非特异性间质性肺炎八例临床病理分析

A clinicopathologic study of nonspecific interstitial pneumonia

目的 探讨非特异性间质性肺炎 (NSIP)的病理和临床特点。方法 对 8例经电视胸腔镜或小切口开胸肺活检诊断为NSIP的病例进行光镜观察和临床病理资料回顾性分析。结果 NSIP多见于女性 (男∶女 =1∶3,2 / 6 ) ,平均年龄 48岁 ,临床表现为渐进性呼吸困难、咳嗽、咳痰、双下肺闻及爆裂音 ,肺功能主要为限制性通气障碍 ,高分辨CT表现为双下肺野为主的网状、片状磨玻璃样改变。病理特征为均一的间质炎症和纤维化 ,4例有闭塞性细支气管炎伴机化性肺炎样改变 ,3例有纤维母细胞灶 ,1例有蜂窝肺 ;组织学类型 :混合型 6例 ,纤维化型 2例。对皮质激素反应 :显效组与有效组各4例 ;与纤维化型相比 ,大部分混合型对皮质激素有较好的反应。结论 NSIP的组织学类型与疗效有密切的关系 ;纤维化性的NSIP与普通型间质性肺炎在病理组织学上不易鉴别 ,诊断需密切联系临床。

Objective To investigate the clinicopathologic features of nonspecific interstitial pneumonia (NSIP) Methods The clinical and pathological data of 8 cases with NSIP confirmed by open or video-assisted thoracoscopic lung biopsy were reviewed retrospectively Results NSIP occurred more in female (male:female 1∶3, 2/6), and the patients′average age was 48 years The clinical manifestation was progressive dyspnea, cough and sputum, crackles over both lungs and restrictive ventilating abnormalities Reticular changes and patchy ground-glass attenuation in lower lung fields were the main findings on high resolution computed tomography The pathological characteristic was uniform interstitial inflammation and fibrosis Three cases showed fibroblast foci , was BOOP like changes in 4 and honeycombing in 1 case of the 8 patients, 6 had cellular and fibrosing patterns; 2 had fibrosing patterns only; the former showed better response to glucocorticoid therapy than the latter Conclusions The histologic patterns of NSIP correlated closely with the therapeutic effect of glucocorticoids It is difficult to differentiate pathologically the fibrosing pattern of NSIP from usual interstitial pneumonia,therefore clinical data are indispensable in the diagnosis