-3)或髓过氧化物酶(MPO)的抗中性粒细胞胞浆抗体的存在。然而,主要诊断为动脉硬化性肾病,而非肾AAV。某些合并症,如糖尿病和/或高血压,可迅速模拟进行性肾小球肾炎。此外,一些AAV患者的肌酐、蛋白尿或血尿水平并不高。单独的抗中性粒细胞胞浆抗体对AAV并不特异,其阳性预测值较低。主要关注点在于预防过度免疫抑制带来的不必要、不适当的并发症,即严重感染或未来发生恶性肿瘤的风险。对于没有AAV肾外表现或任何其他可能的肾脏诊断(如在多血管疾病或糖尿病患者中可能出现的诊断)的患者,肾脏病理确诊很重要。