Chowaniec Małgorzata, Skoczyńska Marta, Sokolik Renata, Wiland Piotr
Department of Rheumatology and Internal Diseases, University Hospital in Wrocław, Poland.
Department of Hygene, Wrocław Medical University, Poland.
Reumatologia. 2018;56(4):249-254. doi: 10.5114/reum.2018.77977. Epub 2018 Aug 31.
Interstitial lung disease (ILD) is a group of lung diseases characterized by thickening of the interstitium surrounding pulmonary alveolar walls. It is related to specific radiographic features in lung imaging and/or the presence of restrictive disorders in pulmonary function tests (PFTs). ILD is one of the leading causes of death in systemic sclerosis patients. Major risk factors of ILD associated with SSc (SSc-ILD) include male sex, diffuse type of cutaneous SSc and presence of anti-Scl-70 antibodies. SSc-ILD is challenging to diagnose at an early stage as the symptoms are non-specific. The greatest risk of its development is during the 4-5 years after the initial diagnosis of systemic sclerosis. Clinical vigilance at the time, including regular pulmonary function tests and/or high-resolution com-puted tomography (HRCT), is needed. The aim of this paper is to summarize the current knowledge on early diagnostic methods and progression risk factors for SSc-ILD.
间质性肺疾病（ILD）是一组以肺泡壁周围间质增厚为特征的肺部疾病。它与肺部影像学的特定表现和/或肺功能测试（PFT）中限制性疾病的存在有关。ILD是系统性硬化症患者的主要死亡原因之一。与系统性硬化症相关的间质性肺疾病（SSc-ILD）的主要危险因素包括男性、弥漫性皮肤型系统性硬化症以及抗Scl-70抗体的存在。由于症状不具有特异性，SSc-ILD在早期诊断具有挑战性。其发展的最大风险发生在系统性硬化症初步诊断后的4至5年。此时需要临床警惕，包括定期进行肺功能测试和/或高分辨率计算机断层扫描（HRCT）。本文的目的是总结目前关于SSc-ILD早期诊断方法和进展危险因素的知识。
