Konopka Kristine E, Myers Jeffrey L
Department of Pathology, University of Michigan, Michigan Medicine, 2800 Plymouth Road, Building 35, Ann Arbor, MI 48109, USA.
Department of Pathology, University of Michigan, Ann Arbor, MI, USA.
Ther Adv Musculoskelet Dis. 2021 Jul 17;13:1759720X211032437. doi: 10.1177/1759720X211032437. eCollection 2021.
Interstitial lung disease is a relatively frequent manifestation of systemic sclerosis with approximately one-third of patients developing clinical restrictive lung disease. Fibrotic nonspecific interstitial pneumonia is the most common cause of diffuse parenchymal lung disease in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD), followed by usual interstitial pneumonia (UIP). Radiographic pleuroparenchymal fibroelastosis-like changes may accompany other forms of interstitial lung disease, most commonly UIP. In an appropriate clinical setting with supportive high-resolution computed tomography findings, lung biopsy is not needed to confirm the presence of interstitial lung disease and surgical lung biopsies are often reserved for atypical presentations. In this review, we discuss the histological findings that define the most common patterns of SSc-ILD and outline other findings sometimes encountered in lung biopsies obtained from systemic sclerosis patients, including pulmonary vascular changes, aspiration, chronic pleuritis, and diffuse alveolar damage.
间质性肺疾病是系统性硬化症相对常见的表现，约三分之一的患者会发展为临床限制性肺疾病。纤维化非特异性间质性肺炎是系统性硬化症相关间质性肺疾病（SSc-ILD）患者弥漫性实质性肺疾病的最常见原因，其次是寻常型间质性肺炎（UIP）。影像学上类似胸膜实质纤维弹性组织增生的改变可能伴随其他形式的间质性肺疾病，最常见的是UIP。在具有支持性高分辨率计算机断层扫描结果的适当临床环境中，无需进行肺活检来确诊间质性肺疾病，手术肺活检通常仅用于非典型表现。在本综述中，我们讨论了定义SSc-ILD最常见模式的组织学发现，并概述了从系统性硬化症患者的肺活检中有时会遇到的其他发现，包括肺血管改变、误吸、慢性胸膜炎和弥漫性肺泡损伤。
