Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
Eur J Clin Invest. 2010 Feb;40(2):110-20. doi: 10.1111/j.1365-2362.2009.02224.x. Epub 2009 Nov 24.
Collagen V shows promise as an inducer of interstitial lung fibrosis in experimental systemic sclerosis (SSc).
Remodelling of the pulmonary interstitium was evaluated based on the clinical data and open lung biopsies from 15 patients with SSc. Normal lung tissues obtained from eight individuals who died of traumatic injuries were used as control group. Immunofluorescence, immunohistochemistry, morphometry, tri-dimensional reconstruction and a real-time polymerase chain reaction were used to evaluate the quantity, structure and molecular chains of collagen V. The impact of these markers was tested on clinical data.
The main difference in collagen V content between SSc patients and the control group was an increased, abnormal and distorted fibre deposition in the alveolar septa and the pre-acinar artery wall. The lungs from SSc patients presented [alpha1(V)] and [alpha2(V)] mRNA chain expression increased, but [alpha2(V)] was proportionally increased compared with the control group. High levels of collagen V were inversely associated with vital capacity (r = -0.72; P = 0.002), forced vital capacity (r = -0.76; P < 0.001), forced expiratory volume in 1-s (r = -0.89; P < 0.001) and diffusing capacity for carbon monoxide (r = -0.62; P = 0.04).
Abnormal collagen V fibres are overproduced in lungs from SSc patients and may play an important role in the pathogenesis of the disease as this molecule regulates tissue collagen assembly. The aberrant histoarchitecture observed in SSc can be related to the overexpression of the [alpha2(V)] gene of unknown origin.
胶原蛋白 V 有望成为实验性系统性硬皮病（SSc）间质肺纤维化的诱导剂。
根据 15 例 SSc 患者的临床资料和开胸肺活检，评估肺间质重塑。从因创伤性损伤而死亡的 8 个人的正常肺组织中获得正常肺组织作为对照组。使用免疫荧光、免疫组织化学、形态计量学、三维重建和实时聚合酶链反应来评估胶原蛋白 V 的数量、结构和分子链。测试这些标志物对临床数据的影响。
SSc 患者和对照组之间胶原蛋白 V 含量的主要差异是肺泡隔和前腺动脉壁中异常和扭曲的纤维沉积增加。SSc 患者的肺呈现[α1(V)]和[α2(V)]mRNA 链表达增加，但与对照组相比，[α2(V)]比例增加。胶原蛋白 V 水平与肺活量（r=-0.72；P=0.002）、用力肺活量（r=-0.76；P<0.001）、1 秒用力呼气量（r=-0.89；P<0.001）和一氧化碳弥散量呈负相关（r=-0.62；P=0.04）。
SSc 患者肺中异常胶原蛋白 V 纤维过度产生，可能在疾病发病机制中发挥重要作用，因为这种分子调节组织胶原蛋白组装。在 SSc 中观察到的异常组织形态学可能与未知来源的[α2(V)]基因的过表达有关。
