Kakugawa Tomoyuki, Mukae Hiroshi, Hayashi Tomayoshi, Ishii Hiroshi, Nakayama Seiko, Sakamoto Noriho, Yoshioka Sumako, Sugiyama Kanako, Mine Mariko, Mizuta Yohei, Kohno Shigeru
Second Department of Internal Medicine, Nagasaki University School of Medicine, Nagasaki, Japan.
Respir Res. 2005 Jun 14;6(1):57. doi: 10.1186/1465-9921-6-57.
Heat shock protein (HSP) 47, a collagen-specific molecular chaperone, is involved in the processing and/or secretion of procollagens, and its expression is increased in various fibrotic diseases. The aim of this study was to determine whether quantitative immunohistochemical evaluation of the expression levels of HSP47, type I procollagen and alpha-smooth muscle actin (SMA) allows the differentiation of idiopathic usual interstitial pneumonia (UIP) from UIP associated with collagen vascular disease (CVD) and idiopathic nonspecific interstitial pneumonia (NSIP).
We reviewed surgical lung biopsy specimens of 19 patients with idiopathic UIP, 7 with CVD-associated UIP and 16 with idiopathic NSIP and assigned a score for the expression of HSP47, type I procollagen and alpha-SMA in type II pneumocytes and/or lung fibroblasts (score 0 = no; 1 = weak; 2 = moderate; 3 = strong staining).
The expression level of HSP47 in type II pneumocytes of idiopathic UIP was significantly higher than in CVD-associated UIP and idiopathic NSIP. The expression of HSP47 in fibroblasts was significantly higher in idiopathic UIP and idiopathic NSIP than in CVD-associated UIP. The expression of type I procollagen in type II pneumocytes was significantly higher in idiopathic UIP than in idiopathic NSIP. The expression of type I procollagen in fibroblasts was not different in the three groups, while the expression of alpha-SMA in fibroblasts was significantly higher in idiopathic UIP than in idiopathic NSIP.
Our results suggest the existence of different fibrotic pathways among these groups involved in the expression of HSP47 and type I procollagen.
热休克蛋白（HSP）47是一种胶原蛋白特异性分子伴侣，参与前胶原蛋白的加工和/或分泌，其表达在各种纤维化疾病中增加。本研究的目的是确定对HSP47、I型前胶原蛋白和α平滑肌肌动蛋白（SMA）表达水平进行定量免疫组织化学评估是否能够区分特发性寻常型间质性肺炎（UIP）与胶原血管病（CVD）相关的UIP以及特发性非特异性间质性肺炎（NSIP）。
我们回顾了19例特发性UIP患者、7例CVD相关UIP患者和16例特发性NSIP患者的手术肺活检标本，并对II型肺细胞和/或肺成纤维细胞中HSP47、I型前胶原蛋白和α-SMA的表达进行评分（评分0 = 无；1 = 弱；2 = 中度；3 = 强染色）。
特发性UIP患者II型肺细胞中HSP47的表达水平显著高于CVD相关UIP和特发性NSIP。特发性UIP和特发性NSIP中，成纤维细胞中HSP47的表达显著高于CVD相关UIP。特发性UIP患者II型肺细胞中I型前胶原蛋白的表达显著高于特发性NSIP。三组中成纤维细胞中I型前胶原蛋白的表达无差异，而特发性UIP中成纤维细胞中α-SMA的表达显著高于特发性NSIP。
我们的结果表明，这些组之间存在不同的纤维化途径，涉及HSP47和I型前胶原蛋白的表达。
