File Ibolya, Pucsok Klára, Trinn Csilla, Ujhelyi László, Balla József, Mátyus János
Debreceni Egyetem, Általános Orvostudományi Kar, Orvos- és Egészségtudományi Centrum Belgyógyászati Intézet, Nefrológiai Tanszék Debrecen Nagyerdei krt. 98. 4032.
Orv Hetil. 2013 Oct 27;154(43):1696-701. doi: 10.1556/OH.2013.29735.
Patients with renopulmonary syndrome who have both anti-neutrophil cytoplasmic and anti-glomerular basement membrane antibodies have been described since 1989.
The aim of the authors was to analyse the data of "double positive" patients diagnosed in their department, and compare these with previous studies.
During the last 16 years, 87 anti-neutrophil cytoplasmic antibody positive and 11 anti-glomerular basement membrane antibody positive patients were diagnosed. Four patients with anti-glomerular basement membrane antibodies (36%) had detectable anti-neutrophil cytoplasmic antibodies, 2 patients were positive for anti-myeloperoxidase and 2 patients for anti-proteinase 3.
In comparison with patients having anti-glomerular basement membrane antibodies, the double-positive patients were characterized by older age (median of 46 vs. 24 years), lack of male dominance (50% vs. 71%), more frequent presence of previous extrarenal symptoms (50% vs. 0%), and lower anti-glomerular basement membrane antibody levels (<100EU/ml: 100% vs. 29%). The double-positive patients had more favourable 1-year survival (100% vs. 71%), despite their older age and similar treatment regimen (immunosuppression 100% in both groups, plasmapheresis in 75% vs. 86%), but 1-year renal survival was not different (25% vs. 14%).
In agreement with literature data, about one third of patients with anti-glomerular basement membrane antibodies had detectable anti-neutrophil cytoplasmic antibodies, and the coexistence of the two antibodies may have clinical consequences.
自1989年以来，已有关于同时患有抗中性粒细胞胞浆抗体和抗肾小球基底膜抗体的肾肺综合征患者的描述。
作者的目的是分析在他们科室诊断出的“双阳性”患者的数据，并与之前的研究进行比较。
在过去16年中，诊断出87例抗中性粒细胞胞浆抗体阳性患者和11例抗肾小球基底膜抗体阳性患者。4例抗肾小球基底膜抗体阳性患者（36%）可检测到抗中性粒细胞胞浆抗体，2例抗髓过氧化物酶阳性，2例抗蛋白酶3阳性。
与抗肾小球基底膜抗体阳性患者相比，双阳性患者的特点是年龄较大（中位数46岁对24岁），无男性优势（50%对71%），既往肾外症状出现频率更高（50%对0%），抗肾小球基底膜抗体水平更低（<100EU/ml：100%对29%）。双阳性患者1年生存率更高（100%对71%），尽管他们年龄较大且治疗方案相似（两组免疫抑制均为100%，血浆置换分别为75%对86%），但1年肾脏生存率无差异（25%对14%）。
与文献数据一致，约三分之一的抗肾小球基底膜抗体阳性患者可检测到抗中性粒细胞胞浆抗体，两种抗体共存可能会产生临床后果。
