Nasr Samih H, D'Agati Vivette D, Said Samar M, Stokes Michael B, Appel Gerald B, Valeri Anthony M, 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. 2008 Sep;3(5):1282-8. doi: 10.2215/CJN.00740208. Epub 2008 May 28.
Pauci-immune necrotizing and crescentic glomerulonephritis (PNCGN) superimposed on diabetic glomerulosclerosis (DGS) is a rare occurrence. Only limited data on this dual glomerulopathy are available.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Twenty-three cases of PNCGN superimposed on DGS were identified from the archives of the Renal Pathology Laboratory of Columbia University. The clinical features, pathologic findings, and outcomes are described.
The majority of patients were white, elderly, and had longstanding diabetes. Patients presented with acute renal failure and an active urine sediment. Antinuclear cytoplasmic autoantibody (ANCA) testing was positive by indirect immunofluorescence in 18 of 22 patients. Sixteen patients had a P-ANCA pattern, 9 of whom underwent further testing and were found to be MPO-ANCA positive by enzyme-linked immunosorbent assay. Among the two patients with C-ANCA by indirect immunofluorescence, enzyme-linked immunosorbent assay was performed in one and revealed PR3-ANCA. Eight patients had extrarenal manifestations of vasculitis, including 6 with pulmonary hemorrhage. At the time of presentation and renal biopsy, 11 patients required hemodialysis. The mean percentages of glomeruli with cellular crescents, fibrous crescents, and necrosis were 24.9, 8.4, and 12.9, respectively. Most patients were treated with cyclophosphamide and prednisone. At a mean follow-up of 14.6 mo (available in 21 patients), 8 patients had died and 8 of the remaining 13 patients had reached end-stage renal disease. Correlates of end-stage renal disease were hemodialysis at presentation and the degree of DGS.
PNCGN may occur superimposed on DGS. The prognosis for this dual glomerulopathy is dismal despite aggressive therapy.
合并糖尿病肾小球硬化症（DGS）的寡免疫性坏死性新月体性肾小球肾炎（PNCGN）较为罕见。关于这种双重肾小球病的资料有限。
设计、研究地点、参与者及测量方法：从哥伦比亚大学肾脏病理实验室档案中识别出23例合并DGS的PNCGN病例。描述了其临床特征、病理表现及转归。
大多数患者为白人、老年人，且患有长期糖尿病。患者表现为急性肾衰竭及活跃的尿沉渣。22例患者中18例通过间接免疫荧光法检测抗核细胞质自身抗体（ANCA）呈阳性。16例患者为P-ANCA型，其中9例进一步检测，通过酶联免疫吸附测定法发现为MPO-ANCA阳性。间接免疫荧光法检测为C-ANCA的2例患者中，1例进行了酶联免疫吸附测定，结果显示为PR3-ANCA。8例患者有血管炎的肾外表现，其中6例有肺出血。就诊及肾活检时，11例患者需要血液透析。有细胞性新月体、纤维性新月体及坏死的肾小球平均百分比分别为24.9%、8.4%和12.9%。大多数患者接受了环磷酰胺和泼尼松治疗。平均随访14.6个月（21例患者可获得随访数据），8例患者死亡，其余13例患者中有8例进展至终末期肾病。终末期肾病的相关因素为就诊时血液透析及DGS的程度。
PNCGN可合并DGS出现。尽管积极治疗，这种双重肾小球病的预后仍很差。
