家族性特发性肺纤维化的X线、CT诊断(附三例报告)

目的:总结特发性肺纤维化的特点,探讨家族性特发性肺纤维化(FIPF)在X线、CT诊断中的价值。方法:对一个家族中3例特发性肺纤维化(IPF)患者的X光、CT、临床、病理资料进行回顾性分析。结果:一个家族中3例(男1例、女2例)特发性肺间质纤维化,平均年龄25岁,病人症状不明显,有轻度的呼吸障碍。X线及高分辨率CT表现:双肺粟粒状的结节状阴影,不规则线状或网格状影、斑片状实变影、蜂窝样影;胸膜钙化,呈蛋壳状包围在肺的周围。结论:X线、CT表现对特发性肺纤维化的诊断具有定性作用。在病人不愿做活检时,作为一种无创检查,高清晰度CT对家族性特发性肺纤维化病人的家族成员的筛查及诊断具有重要价值。

Objective Summarizing the characteristic of idiopathic pulmonary fibrosis （IPF）,to explore the value of Familial idiopathic pul- monary fibrosis in X-ray and CT diagnosis.Methods The X-ray, CT, clinical and pathological data of 3 idiopathic pulmonary fibrosis Patients in a family were retrospectively reviewed.Results There are three idiopathic pulmonary fibrosis eases（1 male, 2 female） in a family, the average age is 25, Patient＇s symptoms was not obvious with mild breathing disorder. X-ray and high resolution CT Findings ： nodular miliary shadow in the lungs, irregular linear or grid-like shadow, patchy consolidation shadow, honeycomb-like shadow; Pleural calcification, Surrounded by lungs like eggshell.Conclusion X-ray and CT findings have a qualitative effect on the Familial idiopathic pulmonary fibrosis diagnosis. As a non-invasive examination, high resolution CT has great value on screening and diagnosing the family of Familial idiopathic pulmonary fibrosis patients when they unwilling to do biopsy.