Baltaro R J, Hoffman G S, Sechler J M, Suffredini A F, Shelhamer J H, Fauci A S, Fleisher T A
Department of Clinical Pathology, Clinical Center, National Institutes of Health, Bethesda, Maryland.
Am Rev Respir Dis. 1991 Feb;143(2):275-8. doi: 10.1164/ajrccm/143.2.275.
Wegener's granulomatosis (WG) is a small-vessel vasculitis of unknown etiology that usually involves the upper and lower respiratory tract and the kidneys. Recently, an association has been made between the presence of serum antineutrophil cytoplasmic antibodies (ANCA) and WG. Because WG frequently involves the lung, we sought to evaluate bronchoalveolar lavage (BAL) fluids obtained from 14 patients with WG for the presence of ANCA. Immunoglobulin (Ig) G ANCA was found in the BAL with the same staining patterns as observed in the serum. Patients with active disease had the highest serum and BAL IgG ANCA titers. IgA or IgM ANCA was not detected in the serum or BAL of these patients. Protein analysis of BAL fluid revealed that patients with active, untreated WG had approximately a fourfold elevation in total protein (41.3 versus 10.5 mg/dl), with a disproportionately greater increase in the ratio of IgG to albumin (BAL IgG index = 1.49, normal = 0.74; p = 0.027). The increase of the IgG index in patients with active WG suggests that local production of IgG ANCA occurs in the lungs.
韦格纳肉芽肿病（WG）是一种病因不明的小血管炎，通常累及上、下呼吸道及肾脏。最近发现血清抗中性粒细胞胞浆抗体（ANCA）与WG有关。由于WG常累及肺部，我们试图评估14例WG患者支气管肺泡灌洗（BAL）液中ANCA的存在情况。在BAL液中发现了免疫球蛋白（Ig）G ANCA，其染色模式与血清中观察到的相同。患有活动性疾病的患者血清和BAL液中IgG ANCA滴度最高。在这些患者的血清或BAL液中未检测到IgA或IgM ANCA。BAL液的蛋白质分析显示，未经治疗的活动性WG患者的总蛋白大约升高了四倍（41.3对10.5mg/dl），IgG与白蛋白的比例升高幅度更大（BAL IgG指数=1.49，正常=0.74；p=0.027）。活动性WG患者IgG指数的升高表明肺部存在IgG ANCA的局部产生。
