Cakmak Gulfidan, Selcuk Can Tuba, Gundogdu Sule, Akman Canan, Ikitimur Hande, Musellim Benan, Ongen Gul
Istanbul Haseki Education and Research Hospital,.
Sarcoidosis Vasc Diffuse Lung Dis. 2016 Dec 23;33(4):349-354.
Introduction and aim: Progressive systemic sclerosis (pSS) is a multisystemic connective tissue disease characterized by fibrosis of the skin and internal organs including lung. The mechanisms that leads to progressive lung fibrosis in scleroderma remain obscure. In this study, we aimed to investigate the correlation between HRCT findings and patients' clinical and functional status and the degree of alveolitis based on the BAL resultsMaterials and methods: 65 patients with pSS were evaluated. Thoracic HRCT, pulmonary function tests, and dyspnea measurements were applied, and BAL was performed. The parenchymal abnormalities identified on HRCT were coded, and scored according to Warrick et al.
Among parameters investigated, a correlation was found between the number of segments with subpleural cysts and the duration of disease. Also there was a correlation between the HRCT score and patient age whereas no correlation was detected between the duration of the disease, manifestation of the symptoms, and the x-ray findings. A correlation was found between the percentage of neutrophils detected in BAL and the extent of the honeycombing on HRCT.
This study showed a strong correlation between the extent of x-ray abnormalities and FVC, RV, and DLCO, as well as an increase in the percentage of BAL fluid neutrophils in patients with SSc-PI.
引言与目的：进行性系统性硬化症（pSS）是一种多系统结缔组织疾病，其特征为皮肤及包括肺在内的内脏器官纤维化。硬皮病中导致进行性肺纤维化的机制仍不清楚。在本研究中，我们旨在基于支气管肺泡灌洗（BAL）结果，研究高分辨率计算机断层扫描（HRCT）表现与患者临床及功能状态以及肺泡炎程度之间的相关性。
对65例pSS患者进行评估。进行胸部HRCT、肺功能测试及呼吸困难测量，并进行BAL。对HRCT上识别出的实质异常进行编码，并根据沃里克等人的方法进行评分。
在所研究的参数中，发现胸膜下囊肿节段数与疾病持续时间之间存在相关性。HRCT评分与患者年龄之间也存在相关性，而疾病持续时间、症状表现与X线表现之间未检测到相关性。在BAL中检测到的中性粒细胞百分比与HRCT上蜂窝状改变的程度之间存在相关性。
本研究表明，X线异常程度与用力肺活量（FVC）、残气量（RV）和一氧化碳弥散量（DLCO）之间存在密切相关性，以及系统性硬化症相关间质性肺炎（SSc-IP）患者BAL液中性粒细胞百分比增加。
