Departments of Pathology, Rheumatology, and Pulmonary Medicine, UPMC Presbyterian, Pittsburgh, PA 15213-2582.
Departments of Pathology, Rheumatology, and Pulmonary Medicine, UPMC Presbyterian, Pittsburgh, PA 15213-2582.
Hum Pathol. 2014 Jun;45(6):1199-204. doi: 10.1016/j.humpath.2014.01.018. Epub 2014 Feb 6.
The pulmonary histopathologic manifestations of the antisynthetase syndromes is poorly understood and reported. Eight cases of interstitial lung disease related to the presence of antitheonyl (PL7) transfer RNA synthetase autoantibodies along with a review of biopsy changes reported in the English literature are described. Most patients presented with dyspnea and cough, with myositis, skin changes including "mechanic's hands," and Raynaud phenomenon. Biopsy patterns of injury included usual interstitial pneumonia (n = 4), organizing pneumonia (n = 2), nonspecific interstitial pneumonia, and lymphoid interstitial pneumonia (n = 1 each), which, in a minority of instances, contrasted with predictions by thoracic radiologists based on high-resolution computed tomographic scans. This study emphasizes the integrated clinical, radiologic, and pathologic approach to interstitial lung disease, especially in connective tissue disorders, and points out the occurrence of usual interstitial pneumonia and organizing pneumonia in this patient group where nonspecific interstitial pneumonia has been the dominant pattern of clinical interest.
抗合成酶综合征的肺部组织病理学表现尚不清楚，相关报道也较少。本文报道了 8 例与抗苏氨酰（PL7）转移 RNA 合成酶自身抗体相关的间质性肺疾病，并对英文文献中报道的活检改变进行了复习。大多数患者以呼吸困难和咳嗽为首发症状，同时伴有肌炎、皮肤改变（包括“技工手”）和雷诺现象。损伤的活检模式包括普通间质性肺炎（n = 4）、机化性肺炎（n = 2）、非特异性间质性肺炎和淋巴间质肺炎（各 1 例），在少数情况下，与基于高分辨率计算机断层扫描的胸部放射科医生的预测结果不一致。本研究强调了间质性肺疾病的综合临床、影像学和病理学方法，特别是在结缔组织疾病中，指出在该患者群体中，常表现为普通间质性肺炎和机化性肺炎，而非特异性间质性肺炎是主要的临床关注模式。
