Gal A A, Salinas F F, Staton G W
Department of Anatomic Pathology, Emory University School of Medicine, Atlanta, Ga.
Arch Pathol Lab Med. 1994 Dec;118(12):1209-14.
Antineutrophil cytoplasmic autoantibodies (ANCAs), classified as either perinuclear (P-ANCAs) or cytoplasmic (C-ANCAs), have been recently recognized as important markers for the diagnosis and monitoring of systemic vasculitic disorders. The purpose of this study was to review retrospectively the clinical and pathological features in patients with P-ANCA-positive (P-ANCA+) patterns and pulmonary disease who underwent open lung biopsies and to contrast these findings with those found in patients with C-ANCA-positive (C-ANCA+) patterns who underwent open lung biopsies. Nine patients with ANCA+ pattern (five with P-ANCA+ and four with C-ANCA+ patterns) who had evidence of systemic vasculitis and pulmonary dysfunction underwent open lung biopsies. A comparison of the clinical presentation in patients with P-ANCA+ vs C-ANCA+ patterns showed few apparent differences in the clinical presentation or in the organ involvement. Histologic review of the findings from the open lung biopsies showed similar patterns of pulmonary injury, irrespective of the specific ANCA-staining pattern. Major pathologic changes included intra-alveolar hemorrhage (four patients with P-ANCA+ patterns vs three patients with C-ANCA+ patterns), necrotizing capillaritis (four patients with P-ANCA+ patterns vs three patients with C-ANCA+ patterns), vasculitis (three patients with P-ANCA+ patterns vs two patients with C-ANCA+ patterns), and necrotizing granulomatous inflammation (two patients with P-ANCA+ patterns vs one patient with C-ANCA+ patterns). Unusual pathologic findings included chronic interstitial fibrosis (two patients with P-ANCA+ patterns vs two patients with C-ANCA+ patterns), cavitary nodules with necrotic neutrophils and minimal granulomatous inflammation (one patient with P-ANCA+ patterns), and bronchiolocentric granulomatous inflammation (one patient with P-ANCA+ patterns vs one patient with C-ANCA+ patterns). In ANCA-related pulmonary disease, there are few significant clinical or pathological differences when patients with P-ANCA+ patterns are compared with patients with C-ANCA+ patterns.
抗中性粒细胞胞浆自身抗体（ANCA）分为核周型（P-ANCA）和胞浆型（C-ANCA），最近已被公认为是诊断和监测系统性血管炎疾病的重要标志物。本研究的目的是回顾性分析接受开胸肺活检的P-ANCA阳性（P-ANCA+）且患有肺部疾病患者的临床和病理特征，并将这些结果与接受开胸肺活检的C-ANCA阳性（C-ANCA+）患者的结果进行对比。9例有系统性血管炎和肺功能障碍证据的ANCA+型患者（5例P-ANCA+型和4例C-ANCA+型）接受了开胸肺活检。P-ANCA+型与C-ANCA+型患者临床表现的比较显示，临床表现或器官受累方面几乎没有明显差异。对开胸肺活检结果的组织学检查显示，无论具体的ANCA染色类型如何，肺损伤模式相似。主要病理变化包括肺泡内出血（P-ANCA+型患者中有4例，C-ANCA+型患者中有3例）、坏死性毛细血管炎（P-ANCA+型患者中有4例，C-ANCA+型患者中有3例）、血管炎（P-ANCA+型患者中有3例，C-ANCA+型患者中有2例）和坏死性肉芽肿性炎症（P-ANCA+型患者中有2例，C-ANCA+型患者中有1例）。不寻常的病理表现包括慢性间质纤维化（P-ANCA+型患者中有2例，C-ANCA+型患者中有2例）、伴有坏死性中性粒细胞和少量肉芽肿性炎症的空洞结节（1例P-ANCA+型患者）以及细支气管中心性肉芽肿性炎症（P-ANCA+型患者中有1例，C-ANCA+型患者中有1例）。在ANCA相关肺部疾病中，P-ANCA+型患者与C-ANCA+型患者相比，临床或病理方面几乎没有显著差异。
