Schoenfeld N, Schmolke B, Fritzsche A, Loddenkemper R
Lungenklinik Heckeshorn, Berlin, Germany.
Sarcoidosis Vasc Diffuse Lung Dis. 1996 Sep;13(2):135-8. doi: 10.1007/BF00389837.
Laminin is a noncollagenous component of the extracellular matrix in the alveolar wall and may play a role in the development of fibrotic lung disease. Serum levels of laminin fragment P1 as well as procollagen III peptide were determined in 28 patients with pulmonary sarcoidosis and 10 healthy controls using specific radioimmunoassays. The patients' results were compared with the clinical appearance, lung function values (vital capacity, total lung capacity, FEV1, transfer coefficient (KCO), and alveolar-arterial oxygen difference during exercise) and serum concentrations of angiotensin converting enzyme and soluble interleukin 2 receptor. Laminin levels in patients were significantly higher than in controls but always remained within normal limits. Although there was a tendency towards higher values in patients with active disease and with radiographic involvement, no significant correlation was found between laminin concentration and clinical, functional or biochemical data. In contrast, procollagen III N-terminal peptide concentrations were elevated in 19 of 28 patients and showed a weak but significant inverse correlation with parameters of restriction with significantly higher values in patients with active disease. In conclusion, serum levels of laminin fragment P1 are not elevated in pulmonary sarcoidosis and do not correlate with other parameters of the disease. Yet serum levels of procollagen III N-terminal peptide were associated with the degree of parenchymal involvement as expressed by functional disturbance and with active disease.
层粘连蛋白是肺泡壁细胞外基质的一种非胶原蛋白成分,可能在肺纤维化疾病的发展中起作用。使用特异性放射免疫分析法测定了28例肺结节病患者和10名健康对照者血清中层粘连蛋白片段P1以及Ⅲ型前胶原肽的水平。将患者的结果与临床表现、肺功能值(肺活量、肺总量、第1秒用力呼气容积、转运系数(KCO)以及运动期间肺泡-动脉氧分压差)以及血清中血管紧张素转换酶和可溶性白细胞介素2受体的浓度进行了比较。患者的层粘连蛋白水平显著高于对照组,但始终保持在正常范围内。尽管活动性疾病患者和有影像学表现的患者层粘连蛋白水平有升高趋势,但层粘连蛋白浓度与临床、功能或生化数据之间未发现显著相关性。相比之下,28例患者中有19例Ⅲ型前胶原N端肽浓度升高,且与限制性参数呈弱但显著的负相关,活动性疾病患者的值显著更高。总之,肺结节病患者血清中层粘连蛋白片段P1水平未升高,且与疾病的其他参数无关。然而,Ⅲ型前胶原N端肽的血清水平与以功能障碍表示的实质受累程度以及活动性疾病有关。