Systemic sclerosis in individuals with exposure to World Trade Center ground-zero rescue/recovery efforts: A case series.

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 those developing systemic sclerosis (SSc) have not been previously described. The purpose of this study was to describe the features of SSc patients with WTC exposure. Data was collected from 11 patients with SSc or SSc-spectrum conditions who reported exposure to the WTC site. Seven completed an exposure assessment. Of the 11 patients, the majority (n=8) were female. The medians (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. 55% had SSc onset > 5 years from WTC exposure. Five patients had limited cutaneous SSc, three patients had diffuse cutaneous SSc, one patient with SSc features met criteria for mixed connective tissue disease, two patients had undifferentiated connective tissue disease with features of SSc. Four patients had overlapping features with other connective tissue diseases. Interstitial lung disease (ILD) was present in ten 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. Only two of seven of the interviewed patients reported personal protective equipment use. A high frequency of ILD and overlap features were observed among SSc patients with WTC exposure. Future studies are needed to characterize this association.