Ashitani Jun-Ichi, Matsumoto Nobuhiro, Nakazato Masamitsu
Third Department of Internal Medicine, Miyazaki University School of Medicine, Miyazaki, Japan.
Respirology. 2007 May;12(3):339-45. doi: 10.1111/j.1440-1843.2007.01061.x.
Pathogens such as mycobacteria and proprionibacterium have been implicated in the pathogenesis of sarcoidosis. alpha-Defensins (DF) are naturally occurring antimicrobial peptides. The aim of the present study was to assess whether DF are increased in the airway and/or systemic circulation in patients with pulmonary sarcoidosis and whether DF levels are related to sarcoidosis disease activity.
DF levels in plasma and BAL fluid (BALF) were measured in 30 patients with pulmonary sarcoidosis and in 10 controls. Plasma and BALF DF levels were compared according to disease activity. Molecular forms were analysed using reverse-phase (RP) HPLC to confirm plasma and BALF DF kinetics in pulmonary sarcoidosis.
DF concentrations in plasma and BALF were higher in patients with pulmonary sarcoidosis than in the controls. Plasma DF levels correlated with lysozyme but not with angiotensin-converting enzyme. These levels were high in patients in whom more organs were involved, whereas BALF DF levels were higher in patients at stage II or III than with those at sarcoidosis I. RP-HPLC showed high plasma levels of pro-defensins, DF precursors from the bone marrow, in sarcoidosis, although DF in peripheral neutrophils and BALF were the mature type.
High plasma DF concentrations resulted from bone marrow stimulation and seemed to be associated with disease activity, whereas BALF DF were released from accumulated neutrophils in the lungs and may contribute to parenchymal involvement in sarcoidosis.
诸如分枝杆菌和丙酸杆菌等病原体已被认为与结节病的发病机制有关。α-防御素（DF）是天然存在的抗菌肽。本研究的目的是评估肺结节病患者气道和/或体循环中DF是否升高，以及DF水平是否与结节病疾病活动相关。
对30例肺结节病患者和10例对照者的血浆和支气管肺泡灌洗液（BALF）中的DF水平进行检测。根据疾病活动情况比较血浆和BALF中的DF水平。使用反相（RP）高效液相色谱法分析分子形式，以确认肺结节病中血浆和BALF中DF的动力学。
肺结节病患者血浆和BALF中的DF浓度高于对照组。血浆DF水平与溶菌酶相关，但与血管紧张素转换酶无关。在累及更多器官的患者中这些水平较高，而II期或III期结节病患者的BALF中DF水平高于I期患者。RP-高效液相色谱法显示，结节病患者血浆中前防御素（来自骨髓的DF前体）水平较高，尽管外周血中性粒细胞和BALF中的DF为成熟型。
血浆中高DF浓度是由骨髓刺激导致的，似乎与疾病活动相关，而BALF中的DF是由肺中积聚的中性粒细胞释放的，可能参与结节病的实质受累。
