Diffuse Lung Disease: Sarcoidosis |

Clinical Characteristics of Sarcoidosis in World Trade Center (WTC) Exposed Fire Department of the City of New York (FDNY) Firefighters FREE TO VIEW

Kerry Hena, MD; Jennifer Yip, MPH; Nadia Jaber, PA-C; David Goldfarb, BA; Kelly Fullam, BA; Krystal Cleven, MD; Vasilios Christodoulou; William Moir, MPH; Zachary Hena, MD; Tesha Crosse, MPH; Rachel Zeig-Owens, DrPH; Mayris Webber, DrPH; Steven Plotycia, MD; David Gritz, MD; Daniel Spevack, MD; Israa Soghier, MD; David Prezant, MD; Thomas Aldrich, MD
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Montefiore Medical Center, Bronx, NY

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):514A. doi:10.1016/j.chest.2016.08.528
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SESSION TITLE: Sarcoidosis

SESSION TYPE: Original Investigation Slide

PRESENTED ON: Wednesday, October 26, 2016 at 07:30 AM - 08:30 AM

PURPOSE: The etiology of sarcoidosis is unknown, but probably involves genetic predisposition and environmental triggers. Inhalation of particulates from the WTC collapse on 9/11/2001 may be one such trigger. Characterization of the nature and course of this sarcoid variant, with known trigger and onset dates, may shed light on the natural history of sarcoidosis in general.

METHODS: Between 2001 and 2014, we identified 75 WTC-exposed FDNY firefighters with newly-diagnosed post-9/11 sarcoidosis through medical records review. We are now undertaking in-depth reassessments via family, occupational, and smoking histories; questionnaires regarding organ involvement and treatment courses; and detailed physical and ophthalmologic exams, skin surveys, PFTs, 48-hour ECGs, chest CTs, cardiac MRIs, echocardiograms; and assessments of calcium homeostasis and beryllium-sensitivity of lymphocytes. Specimens are saved for future genetic analyses. As of 3/21, we have studied 42 subjects.

RESULTS: The majority are white (93%) and arrived at the WTC site on 9/11 (69%). All are male. Median age at diagnosis was 44. In initial exams, 97% had intrathoracic involvement (nodes &/or lungs) at diagnosis, and some had involvement of joints (16%), eyes (5%), skin (8%), brain (1%) and heart (1%). Among the 42 with follow-up exams, 88% are never-smokers. None reports sarcoidosis family history. Previous or current involvement is self-reported in lungs 79%, nodes 55%, joints 24%, eyes 12%, skin 7%, and cardiac involvement in none. Of 13 2015-16 chest CTs performed to date, 6 show resolution of intrathoracic sarcoid. Current PFTs show 7 (17%) with mild obstruction, 2 (5%) with mild restriction, and 1 (2%) with isolated mild impairment of diffusing capacity. Joint symptoms persist in 8 (19%), and skin manifestations in 1 (2%). MRIs show evidence of cardiac sarcoidosis in 4 (10%), of whom 1 required a pacemaker/ICD. None has hypercalcemia, but 6 (14%) have mildly elevated urinary calcium/creatinine ratios. 29 (69%) have low 25-OH vitaminD, but 1,25 di-OH vitaminD is elevated in only 2 and low in none. ACE is elevated in 7 (17%). None has abnormal lymphocyte sensitivity to beryllium. Current medications for sarcoidosis include steroids for 24 (57%), and 7 (17%) with joint involvement have required immuno-modulators and biologics.

CONCLUSIONS: In this ongoing reassessment of sarcoidosis in WTC-exposed firefighters, preliminary results show that pulmonary/nodal involvement was initially most common, but has declined in prevalence, and few have impaired PFTs. Joint symptoms persist in about a fifth and most require more than hydroxychloroquine or corticosteroids. We found minimal abnormalities of vitamin D/calcium homeostasis, and no evidence of active ocular sarcoidosis. Previously unrecognized cardiac involvement is present in a larger number than expected. The absence of beryllium-sensitivity supports a conclusive diagnosis of sarcoidosis, by excluding beryllium-associated granulomatous inflammation.

CLINICAL IMPLICATIONS: In WTC-exposed firefighters, pulmonary manifestations of sarcoidosis predominated at diagnosis, but almost a decade later, rheumatologic and cardiac issues present the greatest challenge.

DISCLOSURE: The following authors have nothing to disclose: Kerry Hena, Jennifer Yip, Nadia Jaber, David Goldfarb, Kelly Fullam, Krystal Cleven, Vasilios Christodoulou, William Moir, Zachary Hena, Tesha Crosse, Rachel Zeig-Owens, Mayris Webber, Steven Plotycia, David Gritz, Daniel Spevack, Israa Soghier, David Prezant, Thomas Aldrich

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