Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi.
Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi.
Chest. 2015 Dec;148(6):1438-1446. doi: 10.1378/chest.14-3174.
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.
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.
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. After 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).
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.
肺为主型结缔组织病（LD-CTD）是一种间质性肺炎的疾病概念，但尚未得到充分验证。本研究旨在阐明 LD-CTD 的临床、放射学和组织学特征。
我们回顾性分析了 44 例经血清学定义的 LD-CTD 患者的连续手术肺活检。如果患者具有特定的自身抗体但不符合结缔组织病标准，则将其确定为 LD-CTD 患者。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（IIPs）分类评估主要组织学模式。还评估了 LD-CTD 的特征性组织学特征（例如，明显的浆细胞浸润、伴有生发中心的淋巴聚集）、高分辨率 CT（HRCT）扫描模式和预后。
主要组织学模式为 25 例患者的寻常型间质性肺炎（UIP）和 13 例患者的非特异性间质性肺炎（NSIP）。15 例组织学 UIP（h-UIP）和 11 例组织学 NSIP（h-NSIP）患者观察到 2 种或更多 LD-CTD 的特征性组织学特征。15 例 h-UIP 患者（60%）在 HRCT 扫描上表现出不一致的 UIP 模式。经过多学科讨论（MDD），18 例 h-UIP 患者被标记为无法分类的 IIP。h-NSIP 患者的预测 FVC 年变化率显著改善（P=.002），且生存状况优于 h-UIP 患者（P=.031）。相比之下，生存率与 HRCT 扫描模式无关（P=.79）。
LD-CTD 的主要组织学模式是 UIP 继之以 NSIP。三分之二的患者具有 LD-CTD 的特征性组织学特征。大多数 h-UIP 患者根据 MDD 被认为是无法分类的 IIP。h-UIP 患者的生存状况比 h-NSIP 患者差。
