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[结缔组织病相关间质性肺疾病:基于模式的诊断方法]

[Connective tissue disease-associated interstitial lung diseases : A pattern-based approach to diagnosis].

作者信息

Mura Rebecca, Kifjak Daria, Kastrati Kastriot, Bogveradze Nino, Beer Lucian, Prosch Helmut

机构信息

Univ. Klinik für Radiologie und Nuklearmedizin, Medizinische Universität Wien, Währinger Gürtel 18-20, 1090, Wien, Österreich.

Abteilung für Rheumatologie, Universitätsklinik für Innere Medizin III, Medizinische Universität Wien, 1090, Wien, Österreich.

出版信息

Radiologie (Heidelb). 2025 Aug 22. doi: 10.1007/s00117-025-01492-4.

Abstract

Connective tissue diseases (CTD) encompass a heterogeneous group of systemic immune-mediated disorders affecting connective tissue throughout the body. Pulmonary involvement is a common and clinically significant manifestation of CTD, with interstitial lung disease (ILD) representing a major contributor to both morbidity and mortality. Therefore, early detection of CTD-ILD is critical, and a multidisciplinary approach is paramount for both diagnosis and patient management. In this context, high-resolution computed tomography (HRCT) plays a pivotal role not only in recognizing characteristic ILD patterns but also in monitoring disease progression and guiding further management. Although the most frequent imaging pattern in CTD-ILD is nonspecific interstitial pneumonia (NSIP), the spectrum of pulmonary manifestations is complex and heterogeneous. Other patterns such as usual interstitial pneumonia (UIP), organizing pneumonia (OP), lymphocytic interstitial pneumonia (LIP), and diffuse alveolar damage (DAD) can also occur, showing distinct associations with specific CTD. Moreover, overlap and transition between patterns are not uncommon, further complicating the diagnostic process. This review aims to provide a comprehensive overview of the most relevant pulmonary manifestations of CTD, particularly ILD, with a focus on HRCT-based pattern recognition in order to enhance radiologist's familiarity with their spectrum of imaging appearances.

摘要

结缔组织病(CTD)是一组异质性的全身性免疫介导疾病,可累及全身的结缔组织。肺部受累是CTD常见且具有临床意义的表现,其中间质性肺疾病(ILD)是发病和死亡的主要原因。因此,早期发现CTD-ILD至关重要,多学科方法对于诊断和患者管理至关重要。在这种情况下,高分辨率计算机断层扫描(HRCT)不仅在识别特征性ILD模式方面发挥关键作用,而且在监测疾病进展和指导进一步管理方面也发挥关键作用。虽然CTD-ILD中最常见的影像学模式是非特异性间质性肺炎(NSIP),但其肺部表现谱复杂且异质性。其他模式,如寻常型间质性肺炎(UIP)、机化性肺炎(OP)、淋巴细胞间质性肺炎(LIP)和弥漫性肺泡损伤(DAD)也可能出现,与特定的CTD有明显关联。此外,模式之间的重叠和转变并不罕见,这进一步使诊断过程复杂化。本综述旨在全面概述CTD最相关的肺部表现,特别是ILD,重点是基于HRCT的模式识别,以提高放射科医生对其影像学表现谱的熟悉程度。

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