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Pulmonary Vascular Disease |

A Rare Presentation of Venous Thromboembolic Disease in the Setting of Cardiopulmonary Sarcoidosis

Jay Patel, MD; Kunal Brahmbhatt, MD; Kumkum Patel, MD; Alzbeta Sykora, MD; Joseph Germano, DO; Girish Nair, MD
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Winthrop-University Hospital, Mineola, NY


Chest. 2014;146(4_MeetingAbstracts):889A. doi:10.1378/chest.1990109
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Abstract

SESSION TITLE: Pulmonary Vascular Disease Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Venous thromboembolic (VTE) disease is an extremely rare presentation of cardiopulmonary sarcoidosis. We present a case of acute VTE in a patient with suspected sarcoidosis and antiphospholipid syndrome (APS).

CASE PRESENTATION: A 44 year old African-American male was admitted with a chief complaint of worsening right-sided, pleuritic chest pain for 2 days, associated with progressive dyspnea on exertion for 2 months. The patient had no significant medical history except for family history of two maternal aunts with venous thromboembolic disease. Physical examination was unremarkable, and laboratory findings were significant for a D-dimer of 821ng/ml, activated partial thromboplastin time of 40.7sec, erythrocyte sedimentation rate of 59mm/hr, and an ACE level of 84U/L. CT angiogram of the chest revealed bilateral pulmonary emboli with left lower extremity deep vein thrombosis, and bilateral mediastinal and hilar adenopathy. Echocardiogram showed left ventricular ejection fraction to be 30-35%. A cardiac MRI showed linear enhancement of the left ventricular basal septal wall, consistent with cardiac sarcoidosis. An implantable cardioverter-defibrillator was placed for primary prevention against ventricular arrhythmias and sudden cardiac death. Hypercoagulable workup revealed positive dilute Russel Viper Venom Test (dRVVT), indicating the presence of lupus anticoagulant (LA), and corroborated by an abnormal mixing study. Patient was treated with enoxaparin and oral steroid therapy. Tissue biopsy of the adenopathy was deferred initially in the setting of acute thromboembolic disease.

DISCUSSION: This case contributes to the small body of evidence suggesting an association between cardiopulmonary sarcoidosis and a hypercoagulable state (1). The diagnosis of APS is based on clinical and laboratory criteria, which include the detection of LA (2). The dRVVT has a 89% sensitivity and 96% specificity for detecting LA (3). However, the definitive diagnosis will require persistent detection at least 12 weeks from the initial diagnosis.

CONCLUSIONS: Further studies are needed to evaluate the association between cardiopulmonary sarcodoisis and systemic thromboembolic disease.

Reference #1: Swigris JJ, Olson AL, Huie TJ, et al. Increased risk of pulmonary embolism among US decedents with sarcoidosis from 1988 to 2007. Chest. 2011;140(5):1261-66.

Reference #2: Myakis S, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4(2):295-306.

Reference #3: Tripodi A, et al. PM. Laboratory diagnosis of lupus anticoagulants for patients on oral anticoagulant treatment: performance of dilute Russell viper venom test and silica clotting time in comparison with Staclot LA. Thrombosis and Haemostasis. 2002;88(4):583-586.

DISCLOSURE: The following authors have nothing to disclose: Jay Patel, Kunal Brahmbhatt, Kumkum Patel, Alzbeta Sykora, Joseph Germano, Girish Nair

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