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-  2016 

伴有自身免疫特征的间质性肺疾病单中心研究

DOI: doi:10.7507/1671-6205.2016036

Keywords: 伴有自身免疫特征的间质性肺疾病, 临床特征, 随访

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Abstract:

目的 总结伴有自身免疫特征的间质性肺疾病(IPAF)的临床特点。 方法 回顾分析2010年1月至2013年8月我科诊断的间质性肺疾病(ILD)患者,筛选出IPAF患者,记录和分析临床、实验室检查、影像学、肺功能和治疗结果。 结果 254例ILD患者中,25例符合IPAF诊断标准,26例为确定的结缔组织病相关ILD(DCTD-ILD)。IPAF和DCTD-ILD在关节痛、干燥症状、技工手、抗核抗体阳性、抗CCP抗体阳性、残气量方面存在显著差异(P<0.05)。5例IPAF患者胸部高分辨率CT出现“薄饼”样改变(磨玻璃伴实变为主,双肺底分布为主),镜下表现为弥漫性胶原纤维增生、肺泡壁增厚,伴有大量淋巴细胞肺间质浸润,部分肺泡腔内可见肉芽肿组织填充。病理确诊为非特异性间质性肺炎(NSIP)重叠机化性肺炎(OP)。随访发现IPAF患者无进展生存时间显著长于DCTD-ILD患者[(14.32±5.74)个月比(10.31±3.70)个月,P<0.05]。 结论 诊治ILD时,出现技工手、抗核抗体(核仁型)、NSIP重叠OP的影像学要考虑IPAF,IPAF可能较DCTD-ILD进展慢,预后好

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