Gaudin P B, Askin F B, Falk R J, Jennette J C
Department of Pathology, Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.
Am J Clin Pathol. 1995 Jul;104(1):7-16. doi: 10.1093/ajcp/104.1.7.
Anti-neutrophil cytoplasmic autoantibodies (ANCA) occur in a subset of patients with systemic small vessel vasculitis, including patients with Wegener's granulomatosis, microscopic polyangiitis (microscopic polyarteritis), and Churg-Strauss syndrome. Pulmonary disease appears at some time during the course in many patients with ANCA-associated vasculitis. The histologic features of 25 open lung biopsies and two autopsy cases were studied from 27 patients with ANCA. Patients' ages ranged from 8 to 79 years with a mean of 52.6 years. There were 12 females and 15 males. Autoantibodies were characterized as C-ANCA in 13 patients and as P-ANCA in 14 patients. Anti-proteinase 3 antibodies were documented in 12 of 13 patients with C-ANCA. Anti-myeloperoxidase antibodies were documented in all 14 patients with P-ANCA. Vascular lesions were present in 21 patients (78%) and 11 patients (41%) had bronchial lesions. Capillaritis was the most common vascular lesion (17 patients, 63%), and was found with similar frequency in patients with C-ANCA and those with P-ANCA. Extravascular structures were a common site of tissue injury. Airway lesions including bronchiolitis obliterans organizing pneumonia (4 patients, 19%), necrotizing granulomatous inflammation (4 patients, 15%), and non-granulomatous inflammation (3 patients, 11%) were more commonly associated with patients with C-ANCA. Interstitial lesions were found in 20 patients (74%), and included necrotizing granulomatous inflammation (8 patients, 30%), fibrosis (13 patients, 48%), and chronic inflammation (12 patients, 44%). No histologic lesion were found that were specific for C-ANCA or P-ANCA. This series demonstrates the wide variety of pulmonary lesions found in patients with ANCA-associated pulmonary disease, and shows that extravascular structures are a common site of injury in ANCA-associated vasculitis.
抗中性粒细胞胞浆自身抗体（ANCA）见于系统性小血管炎患者的一个亚组中，包括韦格纳肉芽肿病、显微镜下多血管炎（显微镜下多动脉炎）和变应性肉芽肿性血管炎患者。在许多ANCA相关性血管炎患者病程中的某个时候会出现肺部疾病。对27例ANCA患者的25例开胸肺活检及2例尸检病例的组织学特征进行了研究。患者年龄范围为8至79岁，平均52.6岁。有12名女性和15名男性。自身抗体在13例患者中为C-ANCA，在14例患者中为P-ANCA。13例C-ANCA患者中有12例检测到抗蛋白酶3抗体。14例P-ANCA患者均检测到抗髓过氧化物酶抗体。21例患者（78%）存在血管病变，11例患者（41%）有支气管病变。毛细血管炎是最常见的血管病变（17例，63%），在C-ANCA患者和P-ANCA患者中出现频率相似。血管外结构是组织损伤的常见部位。气道病变包括机化性肺炎（4例，19%）、坏死性肉芽肿性炎症（4例，15%）和非肉芽肿性炎症（3例，11%），更常见于C-ANCA患者。20例患者（74%）发现间质病变，包括坏死性肉芽肿性炎症（8例，30%）、纤维化（13例，48%）和慢性炎症（12例，44%）。未发现对C-ANCA或P-ANCA具有特异性的组织学病变。该系列研究显示了ANCA相关性肺部疾病患者中发现的多种肺部病变，并表明血管外结构是ANCA相关性血管炎中常见的损伤部位。
