Fulmer J D, Bienkowski R S, Cowan M J, Breul S D, Bradley K M, Ferrans V J, Roberts W C, Crystal R G
Am Rev Respir Dis. 1980 Aug;122(2):289-301. doi: 10.1164/arrd.1980.122.2.289.
To define the biochemical correlates of the apparent morphologic increase in lung interstitial collagen in idiopathic pulmonary fibrosis (IPF), collagen content was quantitated, and the morphologic degree of fibrosis was assessed in 9 patients with IPF and 6 control subjects. There were no significant differences in the collagen content among patients with IPF compared with that of control subjects. In addition, there was no correlation between collagen content and the morphologic assessment of the degree of fibrosis. Analysis of the collagen content from multiple sites made from base to apex of postmortem material from 3 patients with IPF and 2 control subjects demonstrated a wide, but similar, variation in collagen content in both groups; no definite pattern of anatomic distribution was found in either the patients with IPF or the control subjects. Furthermore, the rates of collagen and noncollagen protein synthesis in explants of lung of patients with IPF demonstrated no significant differences compared with those of the control subjects. The results of this study are consistent with the concept that IPF is a disease of an alteration in quality, form, and location of collagen rather than simply a disease of increased interstitial collagen.
为明确特发性肺纤维化(IPF)中肺间质胶原明显的形态学增加的生化关联,对9例IPF患者和6例对照者的胶原含量进行了定量,并评估了纤维化的形态学程度。与对照者相比,IPF患者之间的胶原含量无显著差异。此外,胶原含量与纤维化程度的形态学评估之间无相关性。对3例IPF患者和2例对照者尸检材料从肺底部到肺尖多个部位的胶原含量分析表明,两组的胶原含量均有广泛但相似的变化;在IPF患者或对照者中均未发现明确的解剖分布模式。此外,IPF患者肺组织外植体中胶原和非胶原蛋白质的合成速率与对照者相比无显著差异。本研究结果与以下概念一致,即IPF是一种胶原质量、形式和位置改变的疾病,而不仅仅是间质胶原增加的疾病。