Spiteri M A, Johnson M, Epstein O, Sherlock S, Clarke S W, Poulter L W
Department of Thoracic Medicine, Royal Free Hospital, London.
Gut. 1990 Feb;31(2):208-12. doi: 10.1136/gut.31.2.208.
To investigate the basis of subclinical alveolitis in patients with primary biliary cirrhosis, 10 primary biliary cirrhosis patients were studied by bronchoalveolar lavage. Both bronchoalveolar lavage lymphoid and non-lymphoid cell populations were analysed using immunocytological methods to determine their proportions and phenotypic features in an attempt to gain information as to possible immune mechanisms active in the lung of these patients. Six of the 10 patients in our study showed evidence of an alveolitis (raised lymphocyte count: 27.6 (4.3)% of total count) on lavage. The results were compared with control groups of normal volunteers and patients with active pulmonary sarcoidosis. The six primary biliary cirrhosis patients with lymphocytosis had a raised CD4/CD8 T-cell ratio (4.13:1), similar to the sarcoid patients (5.60:1). A proportion of these T-lymphocytes expressed markers of activation (HLA-DR+ 7.5 (2.1)%); CD25 + 2.3 (0.9)%; CD7 + 5.8 (1.5)%. This increased T-cell activation was also seen in the sarcoid groups (HLA-DR+ 10.0 (1.9)%; CD25 + 3.0 (1.1)%; CD7 + 5.0 (0.2)%). This was not seen in the primary biliary cirrhosis patients without lymphocytosis and the normal volunteers. Within the non-lymphoid cell population, an increase in dendritic (RFD1+) cells was seen in primary biliary cirrhosis patients with lymphocytosis (31.2 (1.9)%) and sarcoid patients (46.3 (5.1)%) in contrast with the normal and primary biliary cirrhosis group without lymphocytosis. The primary biliary cirrhosis patients without lymphocytes had a relatively greater proportion of mature phagocytes (RFD7+). We postulate that these observations suggest the emergence in the lung of a granuloma producing mechanism similar to that occurring in the liver. By comparison, the alveolitis found in primary biliary cirrhosis is consistent with that observed in interstitial granulomatous lung disorders such as sarcoidosis.
为了探究原发性胆汁性肝硬化患者亚临床肺泡炎的发病基础，对10例原发性胆汁性肝硬化患者进行了支气管肺泡灌洗研究。采用免疫细胞学法分析支气管肺泡灌洗中的淋巴细胞和非淋巴细胞群体，以确定它们的比例和表型特征，从而获取有关这些患者肺部可能存在的免疫机制的信息。我们研究中的10例患者中有6例在灌洗时显示出肺泡炎的迹象（淋巴细胞计数升高：占总数的27.6 (4.3)%）。将结果与正常志愿者和活动性肺结节病患者的对照组进行了比较。6例淋巴细胞增多的原发性胆汁性肝硬化患者的CD4/CD8 T细胞比值升高（4.13:1），与结节病患者相似（5.60:1）。这些T淋巴细胞中有一部分表达激活标志物（HLA-DR+ 7.5 (2.1)%；CD25 + 2.3 (0.9)%；CD7 + 5.8 (1.5)%）。在结节病组中也观察到这种T细胞激活增加（HLA-DR+ 10.0 (1.9)%；CD25 + 3.0 (1.1)%；CD7 + 5.0 (0.2)%）。在无淋巴细胞增多的原发性胆汁性肝硬化患者和正常志愿者中未观察到这种情况。在非淋巴细胞群体中，淋巴细胞增多的原发性胆汁性肝硬化患者（31.2 (1.9)%）和结节病患者（46.3 (5.1)%）中树突状（RFD1+）细胞增加，这与正常组和无淋巴细胞增多的原发性胆汁性肝硬化组形成对比。无淋巴细胞的原发性胆汁性肝硬化患者中成熟吞噬细胞（RFD7+）的比例相对较高。我们推测，这些观察结果表明肺部出现了一种类似于肝脏中发生的肉芽肿形成机制。相比之下，原发性胆汁性肝硬化中发现的肺泡炎与间质性肉芽肿性肺病（如结节病）中观察到的情况一致。
