Cicchitto Gaetano, Sanguinetti Claudio M
RespiratoryPhysiopathology Unit, ASL SA, Cava de' Tirreni, SA, Italy.
Multidiscip Respir Med. 2013 Aug 9;8(1):53. doi: 10.1186/2049-6958-8-53.
Idiopathic pulmonary fibrosis (IPF), a chronic fibrosing lung disease of a progressive nature and unknown etiology, has the largest epidemiological impact and the worst prognosis among the idiopathic interstitial pneumonias (IIP). Despite the progress in pathogenetic knowledge, many aspects are still dubious, in particular the biomolecular mechanisms activated in the early stages of the disease. Early diagnosis is desirable not only to better define aspects of the natural history of the disease, but also to customize treatment protocols. An early diagnosis of IPF should necessarily be based on the ability to highlight a number of features drawn not only from a careful composition of specific anamnestic data with clinical, functional and radiological parameters, but also from biological markers that, in a proper context, can provide guidance and confirm a clinical-anamnestic suspicion. The identification of specific biomarkers for IPF is a modern and attractive look for the potential clinical implications in terms of diagnosis, prediction of disease progression and prognosis. Biomolecular investigations on IPF were performed selectively on tissue samples, bronchoalveolar lavage (BAL), or blood: nowadays the "multi-omic" approach may allow studying individual constitutional profiles resorting to a series of biomolecular disciplines, the so-called "omics", which focuses on responses of the entire genomic complex, in line with the current trend to quantitatively analyze the interactions of all components of a biological system. Such refined investigations are an essential base for research now, but they might become a routine in the near future, allowing a more precise classification of patients suffering from a disease of unclear taxonomy.
特发性肺纤维化(IPF)是一种具有进行性且病因不明的慢性纤维化肺部疾病,在特发性间质性肺炎(IIP)中具有最大的流行病学影响和最差的预后。尽管在发病机制方面的知识有所进展,但许多方面仍存在疑问,尤其是疾病早期激活的生物分子机制。早期诊断不仅有助于更好地界定疾病自然史的各个方面,还能定制治疗方案。IPF的早期诊断必然要基于不仅能从特定病史数据与临床、功能和放射学参数的仔细组合中,还能从生物标志物中突出一些特征的能力,这些生物标志物在适当的背景下可以提供指导并证实临床病史怀疑。识别IPF的特定生物标志物是一个具有现代吸引力的研究方向,有望在诊断、疾病进展预测和预后方面产生潜在的临床意义。对IPF的生物分子研究曾选择性地在组织样本、支气管肺泡灌洗(BAL)或血液上进行:如今,“多组学”方法可能允许借助一系列生物分子学科,即所谓的“组学”,来研究个体体质特征,这些学科关注整个基因组复合体的反应,这与当前定量分析生物系统所有组成部分相互作用的趋势一致。这种精细的研究现在是研究的重要基础,但在不久的将来可能会成为常规操作,从而对患有分类不明疾病的患者进行更精确的分类。