The alveolitis of pulmonary sarcoidosis. Evaluation of natural history and alveolitis-dependent changes in lung function.
The alveolitis of pulmonary sarcoidosis. Evaluation of natural history and alveolitis-dependent changes in lung function.
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肺结节病的肺泡炎。
DOI：
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发表时间：
1983
期刊：
影响因子：
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通讯作者：
R. Crystal
中科院分区：
文献类型：
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作者：
B. Keogh;G. Hunninghake;B. Line;R. Crystal
目前关于肺结节病发病机制的概念表明，单个核细胞肺泡炎，由活化的t淋巴细胞和活化的肺泡巨噬细胞组成，先于并调节该疾病特征的肉芽肿和纤维化的形成。为了评估这种肺泡炎的自然病史并确定其与随后肺功能变化的关系，我们研究了19例未经治疗且无肺外表现的肺结节病患者，每隔6个月（10.1 +/- 1.6个月）进行支气管肺泡灌洗、67Ga扫描和肺功能检查，分别评估肺t细胞、肺泡巨噬细胞和肺功能。在这组结节病患者中，低强度肺泡炎（肺t细胞小于或等于所有肺效应细胞的28%和/或67Ga扫描阴性）比高强度肺泡炎（肺t细胞大于28%和67Ga扫描阳性，占所有观察值的20%）更为常见（占所有观察值的80%）。然而，肺泡炎可能不稳定；75%的高强度肺泡炎发作自发地恢复到低强度，而12%的低强度肺泡炎发作自发地恢复到高强度。此外，常规的临床、x线摄影或生理学研究不能预测肺泡炎的状态；低强度组和高强度组用常规标准无法区分（p均大于0.3）。有趣的是，在19例患者的51次肺泡炎评估中，有24次（47%）肺泡炎“分裂”，即67Ga扫描阳性，t细胞低（39%）或67Ga阴性，t细胞高（8%）。事实上，在研究期间，79%的患者67Ga扫描呈阳性和/或至少一次肺t细胞水平较高，这表明大多数未经治疗的结节病患者无肺外症状，其肺泡结构经常存在一些炎症过程。总的来说，无论何时发生高强度肺泡炎，在接下来的6个月里，87%的情况下至少有一项肺功能参数恶化。相比之下，低强度肺泡炎发作后只有8%的时间出现功能恶化。引人注目的是，虽然肺泡炎参数（灌洗和67Ga扫描）清楚地预测预后，但临床、x线和生理检查无法区分那些随后功能恶化的患者。这些观察结果将有助于了解肺结节病的自然史、对该病患者的分期以及做出合理的治疗决定。
Current concepts of the pathogenesis of pulmonary sarcoidosis suggest that a mononuclear cell alveolitis, comprised of activated T-lymphocytes and activated alveolar macrophages, precedes and modulates the formation of granuloma and fibrosis that characterize the disease. To evaluate the natural history of this alveolitis and determine the relationship it has to subsequent changes in lung function, 19 untreated patients with pulmonary sarcoidosis without extrapulmonary manifestations were studied at 6-month intervals over 10.1 +/- 1.6 months with bronchoalveolar lavage, 67Ga scanning, and pulmonary function tests to evaluate lung T-cells, lung alveolar macrophages, and lung function, respectively. In this group of patients with sarcoidosis, low intensity alveolitis (lung T-cells less than or equal to 28% of all lung effector cells and/or 67Ga scan negative) was much more common (80% of all observations) than high intensity alveolitis (lung T-cells greater than 28% and 67Ga scan positive, 20% of all observations). However, the alveolitis can be unstable; 75% of all episodes of high intensity alveolitis spontaneously reverted to low intensity, whereas 12% of all episodes of low intensity alveolitis spontaneously reverted to high intensity. Furthermore, conventional clinical, roentgenographic, or physiologic studies could not predict the alveolitis status; the low intensity and high intensity groups were indistinguishable by usual criteria (p greater than 0.3, all comparisons). Interestingly, of the 51 alveolitis evaluations in the 19 patients, there were 24 occurrences (47%) where the alveolitis was "split," i.e., 67Ga scans positive and T-cells low (39%) or 67Ga negative and T-cells high (8%). In fact, 79% of all patients had either a positive 67Ga scan and/or high lung T-cells at least once during the study period, suggesting that most untreated patients with sarcoidosis without extrapulmonary symptoms often have some inflammatory processes ongoing in their alveolar structures. Overall, whenever a high intensity alveolitis episode occurred, it was followed by deterioration over the next 6 months in at least one lung function parameter 87% of the time. In contrast, a low intensity alveolitis episode was followed by functional deterioration only 8% of the time. Strikingly, while the alveolitis parameters (lavage and 67Ga scanning) clearly predicted prognosis, clinical, roentgenographic, and physiologic tests could not distinguish those patients who would subsequently deteriorate functionally. These observations should prove useful in understanding the natural history of pulmonary sarcoidosis, in staging patients with this disease, and in making rational therapy decisions.