Dépistage des manifestations pulmonaires des connectivites

Pierre-Antoine Juge , Raphaël Borie , Marie-Pierre Debray , Aurélie Cazes , Catherine Bancal , Bruno Crestani , Philippe Dieudé
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Abstract

Pulmonary manifestations often occur in connective tissue diseases (CTD) affecting the different component of the respiratory system (the lung parenchyma, the airways, the pleura, the vascular system…). The frequency and the severity of these manifestations vary according to the type of manifestation and the corresponding CTD. Systematic assessment by chest high resolution computed tomography (HRCT) lead to reconsider the frequency of some pulmonary manifestations with a long asymptomatic phase such as rheumatoid arthritis (RA) associated interstitial lung disease (ILD). Among the pulmonary manifestations associated with CTD, some will progress and may lead to an important disease burden and increase mortality. If a CTD related pulmonary manifestation is detected in a patient, it may impact the therapeutic management including the prescriptions of specific treatments or the restriction of pulmonary toxic drugs. The screening for such manifestations in asymptomatic patients, which enable early deification, is therefore indicated. For systemic sclerosis, dermatomyositis and Sjögren syndrome, lung involvement screening has been established in National Diagnostic and Care Protocols (PNDS) that will be developed in this review. For other CTD, there are still no guidelines. Beside a relatively high frequency of RA-ILD, no recommendations have been developed for the screening of RA-ILD. In this review, we will give the recent data that help to discuss and argue the necessity of a screening, notably in high-risk patients.

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肺结节表现的筛查
肺部表现常发生在影响呼吸系统不同组成部分(肺实质、气道、胸膜、血管系统等)的结缔组织病(CTD)中。这些表现的频率和严重程度根据表现的类型和相应的CTD而变化。胸部高分辨率计算机断层扫描(HRCT)系统评估导致重新考虑一些长期无症状期肺部表现的频率,如类风湿关节炎(RA)相关间质性肺疾病(ILD)。在与CTD相关的肺部表现中,一些会进展,并可能导致重要的疾病负担和增加死亡率。如果在患者中发现CTD相关的肺部表现,可能会影响治疗管理,包括特定治疗的处方或肺毒性药物的限制。因此,在无症状患者中筛查这些表现,可以早期神化。对于系统性硬化症,皮肌炎和Sjögren综合征,肺受累筛查已在国家诊断和护理方案(PNDS)中建立,将在本综述中制定。对于其他CTD,仍然没有指导方针。除了相对较高的RA-ILD发生率外,目前还没有关于RA-ILD筛查的建议。在这篇综述中,我们将给出有助于讨论和争论筛查必要性的最新数据,特别是在高危患者中。
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