Faculty Bibliography

INTRODUCTION: The management of eosinophilic-granulomatosis with polyangiitis (EGPA) with diffuse alveolar hemorrhage (DAH) in patients who have failed first line therapy can be challenging. We present a case of refractory EGPA with DAH and hypoxic respiratory failure that improved with plasma exchange. CASE PRESENTATION: A 75-year-old woman with EGPA with renal and lung involvement presented with hemoptysis. She had multiple EGPA flares in the past and had failed first-line therapy with cyclophosphamide and azathioprine. CT chest was significant for areas of extensive bronchiectasis with areas of fibrosis, as well as new areas of ground glass opacifications (see Fig. 1, A & B). She developed worsening hypoxic respiratory failure requiring intubation. Bronchoscopy with broncheoalveolar lavage was consistent with DAH (see Fig. 1, C). Despite being started on methylprednisolone and antibiotics, she continued to have worsening hypoxia and renal failure. Plasma exchange was initiated with significant improvement in hypoxia. After five sessions of plasma exchange, she had significant clinical improvement in her renal failure and resolution of hypoxic respiratory failure. DISCUSSION: There is a paucity of data available to guide the management of EGPA with DAH and hypoxic respiratory failure in patients who have failed first line therapies. Plasma exchange has been used in glomerulonephritis associated with EGPA with limited results1. Case reports identify a benefit of plasma exchange for treatment of corticosteroid-refractory EGPA with gastrointestinal manifestations2; however, there is no data investigating the role of plasma exchange in the management of pulmonary manifestations of EGPA. From these studies, one can only extrapolate that plasma exchange would treat pulmonary manifestations of EGPA, as was demonstrated in our patient. CONCLUSIONS: The management of hypoxic respiratory failure secondary to EGPA associated DAH is challenging. Treatment options for those that fail conventional therapies are lacking; however, the role of plasma exchange as a new treatment modality is promising

OBJECTIVE: To determine whether lung function trajectories after 9/11/2001 (9/11) differed by sex or race/ethnicity in World Trade Center-exposed Fire Department of the City of New York emergency medical service (EMS) workers. METHOD: Serial cross-sectional study of pulmonary function tests (PFTs) taken between 9/11 and 9/10/2015. We used data from routine PFTs (forced expiratory volume in 1 s (FEV1) and FEV1% predicted), conducted at 12-18 month intervals. FEV1 and FEV1% predicted were assessed over time, stratified by sex, and race/ethnicity. We also assessed FEV1 and FEV1% predicted in current, former and never-smokers. RESULTS: Among 1817 EMS workers, 334 (18.4%) were women, 979 (53.9%) self-identified as white and 939 (51.6%) were never-smokers. The median follow-up was 13.1 years (IQR 10.5-13.6), and the median number of PFTs per person was 11 (IQR 7-13). After large declines associated with 9/11, there was no discernible recovery in lung function. In analyses limited to never-smokers, the trajectory of decline in adjusted FEV1 and FEV1% predicted was relatively parallel for men and women in the 3 racial/ethnic groups. Similarly, small differences in FEV1 annual decline between groups were not clinically meaningful. Analyses including ever-smokers were essentially the same. CONCLUSIONS: 14 years after 9/11, most EMS workers continued to demonstrate a lack of lung function recovery. The trajectories of lung function decline, however, were parallel by sex and by race/ethnicity. These findings support the use of routine, serial measures of lung function over time in first responders and demonstrate no sex or racial sensitivity to exposure-related lung function decline.