University of São Paulo School of Medicine, CEP 01246-903, São Paulo, Brazil.
J Bras Pneumol. 2009 Jun;35(6):529-40. doi: 10.1590/s1806-37132009000600006.
The aim of this study was to examine the parenchymal and extracellular matrix remodeling process in two histologic patterns-nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP)-in cases of idiopathic and sclerosis/systemic sclerosis (SSc)-associated interstitial pneumonia.
We examined 15 cases of idiopathic NSIP, 10 cases of idiopathic UIP, 5 cases of SSc-UIP and 9 cases of SSc-NSIP. In the lung parenchyma, epithelial cells, endothelial cells and myofibroblasts were evaluated by immunohistochemical staining, whereas histochemical staining was used in order to evaluate collagen/elastic fibers in the extracellular matrix.
The percentage of surfactant protein A-positive epithelial cells was significantly greater in idiopathic NSIP than in SSc-NSIP, as well as being greater in idiopathic UIP than in SSc-UIP. Idiopathic NSIP and idiopathic UIP presented significantly higher immunoexpression of alpha smooth muscle actin in myofibroblasts than did SSc-NSIP and SSc-UIP. The percentage of CD34 endothelial cells in the pulmonary microvasculature was significant lower in idiopathic UIP than in SSc-UIP. The density of collagen fibers was significantly greater in idiopathic NSIP and idiopathic UIP than in SSc-NSIP and UIP. In contrast, the elastic fiber density was significantly lower in idiopathic UIP than in SSc-UIP.
Increased collagen synthesis, destruction of elastic fibers, high myofibroblast proliferation and poor microvascularization might represent a remodeling process found in idiopathic interstitial pneumonia, whereas the reverse might represent a repair process in SSc-associated interstitial pneumonia.
本研究旨在研究特发性和硬皮病/系统性硬皮病（SSc）相关间质性肺炎中两种组织学模式——非特异性间质性肺炎（NSIP）和普通间质性肺炎（UIP）的实质和细胞外基质重塑过程。
我们检查了 15 例特发性 NSIP、10 例特发性 UIP、5 例 SSc-UIP 和 9 例 SSc-NSIP。在肺实质中，通过免疫组织化学染色评估上皮细胞、内皮细胞和成肌纤维细胞，而细胞外基质中的胶原/弹性纤维则通过组织化学染色进行评估。
特发性 NSIP 中表面活性蛋白 A 阳性上皮细胞的百分比明显高于 SSc-NSIP，特发性 UIP 中也明显高于 SSc-UIP。特发性 NSIP 和特发性 UIP 中成肌纤维细胞中α平滑肌肌动蛋白的免疫表达明显高于 SSc-NSIP 和 SSc-UIP。特发性 UIP 中肺微血管 CD34 内皮细胞的百分比明显低于 SSc-UIP。特发性 NSIP 和特发性 UIP 中胶原纤维的密度明显高于 SSc-NSIP 和 UIP。相比之下，特发性 UIP 中弹性纤维的密度明显低于 SSc-UIP。
胶原合成增加、弹性纤维破坏、成肌纤维细胞增殖增加和微血管化不良可能代表特发性间质性肺炎中的重塑过程，而相反的过程可能代表 SSc 相关间质性肺炎中的修复过程。
