Kerry M Hena1, Jennifer Yip2, Nadia Jaber2, David Goldfarb2, Kelly Fullam2, Krystal Cleven3, William Moir2, Rachel Zeig-Owens2, Mayris P Webber2, Daniel M Spevack4, Marc A Judson5, Lisa Maier6, Andrew Krumerman4, Anthony Aizer7, Simon D Spivack3, Jessica Berman8, Thomas K Aldrich3, David J Prezant9. 1. Pulmonary & Critical Care Division, Department of Medicine, NYU School of Medicine, New York, NY. 2. Bureau of Health Services, Fire Department of the City of New York, Brooklyn, NY. 3. Pulmonary Division, Department of Medicine, Montefiore Medical Center and the Albert Einstein College of Medicine, Bronx, NY. 4. Cardiology Division, Department of Medicine, Montefiore Medical Center and the Albert Einstein College of Medicine, Bronx, NY. 5. Pulmonary & Critical Care Division, Albany Medical College, Albany, NY. 6. Division of Environmental and Occupational Health Sciences, Department of Medicine, National Jewish Health, Denver, CO. 7. Cardiology Division, Department of Medicine, NYU School of Medicine, New York, NY. 8. Rheumatology Division, Hospital for Special Surgery, New York, NY. 9. Bureau of Health Services, Fire Department of the City of New York, Brooklyn, NY; Pulmonary Division, Department of Medicine, Montefiore Medical Center and the Albert Einstein College of Medicine, Bronx, NY. Electronic address: david.prezant@fdny.nyc.gov.
Abstract
BACKGROUND: Sarcoidosis is believed to represent a genetically primed, abnormal immune response to an antigen exposure or inflammatory trigger, with both genetic and environmental factors playing a role in disease onset and phenotypic expression. In a population of firefighters with post-World Trade Center (WTC) 9/11/2001 (9/11) sarcoidosis, we have a unique opportunity to describe the clinical course of incident sarcoidosis during the 15 years postexposure and, on average, 8 years following diagnosis. METHODS: Among the WTC-exposed cohort, 74 firefighters with post-9/11 sarcoidosis were identified through medical records review. A total of 59 were enrolled in follow-up studies. For each participant, the World Association of Sarcoidosis and Other Granulomatous Diseases organ assessment tool was used to categorize the sarcoidosis involvement of each organ system at time of diagnosis and at follow-up. RESULTS: The incidence of sarcoidosis post-9/11 was 25 per 100,000. Radiographic resolution of intrathoracic involvement occurred in 24 (45%) subjects. Lung function for nearly all subjects was within normal limits. Extrathoracic involvement increased, most prominently joints (15%) and cardiac (16%) involvement. There was no evidence of calcium dysmetabolism. Few subjects had ocular (5%) or skin (2%) involvement, and none had beryllium sensitization. Most (76%) subjects did not receive any treatment. CONCLUSIONS: Extrathoracic disease was more prevalent in WTC-related sarcoidosis than reported for patients with sarcoidosis 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 ECG, 48-h 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.
BACKGROUND:Sarcoidosis is believed to represent a genetically primed, abnormal immune response to an antigen exposure or inflammatory trigger, with both genetic and environmental factors playing a role in disease onset and phenotypic expression. In a population of firefighters with post-World Trade Center (WTC) 9/11/2001 (9/11) sarcoidosis, we have a unique opportunity to describe the clinical course of incident sarcoidosis during the 15 years postexposure and, on average, 8 years following diagnosis. METHODS: Among the WTC-exposed cohort, 74 firefighters with post-9/11 sarcoidosis were identified through medical records review. A total of 59 were enrolled in follow-up studies. For each participant, the World Association of Sarcoidosis and Other Granulomatous Diseases organ assessment tool was used to categorize the sarcoidosis involvement of each organ system at time of diagnosis and at follow-up. RESULTS: The incidence of sarcoidosis post-9/11 was 25 per 100,000. Radiographic resolution of intrathoracic involvement occurred in 24 (45%) subjects. Lung function for nearly all subjects was within normal limits. Extrathoracic involvement increased, most prominently joints (15%) and cardiac (16%) involvement. There was no evidence of calciumdysmetabolism. Few subjects had ocular (5%) or skin (2%) involvement, and none had beryllium sensitization. Most (76%) subjects did not receive any treatment. CONCLUSIONS: Extrathoracic disease was more prevalent in WTC-related sarcoidosis than reported for patients with sarcoidosis without WTC exposure or for other exposure-related granulomatous diseases (beryllium disease and hypersensitivitypneumonitis). Cardiac involvement would have been missed if evaluation stopped after ECG, 48-h 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.
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