Immune complex deposits in ANCA-associated crescentic glomerulonephritis: A study of 126 cases
Immune complex deposits in ANCA-associated crescentic glomerulonephritis: A study of 126 cases
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DOI：
10.1111/j.1523-1755.2004.00632.x
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发表时间：
2004-06-01
影响因子：
19.6
通讯作者：
Eustace, JA
中科院分区：
文献类型：
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作者：
Haas, M;Eustace, JA
背景与抗神经细胞胞质自身抗体（ANCA）相关的坏死性和新月体性肾小球肾炎通常被称为“少免疫性”;然而，在这种情况下，肾活检在免疫荧光和/或电子显微镜研究中显示肾小球内有一些免疫复合物沉积并不罕见。ANCA-肾小球肾炎中这种沉积物的组成和肾小球内定位尚未得到广泛研究，其潜在的病理和临床意义尚不清楚，尽管一些人类和动物研究表明免疫复合物和ANCA在产生更严重的肾小球肾炎中可能存在协同作用。对126例肾活检的电子显微镜照片进行检查，以确定电子致密沉积物的存在、数量和位置，这些肾活检显示坏死性/新月体性肾小球肾炎，其特征为ANCA血清学阳性[C-ANCA、抗蛋白酶3（抗PR 3）或抗髓过氧化物酶（MPO）]或坏死性动脉炎，但不存在已知的ANCA结果。这种沉积物的存在或不存在与组织学检查结果（肾小球新月体和节段性坏死性病变的分数，系膜和毛细血管内细胞过多），免疫荧光结果和临床数据，包括血清肌酐和24小时尿蛋白水平在活检时。68例（54%）活检显示肾小球免疫复合物沉积在电子显微镜下;后者的87%还显示至少一种免疫球蛋白或补体成分的阳性免疫荧光结果，尽管在大多数情况下染色相对较轻（除8例外，所有病例在0至4+量表上均小于或等于2+）。近一半的活检阴性沉积物的电子显微镜也显示出阳性免疫荧光结果，虽然甚至比在电子显微镜沉积物的情况下，这些活检中的免疫荧光染色的强度通常非常弱（在大多数情况下痕量或痕量至1+，没有>2+）。在电镜下有沉积物的活检组织中，50%的肾小球簇内细胞过多，而没有沉积物的活检组织中仅14%的肾小球簇内细胞过多;在每组中，这通常是轻度的系膜细胞过多。值得注意的是，电子显微镜下沉积物的存在与较高的蛋白尿中位数水平相关（3.2 g/24小时对1.3 g/24小时，P
Background. Necrotizing and crescentic glomerulonephritis related to antineutrophil cytoplasmic autoantibodies (ANCA) is typically referred to as "pauci-immune"; however, it is not unusual for renal biopsies in such cases to exhibit some immune complex deposition within glomeruli on immunofluorescence and/or electron microscopic study. The composition and intraglomerular localization of such deposits in ANCA-glomerulonephritis has not been widely studied, and their potential pathologic and clinical significance is not clear, although a possible synergistic effect between immune complexes and ANCA in producing more severe glomerulonephritis is suggested by some human and animal studies.Methods. Electron micrographs from 126 renal biopsies showing necrotizing/crescentic glomerulonephritis characterized by positive ANCA serology [C-ANCA, anti-proteinase 3 (anti-PR3), or anti-myeloperoxidase (MPO)] or necrotizing arteritis in the absence of known ANCA results were examined for the presence, quantity, and location of electron-dense deposits. The presence or absence of such deposits was correlated with histologic findings (fraction of glomeruli with crescents and segmental necrotizing lesions, mesangial and endocapillary hypercellularity),immunofluorescence findings, and clinical data, including serum creatinine and 24-hour urine protein levels at the time of biopsy.Results. Sixty-eight (54%) of these biopsies showed glomerular immune complex deposits on electron microscopy; 87% of the latter also showed positive immunofluorescence findings for at least one immunoglobulin or complement component, although staining was relatively mild in most instances (less than or equal to2+ on a 0 to 4+ scale in all but eight cases). Nearly half of biopsies negative for deposits by electron microscopy also showed positive immunofluorescence findings, though even more so than in cases with deposits on electron microscopy the intensity of immunofluorescence staining in these biopsies was typically very weak (trace or trace to 1+ in most cases, none >2+). Hypercellularity within the glomerular tuft was seen in 50% of biopsies with deposits on electron microscopy but only 14% of those without deposits; in each group this was usually mild and mesangial. Notably, the presence of deposits on electron microscopy was associated with a higher median level of proteinuria (3.2 versus 1.3 g/24 hours, P