Lung-Dominant Connective Tissue Disease Clinical, Radiologic, and Histologic Features
Lung-Dominant Connective Tissue Disease Clinical, Radiologic, and Histologic Features
复制标题
DOI：
10.1378/chest.14-3174
复制
发表时间：
2015-12-01
期刊：
影响因子：
9.6
通讯作者：
Hasegawa, Yoshinori
中科院分区：
文献类型：
--
作者：
Omote, Norihito;Taniguchi, Hiroyuki;Hasegawa, Yoshinori
背景技术背景：肺显性结缔组织病（LD-CTD）是间质性肺炎的一种疾病概念;然而，它尚未得到有力的验证。本研究旨在阐明LD-CTD.METHODS的临床、影像学和组织学特征：我们回顾性分析了44例血清学确诊的LD-CTD患者，他们接受了手术肺活检。如果患者有特异性自身抗体但不符合结缔组织病的标准，则将其确定为患有LD-CTD。我们进行了多学科诊断，并根据2013年现行特发性间质性肺炎（IIP）分类评价了主要组织学模式。LD-CTD的特征性组织学特征（例如，突出的浆细胞浸润，淋巴聚集与生发中心），高分辨率CT（HRCT）扫描模式，和预后也进行了assessed.Results：主要的组织学模式是普通间质性肺炎（UIP）25例和非特异性间质性肺炎（NSIP）13例。在15例组织学UIP（h-UIP）和11例组织学NSIP（h-NSIP）患者中观察到LD-CTD的两个或多个特征性组织学特征。15例h-UIP患者（60%）在HRCT扫描上显示不一致的UIP模式。经过多学科讨论（MDD），18例h-UIP患者被标记为具有不可分类的IIP。h-NSIP患者FVC预测值的年变化百分比显著改善（P = 0.002），其生存率也优于h-UIP患者（P = 0.031）。相反，生存率与HRCT扫描模式无关（P = .79）。结论：LD-CTD的主要组织学模式为UIP，其次为NSIP。三分之二的患者具有LD-CTD的特征性组织学特征。根据MDD，认为大多数h-UIP患者具有不可分类的IIP。h-UIP患者的生存率低于h-NSIP患者。
BACKGROUND: Lung-dominant connective tissue disease (LD-CTD) is a disease concept for interstitial pneumonia; however, it has not been robustly validated. This study was conducted to elucidate the clinical, radiologic, and histologic features of LD-CTD.METHODS: We retrospectively reviewed 44 consecutive patients with serologically defined LD-CTD who underwent surgical lung biopsy. Patients were identified as having LD-CTD if they had specific autoantibodies but did not meet the criteria for connective tissue disease. We conducted a multidisciplinary diagnosis and evaluated major histologic patterns according to the current idiopathic interstitial pneumonias (IIPs) classification of 2013. Characteristic histologic features for LD-CTD (eg, prominent plasmacytic infiltration, lymphoid aggregates with germinal centers), high-resolution CT (HRCT) scan patterns, and prognosis were also assessed.RESULTS: The major histologic patterns were usual interstitial pneumonia (UIP) in 25 patients and nonspecific interstitial pneumonia (NSIP) in 13 patients. Two or more characteristic histologic features for LD-CTD were observed in 15 patients with histologic UIP (h-UIP) and 11 patients with histologic NSIP (h-NSIP). Fifteen patients with h-UIP (60%) showed an inconsistent UIP pattern on HRCT scan. Aft er multidisciplinary discussion (MDD), 18 patients with h-UIP were labeled as having unclassifiable IIP. The annual change in percent predicted FVC improved significantly in patients with h-NSIP (P = .002), who also had better survival than those with h-UIP (P = .031). In contrast, survival was not associated with HRCT scan pattern (P = .79).CONCLUSIONS: The major histologic patterns in LD-CTD were UIP followed by NSIP. Two-thirds of patients had characteristic histologic features for LD-CTD. A majority of patients with h-UIP were considered to have unclassifiable IIP based on MDD. Patients with h-UIP had worse survival than those with h-NSIP.