Abstract：
OBJECTIVE: The World Trade Center (WTC) attack in New York resulted in a dust plume containing silica, hydrocarbons, and asbestos. Autoimmune disorders have been reported among those with WTC site exposure. The characteristics of individuals developing systemic sclerosis (SSc) have not been previously described. The purpose of this study was to describe the features of patients with SSc with WTC exposure.
METHODS: Data were collected from 11 patients with SSc or SSc spectrum conditions who reported exposure to the WTC site. Seven patients completed an exposure assessment.
RESULTS: Of the 11 patients, the majority (n = 8) were female. The median (range) for age at diagnosis was 46 (36-75) years, time between exposure and first non-Raynaud phenomenon SSc symptom was 8 (1-19) years, and time between exposure and diagnosis was 11 (2-18) years. Fifty-five percent had SSc onset > 5 years from WTC exposure. Five patients had limited cutaneous SSc, 3 patients had diffuse cutaneous SSc, 1 patient with SSc features met criteria for mixed connective tissue disease (CTD), and 2 patients had undifferentiated CTD with features of SSc. Four patients had overlapping features with other CTDs. Interstitial lung disease (ILD) was present in 10 patients. Five of 11 patients had a history of tobacco use. Seven of 7 patients who completed the questionnaire reported other hazardous exposures outside of WTC. Of these, only 2 patients reported personal protective equipment use.
CONCLUSIONS: A high frequency of ILD and overlap features were observed among patients with SSc with WTC exposure. Future studies are needed to characterize this association.
METHODS: Data were collected from 11 patients with SSc or SSc spectrum conditions who reported exposure to the WTC site. Seven patients completed an exposure assessment.
RESULTS: Of the 11 patients, the majority (n = 8) were female. The median (range) for age at diagnosis was 46 (36-75) years, time between exposure and first non-Raynaud phenomenon SSc symptom was 8 (1-19) years, and time between exposure and diagnosis was 11 (2-18) years. Fifty-five percent had SSc onset > 5 years from WTC exposure. Five patients had limited cutaneous SSc, 3 patients had diffuse cutaneous SSc, 1 patient with SSc features met criteria for mixed connective tissue disease (CTD), and 2 patients had undifferentiated CTD with features of SSc. Four patients had overlapping features with other CTDs. Interstitial lung disease (ILD) was present in 10 patients. Five of 11 patients had a history of tobacco use. Seven of 7 patients who completed the questionnaire reported other hazardous exposures outside of WTC. Of these, only 2 patients reported personal protective equipment use.
CONCLUSIONS: A high frequency of ILD and overlap features were observed among patients with SSc with WTC exposure. Future studies are needed to characterize this association.
摘要：
目的：纽约世界贸易中心（WTC）的袭击导致了含二氧化硅的尘埃羽流，碳氢化合物，和石棉。已经报道了WTC位点暴露者中的自身免疫性疾病。先前尚未描述发展为系统性硬化症（SSc）的特征。这项研究的目的是描述WTC暴露的SSc患者的特征。
方法：数据来自11例报告暴露于WTC部位的SSc或SSc谱疾病患者。七个人完成了暴露评估。
结果：在11例患者中，大多数（n=8）是女性。诊断时年龄的中位数（范围）为46（36-75）岁，暴露与首次出现非雷诺现象SSc症状之间的时间为8（1-19）年，暴露和诊断之间的时间为11（2-18）年。55%的SSc在WTC暴露后发病>5年。五名患者皮肤SSc有限，三名患者有弥漫性皮肤SSc，一名具有SSc特征的患者符合混合性结缔组织疾病的标准，2例患者患有具有SSc特征的未分化结缔组织病.四名患者与其他结缔组织疾病有重叠特征。10例患者存在间质性肺病（ILD）。11例患者中有5例有烟草使用史。完成问卷的7名患者中有7名报告了WTC以外的其他危险暴露。在接受采访的7名患者中，只有2名报告使用了个人防护设备。
结论：在有WTC暴露的SSc患者中观察到ILD和重叠特征的频率较高。需要未来的研究来描述这种关联。
方法：数据来自11例报告暴露于WTC部位的SSc或SSc谱疾病患者。七个人完成了暴露评估。
结果：在11例患者中，大多数（n=8）是女性。诊断时年龄的中位数（范围）为46（36-75）岁，暴露与首次出现非雷诺现象SSc症状之间的时间为8（1-19）年，暴露和诊断之间的时间为11（2-18）年。55%的SSc在WTC暴露后发病>5年。五名患者皮肤SSc有限，三名患者有弥漫性皮肤SSc，一名具有SSc特征的患者符合混合性结缔组织疾病的标准，2例患者患有具有SSc特征的未分化结缔组织病.四名患者与其他结缔组织疾病有重叠特征。10例患者存在间质性肺病（ILD）。11例患者中有5例有烟草使用史。完成问卷的7名患者中有7名报告了WTC以外的其他危险暴露。在接受采访的7名患者中，只有2名报告使用了个人防护设备。
结论：在有WTC暴露的SSc患者中观察到ILD和重叠特征的频率较高。需要未来的研究来描述这种关联。
