Rutgers Abraham, Slot Marjan, van Paassen Pieter, van Breda Vriesman Peter, Heeringa Peter, Tervaert Jan Willem Cohen
Department of Clinical and Experimental Immunology, Cardiovascular Research Institute Maastricht, Maastricht University, The Netherlands.
Am J Kidney Dis. 2005 Aug;46(2):253-62. doi: 10.1053/j.ajkd.2005.05.003.
In a substantial proportion of patients with crescentic glomerulonephritis (CGN), both anti-glomerular basement membrane (GBM) antibodies and antineutrophil cytoplasmic antibodies (ANCAs) with specificity for myeloperoxidase (MPO-ANCA) are detected. In the present study, we questioned whether histological and clinical features of patients with both ANCA and anti-GBM antibodies differ from those of patients with either ANCA or anti-GBM alone.
We reviewed the Limburg renal biopsy registry (1978 to 2003; n = 1,373) for cases of CGN. The presence of linear fluorescence on renal biopsy and the presence of ANCA and/or anti-GBM antibodies were measured. Subsequently, we assessed patient characteristics and follow-up and compared histological findings among the different groups.
We identified 46 MPO-ANCA-positive, 10 double-positive, and 13 anti-GBM-positive patients. Mean ages were 63, 64, and 52 years (P = 0.04), and serum creatinine levels were 5.0, 10.3, and 9.6 mg/dL (445, 910, and 850 micromol/L), respectively (P = 0.01). Granulomatous periglomerular inflammation was found in either MPO-ANCA- or double-positive patients, but not in anti-GBM-positive patients with CGN without MPO-ANCAs. Patient survival among the 3 groups was different, although not statistically significant (log rank P = 0.17, with 75%, 79%, and 100% alive at 1 year, respectively). Renal survival analysis showed significant differences among the 3 groups (P = 0.04, with 65%, 10%, and 15% off dialysis therapy at 1 year, respectively).
In patients with both anti-GBM antibodies and MPO-ANCAs, histological findings differ from those of patients with anti-GBM antibodies only. However, renal survival in these patients is not better than that in anti-GBM-positive patients and is worse compared with patients with MPO-ANCAs only.
在相当一部分新月体性肾小球肾炎（CGN）患者中，可检测到抗肾小球基底膜（GBM）抗体和抗中性粒细胞胞浆抗体（ANCA），其中抗髓过氧化物酶ANCA（MPO-ANCA）具有特异性。在本研究中，我们探讨了同时存在ANCA和抗GBM抗体的患者的组织学和临床特征是否与仅存在ANCA或抗GBM抗体的患者不同。
我们回顾了林堡肾脏活检登记处（1978年至2003年；n = 1373）中CGN病例。检测肾活检时线性荧光的存在情况以及ANCA和/或抗GBM抗体的存在情况。随后，我们评估了患者的特征和随访情况，并比较了不同组之间的组织学发现。
我们确定了46例MPO-ANCA阳性、10例双阳性和13例抗GBM阳性患者。平均年龄分别为63岁、64岁和52岁（P = 0.04），血清肌酐水平分别为5.0、10.3和9.6mg/dL（445、910和850μmol/L）（P = 0.01）。在MPO-ANCA阳性或双阳性患者中发现了肉芽肿性肾小球周围炎症，但在无MPO-ANCA的CGN抗GBM阳性患者中未发现。三组患者的生存情况不同，尽管无统计学意义（对数秩检验P = 0.17，1年时的生存率分别为75%、79%和100%）。肾脏生存分析显示三组之间存在显著差异（P = 0.04，1年时停止透析治疗的比例分别为65%、10%和15%）。
在同时存在抗GBM抗体和MPO-ANCA的患者中，组织学发现与仅存在抗GBM抗体的患者不同。然而，这些患者的肾脏生存率并不优于抗GBM阳性患者，且与仅存在MPO-ANCA的患者相比更差。
