特发性非特异性间质性肺炎与普通型间质性肺炎疾病特征和预后的比较分析

Comparison of clinicopathologic features and prognosis between idiopathic nonspecific interstitial pneumonia and usual interstitial pneumonia

目的与普通型间质性肺炎（usual interstitial pneumonia，UIP）进行比较分析，探讨特发性非特异性间质性肺炎（idiopathic nonspecific interstitial pneumonia，INSIP）的疾病特征和预后以及与UIP的鉴别诊断。方法对经电视胸腔镜或小切口开胸肺活检诊断的21例INSIP患者和18例UIP患者的临床一影像一病理学资料及疗效、预后进行比较分析。结果INSIP多见于40～50女性，临床表现无特异性，主要表现为活动后气促、咳嗽、咯痰、双下肺可有或无吸气相爆裂音；高分辨率CT（HRCT）表现为双肺弥漫分布的磨玻璃样淡斑片状和不规则网织状阴影，部分可有蜂窝肺。INSIP的病理特征为病变进展相对一致，按病理表现可分为细胞型、纤维化型和混合型。与UIP相比，纤维母细胞灶、肌硬化、镜下蜂窝肺和肺泡结构改建的检出率在INSIP和UIP分别是19．05％和100％（P〈0．001），19．05％和88．89％（P〈0．05），23．81％和94．44％（P〈0．01），33．33％和100％（P〈0．01）。两者对糖皮质激素的反应率分别为76．19％和38．89％（P〈O．01），各型INSIP的预后均明显好于UIP。结论INSIP的一般临床表现差异不明显，HRCT对疑难病例的鉴别诊断有帮助，明确诊断依赖肺活检病理诊断；纤维母细胞灶、伴胶原沉积的瘢痕化和蜂窝变组成不同时相的病变共同构成诊断UIP的形态特征，也是与INSIP的鉴别要点。

Objective To investigate clinicopathologic features and prognosis of idiopathic nonspecific interstitial pneumonia（INSIP）, and its differential diagnosis from usual interstitial pneumonia（UIP）. Methods Analysis was made on 21 INSIP patients and 18 UIP patients with biopsy-proven by open or video-assisted thoracoscopic lung biopsy. Clinical, pathological findings and follow-up information of the patients were reviewed. Results INSIP was more common in female and clinical manifestations were nonspeeific. High- resolution computed tomography（HRCT） demonstrated ground-glass, net and patchy attenuation in lung. Pathological characteristics showed a heterogeneous appearance. According to pathological characteristics, INSIP was separated into cellular, fibrosing and mixed patterns. The cellular INSIP, fibrosing INSIP and UIP had a mean age of 48 and 60,respectively. The frequencies of fibroblast foci,muscle sclerosis, honeycombing change and pulmonary architectural destruction of INSIP and UIP were 19.05% and 100% （ P 〈0. 001）, 19.05% and 88.89%P 〈0.05）,23.81% and 94.44%（P 〈0.01）,33.33% and 100%（P 〈0.01）, respectively. Response to glucocorticoid and prognosis were significantly better in INSIP patients than in UIP patients. Conclusions INSIP is not easily differential from UIP in the general clinical manifestations. HRCT is helpful for the differential diagnosis of cellular INSIP and UIP. The definite diagnosis of INISP depends on open lung biopsy.