Clinical Characteristics of Connective Tissue Disease-Associated Interstitial Lung Disease in 1,044 Chinese Patients
Clinical Characteristics of Connective Tissue Disease-Associated Interstitial Lung Disease in 1,044 Chinese Patients
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1,044 名中国结缔组织病相关间质性肺病患者的临床特征
DOI：
10.1378/chest.15-1145
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发表时间：
2016-01-01
期刊：
影响因子：
9.6
通讯作者：
Li, Hui-Ping
中科院分区：
文献类型：
--
作者：
Hu, Yang;Wang, Liu-Sheng;Li, Hui-Ping
背景技术背景：由于结缔组织病（CTD）相关性间质性肺疾病（ILD; CTD-ILD）在中国的患病率尚不清楚，我们希望分析中国患者的临床特征。方法：回顾1999年1月至2013年1月在上海市肺科医院接受ILD诊断和治疗的患者的病历。根据这些记录，确定了同时接受CTD诊断的患者，并回顾了截至2013年12月底至少12个月的随访检查记录。结果：在2，678例接受ILD诊断的患者中，1，798例（67%）被确定为CTD-ILD; 299例（11.2%）患有特发性肺纤维化（IPF）。获得了1，044例CTD-ILD患者和178例IPF患者的完整临床数据。我们发现，1,044例CTD-ILD患者中有332例（32%）在首次入院时未得到准确诊断，195（18.7%）的1,044例患者显示自身抗体持续阴性检测结果，在1,044例患者中，25.1%的患者在首次入院时自身抗体为阴性，然后在随访检查时变为阳性。在288例确诊CTD-ILD的患者中，41例（14%）以肺部症状为初始临床表现（PSIM），247例（86%）以肺外症状为初始临床表现（EPSIM）。在756例未分化CTD-ILD患者中，PSIM和EPSIM的比例分别为44%和56%。在PSIM患者中，23例确诊CTD-ILD（56%）和216例未确诊CTD-ILD（65%）患者在初次就诊时未得到准确诊断，但在随后的随访检查中最终得到诊断。结缔组织疾病患者-ILD在首次入院时未得到准确诊断，可能是因为自身抗体的血清学检测结果为阴性，没有明显的肺外症状。因此，ILD患者应检查肺外症状，并在随访检查时检测自身抗体。
BACKGROUND: Because the prevalence of connective tissue disease (CTD)-associated interstitial lung disease (ILD; CTD-ILD) in China is unknown, we wanted to analyze the clinical characteristics of this disease in Chinese patients.METHODS: The medical records of patients who received a diagnosis of ILD and treated in Shanghai Pulmonary Hospital from January 1999 to January 2013 were reviewed. Based on the records, patients who also received a diagnosis of CTD were identified, and their records of follow-up examinations for a minimum of 12 months until the end of December 2013 were reviewed.RESULTS: Of the 2,678 patients who received a diagnosis of ILD, 1,798 (67%) were identified as having CTD-ILD; 299 (11.2%) had idiopathic pulmonary fibrosis (IPF). Complete clinical data were available for 1,044 patients with CTD-ILD and 178 with IPF. We found that 332 of the 1,044 patients with CTD-ILD (32%) did not receive an accurate diagnosis at the initial hospital admission, 195 (18.7%) of the 1,044 patients showed persistent negative test results for autoantibodies, and 262 (25.1%) of the 1,044 patients had negative autoantibodies at the initial hospital admission and then became positive at follow-up examinations. Of the 288 patients who had confirmed CTD-ILD, 41 (14%) showed pulmonary symptoms as the initial clinical manifestation (PSIM) and 247 (86%) showed extrapulmonary symptoms as the initial clinical manifestation (EPSIM). For the 756 patients who had undifferentiated CTD-ILD, the proportion of PSIM and EPSIM was 44% and 56%, respectively. For patients who presented with PSIM, 23 who had confirmed CTD-ILD (56%) and 216 who had unconfirmed CTD-ILD (65%) did not receive an accurate diagnosis at the initial visit but were ultimately diagnosed at subsequent follow-up examinations.CONCLUSIONS: Patients with CTD-ILD do not receive an accurate diagnosis at the initial hospital admission possibly because of negative serologic test results for autoantibodies and the absence of obvious extrapulmonary symptoms. Thus, patients with ILD should be examined for extrapulmonary symptoms and tested for autoantibodies at follow-up examinations.