[The clinical, radiological and pathological diagnosis of non-specific interstitial pneumonia].
[The clinical, radiological and pathological diagnosis of non-specific interstitial pneumonia].
复制标题
非特异性间质性肺炎的临床、影像学和病理诊断[J].
DOI：
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发表时间：
2004
期刊：
影响因子：
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通讯作者：
Ming
中科院分区：
文献类型：
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作者：
Yong;W. Yao;Jie Zheng;Tian;F. Pei;Ming
目的
目的探讨非特异性间质性肺炎（NSIP）的临床、影像学及病理学特征，评价NSIP的多学科诊断方法。
方法
出院时诊断或可能诊断为NSIP的患者的临床数据、肺部CT扫描和病理切片由呼吸内科医生、放射科医生和病理科医生组成的小组重新评估。病理诊断和临床诊断由专家组根据美国胸科学会/欧洲呼吸学会2002年的分类进行。
结果
7例经手术肺活检证实，1例以弥漫性支气管扩张为主，1例以机化性肺炎为主。其余5例符合NSIP标准。对2例疑似病例行CT引导下经皮肺穿刺活检，由于标本不足以明确诊断，但由于缺乏特异性表现，且临床和影像学特征一致，维持了疑似NSIP的诊断。在NSIP或可能的NSIP患者中，磨玻璃样阴影是主要的CT特征，没有典型的特发性肺纤维化表现。在2例可能病例中，1例在肺活检后20天死于疾病恶化，1例在初步诊断后2年发现多发性肌炎/皮肌炎。5例确诊病例中，1例确诊为多发性肌炎/皮肌炎，其余4例未发现潜在病因。1例患者在首次诊断后3年死于进行性纤维化和呼吸衰竭。
结论
NSIP的影像学表现无法诊断，因此外科肺活检是确诊的首选方法。NSIP的临床和病理诊断需要呼吸科医生、放射科医生和病理科医生的多学科方法。寻找可能的根本原因是动态诊断过程的重要组成部分。
OBJECTIVE
To describe the clinical,radiological and pathological features of non-specific interstitial pneumonia (NSIP), and to evaluate the multidisciplinary approach to the diagnosis of NSIP.
METHODS
The clinical data, lung CT scans and pathologic slides of patients with a diagnosis or a probable diagnosis of NSIP on discharge were re-evaluated by a panel of respiratory physicians, radiologists and pathologists. A pathological diagnosis and a clinical diagnosis were made by the panel according to the 2002 classification by American Thoracic Society/European Respiratory Society.
RESULTS
In the 7 cases diagnosed by surgical lung biopsy, diffuse bronchiectasis was found to be the predominant feature in 1 case, and organizing pneumonia in another case. The remaining 5 cases met the criteria of NSIP. CT guided percutaneous lung biopsy was performed in 2 probable cases, for which the specimens were inadequate for a definite diagnosis, but because of a lack of specific findings, and with the consistent clinical and radiological features, the diagnosis of probable NSIP was maintained. In the patients with NSIP or probable NSIP, ground glass opacities were the predominant CT features, without the typical appearance of idiopathic pulmonary fibrosis. Of the 2 probable cases, 1 died from disease deterioration 20 days after lung biopsy, and 1 was found to have multiple myositis/dermatomyositis 2 years after the initial diagnosis. Among the 5 definite cases, one was confirmed to have multiple myositis/dermatomyositis, but no underlying causes were found for the other 4 cases. One patient died from progressive fibrosis and respiratory failure 3 years after the initial diagnosis.
CONCLUSIONS
The radiological manifestations of NSIP were not diagnostic, and therefore surgical lung biopsy was the procedure of choice in making a definite diagnosis. The clinical and pathological diagnosis of NSIP needs a multidisciplinary approach by respiratory physicians, radiologists and pathologists. The search for a possible underlying cause is an important part of the dynamic diagnostic process.