Clinical Characteristics and Outcomes of WTC-Associated Sarcoidosis
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2017/09/28
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Series: Grant Final Reports
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Description:Background: Large numbers of new diagnoses of sarcoidosis (or a granulomatous disorder indistinguishable from sarcoidosis, referred to in this document as World Trade Center (WTC)-associated sarcoidosis) have been demonstrated in all of the major cohorts tracking post-WTC conditions, suggesting that WTC exposure may have played an etiologic role. In particular, there have been 74 new cases in Fire Department of the City of New York (FDNY) firefighters exposed to WTC These cases, many of which arguably were triggered by WTC exposure, provided a unique opportunity to explore the clinical characteristics, genetic correlates, temporal patterns of progression or resolution, and response to treatments of WTC associated sarcoidosis. The two specific aims of this proposal were to: 1) Characterize the current and past clinical profiles of sarcoidosis among FDNY firefighters who developed sarcoidosis after WTC-exposure, and 2) Compare the frequency of known genetic markers associated with sarcoidosis in WTC-associated sarcoidosis with controls who did not have sarcoidosis. Methods: For Aim 1 we characterized organ involvement the 15 organs or systems using an organ assessment tool adapted from the WASOG (World Association of Sarcoidosis and other Granulomatous Diseases) Organ assessment instrument and the response to treatment. We also assessed the lung function of among the patients at diagnosis and follow-up. For Aim 2, we conducted a case-control study with controls matched for race, age, smoking history, and WTC-exposure history. Genetic markers included: HLA alleles, BTNL2 haplotype, Annexin A11 single nucleotide polymorphisms and beryllium-responsive CD4 T cells were also be examined. Results: In Aim 1 we found radiographic resolution of intrathoracic involvement occurred in 24 (45%). Lung function for nearly all was within normal limits. Extrathoracic involvement increased, most prominently joints (15%) and cardiac (16%). There was no evidence for calcium dysmetabolism. Few had ocular (5%) or skin (2%) involvement. None had beryllium sensitization. Most (76%) did not receive any treatment. Extrathoracic disease was more prevalent in WTC-related sarcoidosis than reported for sarcoidosis patients without WTC-exposure or for other exposure-related granulomatous diseases (beryllium disease and hypersensitivity pneumonitis). Cardiac involvement would have been missed if evaluation stopped after electrocardiogram, 48-hour recordings and echocardiogram. Our results also support the need for advanced cardiac screening in asymptomatic patients with strenuous, stressful, public safety occupations given the potential fatality of a missed diagnosis. In Aim 2 we found that all samples showed no evidence for beryllium-responsive CD4 T cells. Blood samples with matching controls have been obtained for the genetic markers analyses and are currently being processed. Conclusions: The results from this proposal show how WTC-exposed rescue/recovery workers with sarcoidosis should be clinically evaluated at diagnosis and during follow-up to maximize worker health and safety. Our study also demonstrates no evidence for beryllium as the cause for the sarcoid granulomatous inflammatory response after WTC-exposure. We are currently doing further genetic variant analyses to determine if: (i) There are predisposing inherited variants for sarcoid in our population, and (ii) There are any genetic x environmental interactions that might explain our findings. [Description provided by NIOSH]
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Pages in Document:1-24
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NIOSHTIC Number:nn:20053012
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NTIS Accession Number:PB2019-100184
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Citation:Atlanta, GA: U.S. Department of Health and Human Services, Public Health Service, Centers for Disease Control and Prevention, National Institute for Occupational Safety and Health, U01-OH-010993, 2017 Sep; :1-24
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Contact Point Address:Simon Spivack, MD, MPH, Albert Einstein College of Medicine, Michael F. Price Center, 1301 Morris Park Avenue, Room 301, Bronx, NY 10461
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Federal Fiscal Year:2017
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Performing Organization:Albert Einstein College of Medicine, Bronx, New York
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Peer Reviewed:False
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Start Date:20150701
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Source Full Name:National Institute for Occupational Safety and Health
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End Date:20170630
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Main Document Checksum:urn:sha-512:95a452226bde51792ae8dbe7c43f24cc5c66b84615f04644a62bee12015bf47b5c6b681be1d384f5f1702ee5cf6e5ed3f54f2c14591f8d1763098679d5aa529b
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