Florida State University, Sarasota, FL, USA.
J Investig Med High Impact Case Rep. 2021 Jan-Dec;9:23247096211013193. doi: 10.1177/23247096211013193.
Crescentic glomerulonephritis, also known as rapidly progressive glomerulonephritis, is a syndrome characterized by progressive and rapid deterioration of renal function over the course of weeks to months. Oliguria, hematuria, azotemia, and hypertension are characteristic features of this condition. Crescentic glomerulonephritis is further classified according to the staining pattern on immunofluorescence. In rare instances, a mixed pattern of injury is encountered as in the case of double antibody-positive rapidly progressive glomerulonephritis (RPGN). This case illustrates the challenge in treatment of double antibody-positive RPGN in an elderly female with no previous renal disease. The patient was found to be positive for anti-GBM antibody and MPO-ANCA. Treatment was initially targeted against MPO-ANCA as the biopsy was most consistent with this process; however, the patient failed to respond to treatment and was subsequently transitioned to oral cyclophosphamide directed against anti-GBM disease. In cases of doubly antibody-positive RPGN with anti-GBM disease and ANCA-associated vasculitis, initial treatment should focus on inducing remission of anti-GBM disease as double antibody-positive disease often presents with the aggressive morbidity and mortality seen in anti-GBM disease, and the chronic risk of relapse seen in ANCA-mediated vasculitis.
新月体肾小球肾炎，又称急进性肾小球肾炎，是一种以数周至数月内肾功能进行性和快速恶化为特征的综合征。少尿、血尿、氮质血症和高血压是该病的特征性表现。新月体肾小球肾炎根据免疫荧光染色模式进一步分类。在极少数情况下，如双抗体阳性急进性肾小球肾炎（RPGN），会遇到混合损伤模式。该病例说明了在无既往肾脏疾病的老年女性中治疗双抗体阳性 RPGN 的挑战。该患者抗肾小球基底膜抗体和 MPO-ANCA 阳性。治疗最初针对 MPO-ANCA，因为活检最符合这一过程；然而，该患者对治疗无反应，随后被转为口服环磷酰胺针对抗肾小球基底膜疾病。在双抗体阳性 RPGN 合并抗肾小球基底膜疾病和 ANCA 相关性血管炎的情况下，初始治疗应侧重于诱导抗肾小球基底膜疾病缓解，因为双抗体阳性疾病常表现出抗肾小球基底膜疾病的侵袭性发病率和死亡率，以及 ANCA 介导的血管炎的慢性复发风险。
