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.
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。