普通型间质性肺炎的临床病理特征及其与特发性非特异性间质性肺炎的鉴别诊断

目的 探讨普通型间质性肺炎(UIP)的临床病理特征以及与特发性非特异性间质性肺炎(INSIP)的鉴别诊断。方法 对15例经电视胸腔镜或小切口开胸肺活检诊断为UIP的病例进行光镜观察和临床病理资料回顾性分析,治疗后随访,与11例病理诊断的INSIP进行比较分析。结果UIP多见于50岁以上的男性,临床主要表现为活动后气促、咳嗽咳痰、双下肺闻及吸气相爆裂音;高分辨CT表现为以中下肺和胸膜下为主的片状和网状阴影,8例有蜂窝肺。UIP的病理特征为病变进展不一致,间质的炎症、纤维化和蜂窝变与正常肺组织呈交替分布,轻重不一。纤维母细胞灶、肌硬化、镜下蜂窝肺、弥漫胶原沉积和肺泡结构改建的检出情况在UIP和INSIP分别是15/15和3/11(P<0.001)、12/15和4/11(P<0.05)、13/15和3/11(P<0.01)、15/15和6/11(P<0.01)、15/15和5/11(P<0.01)。两者对糖皮质激素的反应率分别为3/15和8/11(P<0.05)。结论 UIP和INSIP的一般临床表现差异不明显,高分辨CT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。

Clinicopathologic features of usual interstitial pneumonia and its differential diagnosis from idiopathic nonspecific interstitial pneumonia

Objective To investigate the clinicopathologic features of usual interstitial pneumonia (UIP) and its differential diagnosis from idiopathic nonspecific interstitial pneumonia (INSIP). Methods The clinical and pathological features of 15 UIP and 11 cases of INSIP, having received open or video-assisted thoracoscopic lung biopsies and having follow-up information were reviewed. Results UIP occurred more often in males over 50 years of age. Clinical findings included progressive shortness of breath, cough, sputum and crackles over both lung fields. High resolution computerized tomography ( HRCT) showed patchy attenuation, especially over both lower lobes. Honeycombing was found in 8 cases. Histologically, UIP was characterized by scattered fibrotic foci, fibrosis ( often associated with honeycombing) and pulmonary architectural destruction. It had a heterogeneous appearance, with alternating areas of normal lung, interstitial inflammation, fibrosis, and honeycomb changes. The frequencies of fibroblastic foci, muscle sclerosis, honeycomb changes, diffuse fibrosis and pulmonary architectural destruction in UIP and INSIP were 100% and 27. 3% (P < 0. 001) , 80. 0% and 36. 4% (P < 0.05), 86.7% and 27. 3% (P< 0.001), 100% and 54. 5% (P<0.01) and 100% and 45. 5% (P<0. 05), respectively. The response rate to glucocorticoid was 26. 7% and 72. 7% (P < 0. 05) in UIP and INSIP respectively. Conclusions The distinction between UIP and INSIP is difficult if based on clinical examination alone. HRCT is helpful for differential diagnosis in some difficult cases. Definite diagnosis depends mainly on open lung biopsies. Key histologic features of UIP include heterogeneous appearance with interstitial inflammation, fibroblastic foci, scar formation and honeycomb changes.