IDIOPATHIC BRONCHIOLITIS OBLITERANS ORGANIZING PNEUMONIA - DEFINITION OF CHARACTERISTIC CLINICAL PROFILES IN A SERIES OF 16 PATIENTS

IDIOPATHIC BRONCHIOLITIS OBLITERANS ORGANIZING PNEUMONIA - DEFINITION OF CHARACTERISTIC CLINICAL PROFILES IN A SERIES OF 16 PATIENTS
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DOI:
10.1378/chest.96.5.999
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发表时间:
1989-11-01
期刊:
影响因子:
9.6
通讯作者:
BRUNE, J
BRUNE, J
中科院分区:
医学1区
文献类型:
--
作者:
CORDIER, JF;LOIRE, R;BRUNE, J

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闭塞性细支气管炎机化性肺炎(BOOP)是一种常见于各种明确或特发性肺损伤的病理改变。由于特发性BOOP患者的临床表现各不相同,我们对16例特发性BOOP患者进行了肺组织学研究,以明确特发性BOOP更清晰、更均匀的临床和影像特征。我们将患者分为三组:第一组(n=4),肺炎类型的多发斑片状移行肺受累。他们的临床病程为亚急性,伴有咳嗽、发热、体重减轻、轻度呼吸困难和血沉升高。胸部X线片和CT扫描显示多发性肺泡影。所有患者经皮质类固醇治疗后均完全恢复,但在治疗停止过快时复发。第2组(n=5)为临床表现相似的肺炎类型的孤立性肺损害。由于怀疑是癌症,他们接受了肺脏区域的手术切除,没有复发。第3组7例,表现为肺间质病型弥漫性肺损害。他们有更严重的呼吸困难的进行性发作,在所有的肺表面都能听到爆裂声,在胸部影像上有或没有肺泡混浊的间质混浊。皮质类固醇治疗的改善仅在3例患者中获得。在所有三组中,肺功能测试结果都显示出限制性模式。无一例具有单纯闭塞性毛细支气管炎的梗阻型特征。前两组BAL均呈混合型(淋巴细胞和中性粒细胞增多)。因此,我们区分了三种特发性BOOP患者的临床和影像特征:多发性斑块性肺炎、孤立性肺炎和弥漫性间质性肺疾病。这些特征是如此不同,以至于在特发性BOOP的临床研究中应该加以区分。
Bronchiolitis obliterans organizing pneumonia (BOOP) is a pathologic finding common to various injuries to the lung of either definite or idiopathic etiology. Since the presentation of patients with idiopathic BOOP varies, we studied 16 patients with BOOP on pulmonary histology to define more distinct and homogeneous clinical and imaging profiles of idiopathic BOOP. We distinguished three groups of patients: grouup 1 (n=4), with multiple patchy migratory pulmonary involvement of the pneumonia type. Their clinical course was subacute, with cough, fever, weight loss, mild dyspnea, and increased ESR. Chest x-ray film and CT scan showed multiple alveolar opacities. All patients completely recovered with corticosteroid therapy but relapsed when therapy was stopped too rapidly. Group 2 (n=5) had solitary pulmonary involvement of the pneumonia type occurring in a similar clinical context. Since carcinoma was suspected, they underwent surgical excision of the pneumonic area and recovered without relpase. Group 3 patients (n=7) presented with diffuse pulmonary involvement of the interstitial lung disease type. They had more progressive onset of more severe dyspnea, crackles heard over all lung surfaces, and interstitial opacities with or without alveolar opacities on chest imaging. Improvement with corticosteroid therapy was obtained in only three patients. In all three groups, lung function test results showed a restrictive pattern. The obstructive pattern characteristic of pure bronchiolitis obliterans was found in none. BAL showed a mixed pattern (increase of both lymphocytes and polymorphonuclear cells) in the patients of the first two groups. Thus, we distinguished three characteristic clinical and imaging profiles in patients with idiopathic BOOP: multiple patchy pneumonia, solitary pneumonia, and diffuse interstitial lung disease. These profiles are so different that they should be distinguished in clinical studies of idiopathic BOOP.