Localization of the immune response in sarcoidosis.
Localization of the immune response in sarcoidosis.
复制标题
结节病免疫反应的定位。
DOI：
10.1164/arrd.1979.120.1.49
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发表时间：
2015
期刊：
影响因子：
--
通讯作者：
R. Crystal
中科院分区：
文献类型：
--
作者：
G. Hunninghake;J. Fulmer;R. Young;J. Gadek;R. Crystal
肺结节病是一种间质性疾病，其特征是肺实质内出现肉芽肿、对各种皮肤测试无反应以及循环 T 淋巴细胞数量减少。为了评估活动性肺结节病患者疾病部位存在的效应细胞群，通过支气管肺泡灌洗从肺部分离炎症和免疫效应细胞，并与来自相同患者外周血的可比较细胞群以及正常受试者和特发性肺纤维化患者的类似细胞群进行比较。结节病患者尽管外周血 T 淋巴细胞明显减少，但肺部 T 淋巴细胞百分比显着增加。此外，许多T淋巴细胞表现出与淋巴细胞活化相关的表面标记特征，并且它们自发分泌白细胞抑制因子。与结节病患者相比，正常受试者和特发性肺纤维化患者的肺和血液中 T 淋巴细胞百分比相似，并且没有 T 淋巴细胞激活的证据。对 8 名结节病患者中的 7 名未受累骨髓中的淋巴细胞进行分析，结果显示 T 淋巴细胞的比例与正常受试者和特发性肺纤维化患者的骨髓中的比例相似。相比之下，一名结节病患者的骨髓中存在大量 T 淋巴细胞，但仅限于骨髓中有肉芽肿的区域。这些研究表明：(1) 肺结节病的肺泡炎的特征是大量活化的 T 淋巴细胞，(2) 结节病中的免疫反应存在解剖学定位，对未受累组织（例如外周血）的分析可能无法反映肉芽肿形成部位的局部免疫反应。
Pulmonary sarcoidosis is an interstitial disease characterized by granulomas within the lung parenchyma, anergy to a variety of skin tests, and decreased numbers of circulating T-lymphocytes. To evaluate the effector cell populations present at sites of disease in patients with active pulmonary sarcoidosis, inflammatory and immune effector cells were isolated from lung via bronchoalveolar lavage and compared to comparable cell populations from the peripheral blood of the same patients and similar cell populations of normal subjects and patients with idiopathic pulmonary fibrosis. Patients with sarcoidosis had a marked increase in the percentage of T-lymphocytes in the lung despite a significant peripheral blood T-lymphocytopenia. In addition, many of these T-lymphocytes demonstrated surface marker characteristics associated with lymphocyte activation, and they spontaneously secreted leukocyte inhibitory factor. In contrast to patients with sarcoidosis, normal subjects and patients with idiopathic pulmonary fibrosis had similar percentages of T-lymphocytes in lung and blood, and there was no evidence for T-lymphocyte activation. Analysis of lymphocytes in uninvolved marrow from 7 of 8 patients with sarcoidosis revealed proportions of T-lymphocytes similar to those in the marrows of normal subjects and patients with idiopathic pulmonary fibrosis. In comparison, one patient with sarcoidosis had large numbers of T-lymphocytes in bone marrow, but only in areas where there were granulomas in the marrow. These studies suggest that: (1) the alveolitis of pulmonary sarcoidosis is characterized by large numbers of activated T-lymphocytes, and (2) there is an anatomic localization of the immune response in sarcoidosis in that analysis of uninvolved tissues such as peripheral blood may not reflect local immune responses at sites of granuloma formation.