Nasr Samih H, Said Samar M, Valeri Anthony M, Stokes Michael B, Masani Naveed N, D'Agati Vivette D, Markowitz Glen S
Department of Pathology, Columbia University, College of Physicians & Surgeons, 630 West 168th Street, VC14-224, New York, NY 10032, USA.
Clin J Am Soc Nephrol. 2009 Feb;4(2):299-308. doi: 10.2215/CJN.04060808. Epub 2009 Jan 21.
Only rare cases of concurrent membranous glomerulonephritis (MGN) and antineutrophil cytoplasmic antibody (ANCA)-associated necrotizing and crescentic glomerulonephritis (NCGN) have been reported.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: The authors report the clinical and pathologic findings in 14 patients with MGN and ANCA-associated NCGN.
The cohort consisted of eight men and six women with a mean age of 58.7 yr. ANCA positivity was documented by indirect immunofluorescence or ELISA in all patients. Indirect immunofluorescence was positive in 13 patients (seven P-ANCA, five C-ANCA, one atypical ANCA). ELISA was positive in nine of 10 patients (five MPO-ANCA, three PR3-ANCA, one MPO- and PR3-ANCA). Clinical presentation included heavy proteinuria (mean 24-hr urine protein 6.5 g/d), hematuria, and acute renal failure (mean creatinine 4.4 mg/dl). Pathologic evaluation revealed MGN and NCGN, with crescents involving a mean of 32% of glomeruli. On ultrastructural evaluation, the majority of cases showed stage I or II membranous changes. Follow-up was available for 13 patients, 12 of whom were treated with steroids and cyclophosphamide. At a mean follow-up of 24.3 mo, five patients progressed to ESRD, seven had stabilization or improvement in renal function, and one had worsening renal function. Five patients, including three with ESRD, died during the follow-up period. The only independent predictor of progression to ESRD was serum creatinine at biopsy.
MGN with ANCA-associated NCGN is a rare dual glomerulopathy seen in patients with heavy proteinuria, acute renal failure, and active urine sediment. Prognosis is variable, with 50% of patients reaching endpoints of ESRD or death.
仅有罕见病例报道过膜性肾小球肾炎（MGN）与抗中性粒细胞胞浆抗体（ANCA）相关的坏死性新月体性肾小球肾炎（NCGN）并存。
设计、研究地点、参与者及测量指标：作者报告了14例MGN合并ANCA相关NCGN患者的临床及病理表现。
该队列包括8名男性和6名女性，平均年龄58.7岁。所有患者均通过间接免疫荧光法或酶联免疫吸附测定（ELISA）证实ANCA阳性。间接免疫荧光法在13例患者中呈阳性（7例P-ANCA、5例C-ANCA、1例非典型ANCA）。ELISA在10例患者中的9例呈阳性（5例髓过氧化物酶（MPO）-ANCA、3例蛋白酶3（PR3）-ANCA、1例MPO和PR3-ANCA）。临床表现包括大量蛋白尿（平均24小时尿蛋白6.5g/d）、血尿和急性肾衰竭（平均肌酐4.4mg/dl）。病理评估显示为MGN和NCGN，新月体平均累及32%的肾小球。超微结构评估显示，大多数病例表现为I期或II期膜性改变。13例患者有随访资料，其中12例接受了类固醇和环磷酰胺治疗。平均随访24.3个月时，5例患者进展为终末期肾病（ESRD），7例患者肾功能稳定或改善，1例患者肾功能恶化。5例患者（包括3例ESRD患者）在随访期间死亡。活检时血清肌酐是进展为ESRD的唯一独立预测因素。
MGN合并ANCA相关NCGN是一种罕见的双重肾小球病，见于大量蛋白尿、急性肾衰竭和活动性尿沉渣的患者。预后不一，50%的患者达到ESRD或死亡终点。
