结缔组织相关性间质性肺疾病与特发性间质性肺疾病合并肺血栓栓塞症17例的临床特点分析

Analysis of the clinical features of pulmonary thromboembolism in connective tissue disease related interstitial lung disease and idiopathic interstitial pneumonia in 17 cases

目的 分析结缔组织相关性间质性肺疾病（CTD-ILD）和特发性间质性肺疾病（IIP）合并肺血栓栓塞症（PTE）患者的临床特征。方法 收集2011-2014年在广州呼吸疾病研究所住院的CTD-ILD合并PTE及IIP合并PTE的患者17例（ILD合并PTE组）,并募集同期肺通气功能相匹配的CTD-ILD及IIP共20例患者（单纯ILD组）作为对照,对其临床资料进行分析。并在此基础上再对比ILD合并PTE组中的CTD-ILD亚组及IIP亚组。结果1与单纯ILD组比较,在ILD合并PTE组中,患者出现近期新发呼吸困难或呼吸困难加重的发生率有增高的趋势;ILD合并PTE组的动脉血氧分压更低,分别为（76.4±22.3）mm Hg和（63.4±19.2）mm Hg（P〈0.05）;气体交换功能（DLCO%预计值）更差,分别为（48.5±11.6）%和（37.9±12.8）%。2ILD合并PTE组中,CTD-ILD亚组和IIP亚组的平均年龄分别为（59.83±13.40）岁和（71.90±4.68）岁,病程中出现PTE症状的时间分别为（18.07±16.71）个月和（34.75±14.69）个月,CTD-ILD患者发病年龄更小（P〈0.05）,病程中出现PTE症状的时间更短（P〈0.05）;IIP亚组患者共5例（71.43%）出现下肢水肿,CTD-ILD亚组中没有患者出现下肢水肿;IIP亚组中3例（30.00%）患者长期口服糖皮质激素,而CTD-ILD亚组共5例（71.43%）患者长期口服糖皮质激素。结论 ILD合并PTE可使患者气体交换功能障碍进一步加重,CTD本身的发生进展和长期口服糖皮质激素是PTE发生的高危因素。与IIP合并PTE比较,CTD-ILD合并PTE患者起病年龄更小,出现PTE的病程更短。

Objective To study the clinical characteristics of pulmonary thromboembolism in connective tissue disease related interstitial lung disease（CTD-ILD） and the idiopathic interstitial pneumonia（IIP） respectively. Methods From2011 to 2014, 17 ILD（CTD-ILD and IIP） patients with pulmonary thromboembolism（PTE） in Guangzhou Institute of Respiratory Disease were retrospectively analyzed. 20 simplex ILD（CTD-ILD and IIP） patients with well-matched lung function were also recruited as control group during the same period. The clinical data and symptoms were summarized and statistically calculated. And a further analysis of clinical difference between CTD-ILD with PTE subgroup and IIP with PTE subgroup was followed. Results 1 Compared with simplex ILD group, incidence of new dyspnea or aggravation of dyspnea was higher in ILD with PTE group; arterial partial pressure of oxygen was significantly lower in ILD with PTE group（P〈0.05）, which were（76.4±22.3） mm Hg and（63.4±19.2） mm Hg respectively. And a significant lower pulmonary diffusion function（DLCO% of the predicted values） was found in ILD with PE group, which were（48.5±11.6）% and（37.9±12.8）% respectively（P〈0.05）. 2In ILD with PTE group, average age of CTD-ILD and IIP subgroups were（59.83±13.40） years and（71.90±4.68） years The occurrence of PTE were（18.07±16.71） months and（34.75 ±14.69） months. It indicated that the age at onset of CTD-ILD was younger than IIP（P〈0.05）, and the occurrence of PTE during ILD course was earlier than IIP. No patients of CTD-ILD subgroup had edema of lower limbs, while 5 patients（71.43%） of IIP subgroup had the symptom. 3 patients（30.00%） of IIP subgroup had oral adminstration of corticosteroids histories, while 5 patients（71.43%） of CTD-ILD subgroup had oral adminstration of corticosteroids histories. Conclusion Association of PTE can aggravate pulmonary diffusion dysfunction. Occurance and progression of CTD and long-term oral adminstration of corticosteroids are all high risk factors of PTE.Compared with IIP patients with PTE, CTD-ILD patients with PTE are younger and have a shorter course of PTE onset.