Epler G R, Colby T V, McLoud T C, Carrington C B, Gaensler E A
N Engl J Med. 1985 Jan 17;312(3):152-8. doi: 10.1056/NEJM198501173120304.
In 50 of 94 patients with bronchiolitis obliterans we found no apparent cause or associated disease, and the bronchiolitis obliterans occurred with patchy organizing pneumonia. Histologic characteristics included polypoid masses of granulation tissue in lumens of small airways, alveolar ducts, and some alveoli. The fibrosis was uniform in age, suggesting that all repair had begun at the same time. The distribution was patchy, with preservation of background architecture. Clinically, there was cough or flu-like illness for 4 to 10 weeks, and crackles were heard in the lungs of 68 per cent of the patients. Radiographs showed an unusual pattern of patchy densities with a "ground glass" appearance in 81 per cent. Physiologically, there was restriction in 72 per cent of the patients, and 86 per cent had impaired diffusing capacity. Obstruction was limited to smokers. The mean follow-up period was four years. With corticosteroids, there was complete clinical and physiologic recovery in 65 per cent of the subjects; two died from progressive disease. This disorder differs from bronchiolitis obliterans with irreversible obstruction. It was confused most often with idiopathic pulmonary fibrosis. In view of the benign course and therapeutic response, a histologic distinction is important.
在94例闭塞性细支气管炎患者中,50例未发现明显病因或相关疾病,闭塞性细支气管炎伴有斑片状机化性肺炎。组织学特征包括小气道、肺泡管和部分肺泡腔内的息肉样肉芽组织肿块。纤维化在时间上是一致的,表明所有修复过程均在同一时间开始。分布呈斑片状,背景结构得以保留。临床上,患者咳嗽或出现流感样症状4至10周,68%的患者肺部可闻及啰音。81%的患者胸部X线片显示出一种异常的斑片状密度影,呈“磨玻璃”样外观。生理方面,72%的患者存在肺功能受限,86%的患者弥散功能受损。阻塞仅限于吸烟者。平均随访期为4年。使用皮质类固醇治疗后,65%的患者临床和生理功能完全恢复;2例死于疾病进展。这种疾病不同于伴有不可逆性阻塞的闭塞性细支气管炎。它最常与特发性肺纤维化相混淆。鉴于其良性病程和治疗反应,组织学鉴别很重要。