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Eosinophilic Granulomatosis With Polyangiitis Presenting as Pericardial Tamponade
Cardiovascular Disease SESSION TITLE: Heart Failure and Cardiogenic Shock SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM
Eosinophilic Granulomatosis With Polyangiitis Presenting as Pericardial Tamponade Jorge Sanchez* Loyola University Medical Center, Maywood, IL INTRODUCTION: Eosinophilic granulomatosis with polyangiitis (EGPA) classically presents with pulmonary manifestations and peripheral eosinophilia. However, cardiac involvment is increasingly recognized in anti-neutrophil cytoplasmic antibody (ANCA) negative disease, and is associated with significant morbidity and mortality. It is important to identify this condition early, even when it presents as cardiac disease, so as to avoid late diagnosis and delay in therapy.
CARDIOVASCULAR DISEASE
CASE PRESENTATION: CS is a 49 yr old female with history of asthma who presented with chest pain and exertional dyspnea. Initial labs demonstrated a peripheral eosinophilia of 30%. Cardiac imaging demonstrated a pericardial effusion with tamponade requiring evacuation. Chest computed tomography demonstrated pleural effusions and dense pulmonary infiltrates. Thoracentesis revealed an exudate with 55% eosinophils. ANCA testing was negative. The patient underwent bronchoscopy with bronchoalveolar lavage (BAL) which demonstrated 1,310 white bood cells with 74% eosinophils. Transbronchial biopsy confirmed the diagnosis by showing rare granulomas with eosinophilic debris and eosinophilic infiltration of small vessels. Treatment with glucocorticoids led to rapid improvement in her condition. DISCUSSION: EGPA is a mutlisystem disorder characterized by vasculitis of small and medium sized vessels. The classic presentation consists of transient or “migrating” pulmonary infiltrates in a person with history of asthma, sinusitis, and eosinophilia. Biopsy of an affected organ shows eosinophilic infiltration, vasculitis of small vessels, and necrotizing granulomas. The most common organs involved are lung and skin. When the heart is involved, the common manifestations are heart failure and cardiac rhythm abnormalities. Cardiac involvement accounts for approximately 50% of deaths attributed to EGPA, and is more frequent in patients with higher eosinophil counts and absent ANCAs.1 CONCLUSIONS: Cardiac involvment of EGPA leads to significant morbidity and mortality among patients with this condition. Despite atypical presentation, early recognition of EGPA can lead to timely diagnosis and insitution of life-saving therapy. Reference #1: Neumann T. et al. Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis. Medicine (Baltimore). 2009;88(4):236. DISCLOSURE: The following authors have nothing to disclose: Jorge Sanchez No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2017.08.120
Copyright ª 2017 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
90A
[
152#4S CHEST OCTOBER 2017
]
Eosinophilic Granulomatosis With Polyangiitis Presenting as Pericardial Tamponade Jorge Sanchez* Loyola University Medical Center, Maywood, IL INTRODUCTION: Eosinophilic granulomatosis with polyangiitis (EGPA) classically presents with pulmonary manifestations and peripheral eosinophilia. However, cardiac involvment is increasingly recognized in anti-neutrophil cytoplasmic antibody (ANCA) negative disease, and is associated with significant morbidity and mortality. It is important to identify this condition early, even when it presents as cardiac disease, so as to avoid late diagnosis and delay in therapy.
CARDIOVASCULAR DISEASE
CASE PRESENTATION: CS is a 49 yr old female with history of asthma who presented with chest pain and exertional dyspnea. Initial labs demonstrated a peripheral eosinophilia of 30%. Cardiac imaging demonstrated a pericardial effusion with tamponade requiring evacuation. Chest computed tomography demonstrated pleural effusions and dense pulmonary infiltrates. Thoracentesis revealed an exudate with 55% eosinophils. ANCA testing was negative. The patient underwent bronchoscopy with bronchoalveolar lavage (BAL) which demonstrated 1,310 white bood cells with 74% eosinophils. Transbronchial biopsy confirmed the diagnosis by showing rare granulomas with eosinophilic debris and eosinophilic infiltration of small vessels. Treatment with glucocorticoids led to rapid improvement in her condition. DISCUSSION: EGPA is a mutlisystem disorder characterized by vasculitis of small and medium sized vessels. The classic presentation consists of transient or “migrating” pulmonary infiltrates in a person with history of asthma, sinusitis, and eosinophilia. Biopsy of an affected organ shows eosinophilic infiltration, vasculitis of small vessels, and necrotizing granulomas. The most common organs involved are lung and skin. When the heart is involved, the common manifestations are heart failure and cardiac rhythm abnormalities. Cardiac involvement accounts for approximately 50% of deaths attributed to EGPA, and is more frequent in patients with higher eosinophil counts and absent ANCAs.1 CONCLUSIONS: Cardiac involvment of EGPA leads to significant morbidity and mortality among patients with this condition. Despite atypical presentation, early recognition of EGPA can lead to timely diagnosis and insitution of life-saving therapy. Reference #1: Neumann T. et al. Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis. Medicine (Baltimore). 2009;88(4):236. DISCLOSURE: The following authors have nothing to disclose: Jorge Sanchez No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2017.08.120
Copyright ª 2017 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
90A
[
152#4S CHEST OCTOBER 2017
]