论著

甲周毛细血管异常与系统性硬化相关肺间质纤维化的关系

  • 季兰岚 ,
  • 王鹤 ,
  • 张晓慧 ,
  • 张卓莉
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  • (北京大学第一医院1.风湿免疫科,2.影像科, 北京100034)

网络出版日期: 2018-06-18

基金资助

北京大学第一医院院级青年基金(2017QN06)资助

Correlation between nailfold capillaroscopic findings and presence of interstitial lung disease in systemic sclerosis patients

  • JI Lan-lan ,
  • WANG He ,
  • ZHANG Xiao-hui ,
  • ZHANG Zhuo-li
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  • (1. Department of Rheumatology and Immunology, 2. Department of Radiology, Peking University First Hospital, Beijing 100034, China)

Online published: 2018-06-18

Supported by

Supported by Youth Research Project of Peking University First Hospital(2017QN06)

摘要

 目的: 评估甲周毛细血管异常与系统性硬化(systemic sclerosis,SSc)患者发生肺间质病(interstitial lung disease,ILD)的关系。方法:共纳入71例SSc患者,其中合并ILD患者45例、无ILD患者26例,对所有入选患者均进行了甲周毛细血管显微镜(nailfold capillaroscopy,NC)检查,并对显微镜下的异常表现包括毛细血管扩张、巨大毛细血管、微出血、毛细血管数量减少、无血管区、分叉/绒毛样毛细血管以及毛细血管分布紊乱进行了半定量评分。进一步将这些患者的甲周毛细血管显微镜改变与患者的性别、年龄、病程、有/无雷诺现象等临床表现、血清学指标、有/无ILD、ILD患者的肺CT评分以及肺功能参数进行相关分析。结果:71例患者中女性占91.5%,平均年龄(52.59±12.77)岁,平均病程(3.00±6.00)年。90.1%的患者甲周毛细血管呈硬皮病样改变,71例患者中45例(63.3%)合并ILD。与无ILD的患者相比,合并ILD患者的NC中毛细血管数量减少[0.50(1.03) vs. 0.00(0.43),P=0.003]、无血管区评分[0.75(1.24) vs. 0.25(0.83),P=0.006]和分叉/绒毛样毛细血管评分[0.33(0.88) vs. 0.13(0.25),P=0.006]均显著升高,而其他NC评分在合并ILD和无ILD患者间差异均无统计学意义。Logistic分析发现,分叉/绒毛样毛细血管评分与弥漫型SSc同为预测ILD发生的独立危险因素。ILD受累更严重的患者巨大毛细血管评分更低,轻度ILD和严重ILD组分别为0.25(0.94)和0.00(0.28)(P=0.019)。结论:SSc中甲周毛细血管的缺失及严重畸形与ILD的发生存在相关性,其中严重畸形毛细血管评分是预测ILD发生的独立危险因素,巨大毛细血管的减少与ILD的严重程度相关,提示NC表现在SSc相关ILD中可能具有一定的临床意义。

本文引用格式

季兰岚 , 王鹤 , 张晓慧 , 张卓莉 . 甲周毛细血管异常与系统性硬化相关肺间质纤维化的关系[J]. 北京大学学报(医学版), 2018 , 50(3) : 501 -506 . DOI: 10.3969/j.issn.1671-167X.2018.03.018

Abstract

Objective: To evaluate the correlation between nailfold capillaroscopic (NC) findings and the presence of interstitial lung disease (ILD) in systemic sclerosis (SSc) patients. Methods: We retrospectively involved 71 SSc patients, 45 patients with ILD. NC was performed in all the patients according to the standard method. The NC findings were semi-quantitatively scored, including enlarged and giant capillaries, hemorrhages, loss of capillaries, avascular areas, ramified/bushy capillaries and disorganization of the vascular array. The demographic and clinical data collected were gender, age, presence/absence of Raynaud phenomenon (RP), duration of RP, serological acute phase reactants and antibodies, presence of ILD (also evaluated the CT score for ILD) and pulmonary function parameters. Results: Among the 71 patients, the frequency of the women was 91.5%, the mean age was (52.59±12.77) years, and disease duration was (3.00±6.00) years. NC changes of the scleroderma pattern were observed in 90.1% patients. There were 45 patients with ILD and 26 patients without ILD. The patients with ILD had significantly higher loss of capillaries score [0.50 (1.03) vs. 0.00 (0.43), P=0.003], avascular area score [0.75 (1.24) vs. 0.25 (0.83), P=0.006] and ramified/bushy capillaries score [0.33 (0.88) vs. 0.13 (0.25), P=0.006] compared with those without ILD. Moreover, ramified/bushy capillaries score together with diffused SSc were independent risk factors for the presence of ILD. And the score of giant capillaries were significantly lower in the patients with more severe ILD group [0.25 (0.94) vs. 0.00 (0.28), for the mild and severe ILD groups respectively, P=0.019]. There was no statistically significant difference between the two groups with respect to the scores of enlarged capillaries, hemorrhages, or disorganization of the vascular array. Conclusion: Capillary deletion and severe deformity in NC were associated with the presence of ILD in SSc patients. And patients with less giant capillaries had more severe ILD involvement. These indicated that NC maybe a useful tool to evaluate ILD in SSc.
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