基因表达模式预测特发性肺纤维化患者的快速下降

特发性肺纤维化（IPF）是一种慢性肺病，其特征通常是呼吸急促或咳嗽的缓慢而进展。大多数患者诊断后大约五年。然而，根据今天在在线期刊PLOS中发表的一项新的研究，特定遗传型概况的患者的子集在没有肺移植的情况下完全肺部衰竭和死亡具有更快的进展。

基于诊所的观察结果，一些IPF患者展示更快的疾病课程，匹兹堡大学医学院的间质肺病患者的研究人员，与墨西哥肺科学家和加利福尼亚州合作，使用DNA微阵列分析测量26个快速进展的基因表达模式和88个缓慢进展。它们在组之间鉴定了437个差异表达的基因。具体而言，快速进展者的肺主要是患者，患者吸烟，过表达基因参与组织和器官（形态发生），氧化应激，细胞迁移和来自成纤维细胞和平滑肌细胞的基因。

According to Naftali Kaminski, M.D., director of the Simmons Center and director of the Lung Translational Genomics Center, division of pulmonary, allergy and critical care medicine, University of Pittsburgh School of Medicine, these findings offer strong evidence that rapid progressors represent a distinct clinical phenotype compared with the usual slower progressing patients.

“我们现在只开始真正了解IPF并表征它，”Kaminski博士说。“因此，在积极参与IPF研究的中心中可以看到疾病的患者至关重要，因此我们可以帮助他们更好地决定一个行动方案。”

These findings also highlight the variability in the progression and outcome of IPF and may explain, in part, the difficulty in obtaining significant and reproducible results in studies of therapeutic interventions in patients with IPF, noted first author Moisés Selman, M.D., director of research at the National Institute of Respiratory Research in Mexico City. "This study suggests that physicians should pay more attention to the time of onset of symptoms in their patients," Dr. Selman said.

"Although preliminary, these results may allow investigators to identify biomarkers of disease progression and, more importantly, better evaluate the effectiveness of potential therapies," added Talmadge E. King Jr., M.D., chief of medicine at San Francisco General Hospital and an internationally renowned expert in research and management of pulmonary fibrosis.

资料来源：匹兹堡大学健康科学学校

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