Interstitial lung disease in connective tissue diseases: evolving concepts of pathogenesis and management.

Interstitial lung disease in connective tissue diseases: evolving concepts of pathogenesis and management.
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DOI:
10.1186/ar3097
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发表时间:
2010
影响因子:
4.9
通讯作者:
Varga J
Varga J
中科院分区:
医学2区
文献类型:
--
作者:
Castelino FV;Varga J

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间质性肺疾病(ILD)是一种与多种结缔组织疾病相关的具有挑战性的临床实体,是发病率和死亡率的重要原因。结缔组织病相关间质性肺疾病(CTD-ILD)的有效治疗方法仍然缺乏。现在正在建立致力于CTD-ILD患者早期诊断和改善管理的多学科诊所。在认识和识别效应细胞、促炎和促纤维化介质以及参与CTD-ILD发病机制的途径方面取得了很大进展。血清生物标记物可能作为肺纤维化的危险因素和疾病进展的衡量标准提供新的见解。尽管最近取得了这些进展,但CTD-ILD患者的治疗仍然不够理想。因此,迫切需要进一步的研究,以更好地了解这些情况,并开发有效的治疗干预措施。
Interstitial lung disease (ILD) is a challenging clinical entity associated with multiple connective tissue diseases, and is a significant cause of morbidity and mortality. Effective therapies for connective tissue disease-associated interstitial lung disease (CTD-ILD) are still lacking. Multidisciplinary clinics dedicated to the early diagnosis and improved management of patients with CTD-ILD are now being established. There is rapid progress in understanding and identifying the effector cells, the proinflammatory and profibrotic mediators, and the pathways involved in the pathogenesis of CTD-ILD. Serum biomarkers may provide new insights as risk factors for pulmonary fibrosis and as measures of disease progression. Despite these recent advances, the management of patients with CTD-ILD remains suboptimal. Further studies are therefore urgently needed to better understand these conditions, and to develop effective therapeutic interventions.
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