Nagai S, Kitaichi M, Itoh H, Nishimura K, Izumi T, Colby T V
Dept of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Japan.
Eur Respir J. 1998 Nov;12(5):1010-9. doi: 10.1183/09031936.98.12051010.
Based on past difficulties in clinically differentiating patients with idiopathic pulmonary fibrosis (IPF), bronchiolitis obliterans-organizing pneumonia (BOOP), and nonspecific interstitial pneumonia/fibrosis (NSIP), which all manifest clinically as interstitial lung disease, experience with pathologically confirmed examples of the three diseases was reviewed to compare clinical profiles and prognosis and to define NSIP more clearly. Thirty-one patients (15 males and 16 females) were pathologically identified as NSIP and subclassified into either the cellular (n=16) or fibrotic group (n=15). All 31 patients were clinically considered to be idiopathic NSIP cases. Patients with idiopathic BOOP (n=16) and IPF (n=64) were compared with the NSIP patients. Subacute presentation of interstitial lung disease characterized both idiopathic NSIP and idiopathic BOOP. NSIP patients showed volume loss on a chest radiograph (29.0%) and honeycombing on a computed tomography scan (25.8%); these features were not found in BOOP patients. Bronchoalveolar lavage lymphocytosis was characteristic of both BOOP and NSIP. Two subgroups of NSIP can be recognized histologically: patients in the fibrotic group had a less favourable outcome than those in the cellular group. BOOP and NSIP had a more favourable outcome than IPF. In conclusion, idiopathic nonspecific interstitial pneumonia can be differentiated from other types of idiopathic interstitial pneumonia, both pathologically and clinically.
鉴于过去在临床上难以区分特发性肺纤维化（IPF）、闭塞性细支气管炎伴机化性肺炎（BOOP）和非特异性间质性肺炎/纤维化（NSIP）患者，这三种疾病在临床上均表现为间质性肺疾病，因此回顾了这三种疾病经病理证实的病例，以比较临床特征和预后，并更明确地定义NSIP。31例患者（15例男性和16例女性）经病理确诊为NSIP，并分为细胞型（n = 16）或纤维化型（n = 15）。所有31例患者临床上均被认为是特发性NSIP病例。将特发性BOOP患者（n = 16）和IPF患者（n = 64）与NSIP患者进行比较。特发性NSIP和特发性BOOP的间质性肺疾病均表现为亚急性。NSIP患者胸部X线片显示肺容积缩小（29.0%），计算机断层扫描显示有蜂窝状改变（25.8%）；BOOP患者未发现这些特征。支气管肺泡灌洗淋巴细胞增多是BOOP和NSIP的特征。NSIP在组织学上可分为两个亚组：纤维化型患者的预后比细胞型患者差。BOOP和NSIP的预后比IPF好。总之，特发性非特异性间质性肺炎在病理和临床方面均可与其他类型的特发性间质性肺炎相鉴别。
