The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis
The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis
复制标题
DOI：
10.1164/ajrccm.162.6.2003049
复制
发表时间：
2000-12-01
影响因子：
24.7
通讯作者：
Wells, AU
中科院分区：
文献类型：
--
作者：
Nicholson, AC;Colby, TV;Wells, AU
孤立性不明原因：纤维性肺泡炎(CFA)是一种进行性间质性肺部疾病，从呼吸困难开始，中位生存期为3至6年。CFA可细分为有预后意义的组织病理学类型，包括非特异性间质性肺炎(NSIP)。我们回顾了1978年至1989年间78例临床病理诊断为CFA的患者，以评估这些组织病理类型的患病率和预后意义，尤其是NSIP。两位肺组织病理学专家将活检表现重新分类为普通型间质性肺炎(UIP)(47%)、非间质性肺炎(36%)或脱屑性间质性肺炎(DIP)/呼吸性毛细支气管炎相关性间质性肺疾病(RBILD)(17%)。病理学家之间的kappa一致性系数为0.49。67例随访至死亡或活检后10年，其中50例死亡，中位随访期42个月(UIP，%；NSIP，61%，DIP/RBILD，0%)。DIP/RBILD组的存活率最高，NSIP组的存活率高于UIP，p<；0.0005。当分析仅限于UIP或NSIP患者时，UIP的死亡率仍然较高，p<；0.01，但纤维性NSIP患者的5年生存率仅为45%，表明这种组织学表现往往与不良预后相关。DIP/RBILD组对治疗的反应比NSIP(P<；0.01)或UIP(P<；0.0005)更常见。这项研究证实了预后：根据组织病理学模式对CFA患者进行亚型的价值。然而，在临床典型的CFA患者中，纤维性NSIP的组织学诊断需要谨慎解释，并不一定意味着良好的结果。
Lone cryptogenic: fibrosing alveolitis (CFA) is a progressive interstitial lung disease, with a median survival of 3 to 6 yr from the onset of dyspnea. CFA can be subdivided into prognostically significant histopathologic patterns, including nonspecific interstitial pneumonia (NSIP). We reviewed 78 patients with a clinicopathologic diagnosis of CFA, biopsied between 1978 and 1989, to evaluate the prevalence and prognostic significance of these histopathologic: patterns, in particular NSIP. Biopsy appearances were reclassified by two pulmonary histopathologists as usual interstitial pneumonia (UIP) (47%), NSIP (36%), or desquamative interstitial pneumonia (DIP)/respiratory bronchiolitis-associated interstitial lung disease (RBILD) (17%). The kappa coefficient of agreement between pathologists was 0.49. In 67 cases, follow-up was complete to death or 10 yr after biopsy, with 50 deaths during a median follow-up of 42 mo (UIP, 89%; NSIP, 61%, DIP/RBILD, 0%). Survival was highest in DIP/RBILD and higher in NSIP than UIP, p < 0.0005. When analysis was confined to patients with UIP or NSIP, the mortality of UIP remained higher, p < 0.01, but the 5-yr survival in patients with fibrotic NSIP was only 45%, indicating that this histologic appearance is often associated with a poor outcome. A response to treatment was more frequent in DIP/RBILD than in NSIP (p < 0.01) or UIP (p < 0.0005). This study confirms the prognostic: value of subclassifying patients with CFA according to histopathologic pattern. However, in patients with clinically typical CFA, a histologic diagnosis of fibrotic NSIP needs to be interpreted with caution and does not necessarily denote a good outcome.