ATS 2021 will feature presentations of original research from accepted abstracts. Mini Symposia and Thematic Poster Sessions are abstract based sessions.
Please use the form below to browse scientific abstracts and case reports accepted for ATS 2021. Abstracts presented at the ATS 2021 will be published in the Online Abstract Issue of the American Journal of Respiratory and Critical Care Medicine, Volume 203, May 3, 2021.
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Diagnostic Dilemma: EGPA Without Vasculitis on Biopsy?
Session Title
TP33 - TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE
Abstract
A2033 - Diagnostic Dilemma: EGPA Without Vasculitis on Biopsy?
Author Block: M. Ondeck1, S. Gayen2, B. Gupta3, M. Patel2, P. B. Desai2; 1Lewis Katz School of Medicine at Temple University, Philadelphia, PA, United States, 2Thoracic Medicine and Surgery, Temple University Hospital, Philadelphia, PA, United States, 3Internal Medicine, Temple University Hospital, Philadelphia, PA, United States.
Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis characterized by eosinophilia and asthma. We present a rare case of EGPA with cardiac involvement.
Case: A 46-year-old female with a history of hypertension, childhood asthma, allergic rhinitis, inferior ST-elevation myocardial infarction (STEMI) complicated by ventricular fibrillation arrest in the setting of coronary vasospasm presented with acute dyspnea and palpitations for one day. Review of systems was significant for sinus congestion (~1 year) and intermittent, bilateral petechiae on her shins. She also reported a new maintenance engineering job of 1 year. Initial laboratory testing was notable for peripheral eosinophilia (see Table 1), positive antinuclear antibody titer (1:160, speckled pattern), negative complements and ANCA, unremarkable peripheral smear, and normal tryptase and vitamin B12 levels. Physical examination was significant for wheezing and peripheral edema. A computed tomography angiogram of the thorax showed mild diffuse interlobular septal thickening, bronchial wall thickening, and diffuse 2-3 mm pulmonary nodules. Due to her eosinophilia and suspicion of EGPA, computed tomography of the sinuses was performed which revealed near complete opacification of all sinuses. She was started on a mometasone-formoterol inhaler, fluticasone nasal spray, and prednisone at 40 mg/day. Her peripheral eosinophilia decreased with prednisone administration (Table 1). On day 7 of steroids, she underwent a bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial biopsies. BAL cell count showed 22% eosinophils, and pathology revealed alveolated lung parenchyma with tissue eosinophilia. A diagnosis of EGPA was made based on the American College of Rheumatology diagnostic criteria. She was discharged on a prednisone taper with plans for initiation of mepolizumab, an IL-5 inhibitor.
Discussion: Hypereosinophilia (absolute eosinophil count > 1500 cells/microL) in a patient with worsening asthma symptoms, such as our patient, warrants further diagnostic evaluation. When hypereosinophilia is seen, evaluation of end-organ damage/involvement should occur. Our patient already had evidence of cardiac (recent STEMI without atherosclerosis), lung and sinus involvement based on her symptoms and imaging. ANCA negativity has been well known to occur with EGPA in the eosinophilic infiltration phase preceding the vasculitis phase. ANCA negativity has also been associated with increased cardiac involvement in EGPA. Our patient had cardiac involvement with no vasculitis, yet had significant tissue eosinophilia. Presence of systemic signs ruled out other entities such as eosinophilic pneumonia, allergic bronchopulmonary aspergillosis and aspirin-exacerbated respiratory disease. In conclusion, one should not overlook the diagnosis of EGPA with lack of ANCA and vasculitis.
Author Block: M. Ondeck1, S. Gayen2, B. Gupta3, M. Patel2, P. B. Desai2; 1Lewis Katz School of Medicine at Temple University, Philadelphia, PA, United States, 2Thoracic Medicine and Surgery, Temple University Hospital, Philadelphia, PA, United States, 3Internal Medicine, Temple University Hospital, Philadelphia, PA, United States.
Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis characterized by eosinophilia and asthma. We present a rare case of EGPA with cardiac involvement.
Case: A 46-year-old female with a history of hypertension, childhood asthma, allergic rhinitis, inferior ST-elevation myocardial infarction (STEMI) complicated by ventricular fibrillation arrest in the setting of coronary vasospasm presented with acute dyspnea and palpitations for one day. Review of systems was significant for sinus congestion (~1 year) and intermittent, bilateral petechiae on her shins. She also reported a new maintenance engineering job of 1 year. Initial laboratory testing was notable for peripheral eosinophilia (see Table 1), positive antinuclear antibody titer (1:160, speckled pattern), negative complements and ANCA, unremarkable peripheral smear, and normal tryptase and vitamin B12 levels. Physical examination was significant for wheezing and peripheral edema. A computed tomography angiogram of the thorax showed mild diffuse interlobular septal thickening, bronchial wall thickening, and diffuse 2-3 mm pulmonary nodules. Due to her eosinophilia and suspicion of EGPA, computed tomography of the sinuses was performed which revealed near complete opacification of all sinuses. She was started on a mometasone-formoterol inhaler, fluticasone nasal spray, and prednisone at 40 mg/day. Her peripheral eosinophilia decreased with prednisone administration (Table 1). On day 7 of steroids, she underwent a bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial biopsies. BAL cell count showed 22% eosinophils, and pathology revealed alveolated lung parenchyma with tissue eosinophilia. A diagnosis of EGPA was made based on the American College of Rheumatology diagnostic criteria. She was discharged on a prednisone taper with plans for initiation of mepolizumab, an IL-5 inhibitor.
Discussion: Hypereosinophilia (absolute eosinophil count > 1500 cells/microL) in a patient with worsening asthma symptoms, such as our patient, warrants further diagnostic evaluation. When hypereosinophilia is seen, evaluation of end-organ damage/involvement should occur. Our patient already had evidence of cardiac (recent STEMI without atherosclerosis), lung and sinus involvement based on her symptoms and imaging. ANCA negativity has been well known to occur with EGPA in the eosinophilic infiltration phase preceding the vasculitis phase. ANCA negativity has also been associated with increased cardiac involvement in EGPA. Our patient had cardiac involvement with no vasculitis, yet had significant tissue eosinophilia. Presence of systemic signs ruled out other entities such as eosinophilic pneumonia, allergic bronchopulmonary aspergillosis and aspirin-exacerbated respiratory disease. In conclusion, one should not overlook the diagnosis of EGPA with lack of ANCA and vasculitis.
