Hoffman G S, Sechler J M, Gallin J I, Shelhamer J H, Suffredini A, Ognibene F P, Baltaro R J, Fleisher T A, Leavitt R Y, Travis W D
Laboratory of Immunoregulation, National Institutes of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892.
Am Rev Respir Dis. 1991 Feb;143(2):401-7. doi: 10.1164/ajrccm/143.2.401.
A prospective analysis of bronchoalveolar lavage (BAL) in 13 patients with Wegener's granulomatosis (WG), 20 disease control subjects with idiopathic pulmonary fibrosis (IPF), and 24 normal control subjects was conducted to (1) evaluate the quality of the alveolar inflammatory response associated with active WG; (2) determine whether antineutrophil cytoplasmic antibody (ANCA) is present in alveolar fluid and produced in the lungs of patients with WG; and (3) determine whether inhaled particles or infectious agents may play an etiologic role in WG. BAL in untreated active WG had a marked increase in neutrophils (mean = 42% of total WBC count), and usually in eosinophils (mean = 4%) compared with that in normal control subjects (1.6% neutrophils, 0% eosinophils), and untreated WG in remission (5.9% neutrophils, 0% eosinophils). Disease control subjects with IPF, a process known to be associated with neutrophilic alveolitis, had an increased population of neutrophils (15.4%) and eosinophils (2.7%) in BAL. Leukocyte remnants, as well as intact leukocytes, could be identified within BAL macrophages in the patients with WG and IPF, and rarely in the normal control subjects. Normal subjects and control patients with IPF were all negative for ANCA in serum, whereas ANCA was found in serum and BAL in all patients with active WG who had generalized disease. Protein analysis of BAL revealed a disproportionate increase in the IgG to albumin ration compared with serum values (IgG index) in patients with active untreated disease. The increase in the IgG index suggests that IgG with ANCA reactivity is produced by pulmonary lymphoid tissue. An infectious agent in BAL was not identified by any of the techniques applied in this study.(ABSTRACT TRUNCATED AT 250 WORDS)
对13例韦格纳肉芽肿（WG）患者、20例特发性肺纤维化（IPF）疾病对照受试者和24例正常对照受试者进行支气管肺泡灌洗（BAL）的前瞻性分析，目的是：（1）评估与活动性WG相关的肺泡炎症反应质量；（2）确定抗中性粒细胞胞浆抗体（ANCA）是否存在于肺泡液中以及是否在WG患者的肺中产生；（3）确定吸入颗粒或感染因子是否可能在WG的病因中起作用。与正常对照受试者（中性粒细胞1.6%，嗜酸性粒细胞0%）和未治疗的缓解期WG（中性粒细胞5.9%，嗜酸性粒细胞0%）相比，未经治疗的活动性WG患者的BAL中中性粒细胞显著增加（平均占白细胞总数的42%），通常嗜酸性粒细胞也增加（平均4%）。已知与嗜中性肺泡炎相关的IPF疾病对照受试者的BAL中中性粒细胞（15.4%）和嗜酸性粒细胞（2.7%）数量增加。在WG和IPF患者的BAL巨噬细胞内可识别白细胞残余物以及完整白细胞，而在正常对照受试者中很少见。正常受试者和IPF对照患者血清中的ANCA均为阴性，而所有患有全身性疾病的活动性WG患者的血清和BAL中均发现有ANCA。BAL的蛋白质分析显示，与未治疗的活动性疾病患者的血清值相比，IgG与白蛋白比值（IgG指数）不成比例地增加。IgG指数的增加表明具有ANCA反应性的IgG是由肺淋巴组织产生的。本研究应用的任何技术均未在BAL中鉴定出感染因子。（摘要截短于250字）
