非特异性间质性肺炎临床-放射-病理诊断分析

目的对经肺活检诊断的非特异性间质性肺炎(NSIP)病例进行分析，探讨临床一放射一病理诊断的重要性。方法呼吸科、放射科和病理科医师对出院诊断和疑诊的9例NSIP病例的临床资料、影像特征和病理诊断进行回顾性分析，根据美国胸科学会和欧洲呼吸学会(ATS／ERS)的分类标准，重新作出一致的临床和病理诊断。结果出院诊断NSIP患7例，病理标本来自外科肺活检；疑诊NSIP患2例，病理标本来自CT引导下肺穿刺活检。回顾性病理分析发现，外科肺活检诊断的7例中5例符合ATS／ERS诊断标准，确诊为NSIP；1例主要病理特征为弥漫性支气管扩张，1例为机化性肺炎，不能诊断NSIP。经CT肺活检疑诊的2例因组织标本较小，不能进行全面病理评价，但因缺乏其他特征性病变，结合临床和影像表现，仍拟诊NSIP。全部病例的影像学表现以磨玻璃样阴影为主，均不具备特发性肺纤维化(IPF)的典型特征。确诊NSIP的5例中1例存在多肌炎／皮肌炎，临床诊断继发性NSIP；其余4例未发现潜在病因，临床诊断特发性NSIP，其中1例在诊断后3年因肺纤维化进行性加重、呼吸衰竭死亡。2例疑诊病例中1例在人院后20d死亡，1例在2年后确诊多肌炎／皮肌炎。结论NSIP的影像学表现缺乏特征性，外科肺活检是确立诊断的主要手段。NSIP的临床和病理诊断需要临床、放射和病理科医师的共同参与，而进一步寻找潜在病因是诊断过程中的重要目标。

The clinical,radiological and pathological diagnosis of non-specific interstitial pneumonia

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 bronchioectasis 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/dermomyositis 2 years after the initial diagnosis. Among the 5 definite cases, one was confirmed to have multiple myositis/dermomyositis, 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.