Syncope in Behçet Disease: Is It a Pulmonary Embolism?

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 4 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: The purpose of this case report is to highlight the unusual pulmonary findings in Behcet’s Disease. This presentation also showcases the challenge in treating pulmonary embolism in patients with an underlying vasculitis secondary to autoimmune disease. CASE PRESENTATION: A 34 year old female presented with a chief complaint of recurrent abdominal pain. She had a past medical history of Behcet’s disease with multiple abdominal symptoms. Past surgical history included an appendectomy and cholecystectomy. Abdominal x-ray was negative. The patient was evaluated by pain management and started on a dilaudid PCA along with her home azathioprine and prednisone. Three days into her hospital stay, the patient syncopized in the bathroom. Vitals at the time showed a BP of 70/40 with sinus rhythm. CT Head was negative for an acute bleed. She continued to have rising troponins, peaking at 1.68. Transthoracic echocardiogram showed reduced RV systolic function and RA and RV dilation. D-dimer was elevated. Chest CTA showed bilateral segmental pulmonary emboli. Lower extremity DVT studies were negative. She was started on a heparin drip and transferred to the ICU. Her hypercoagulable workup resulted negative. The patient was bridged to xarelto and immunosuppressive agents were continued with significant improvement in clinical status. DISCUSSION: This case highlights an unprovoked massive pulmonary embolism in a young patient with Behcet’s Disease (BD). BD is an autoimmune disease commonly presenting with recurrent oral and genital ulcers. The etiology of this disease is not well-understood and it is thought vascular involvement is due to inflammation localized to the vasa vasorum. Thrombosis is usually secondary to underlying vasculitis, with lower extremity venous thromboembolism occurring most commonly. Pulmonary artery involvement has a prevalence rate of less than 5%, presenting as pulmonary artery aneurysm and less likely as pulmonary artery thrombosis. In most patients, pulmonary artery embolism is isolated and does not occur in concordance with lower extremity venous thrombosis. Pulmonary artery embolism is considered one of the worst prognostic manifestations of BD. In 2012, a retrospective cohort of 807 patients with BD and venous thrombosis showed that immunosuppressive agents significantly reduced the risk of venous thrombosis relapse rate. CONCLUSIONS: This case highlights the importance of possessing high clinical suspicion and knowledge of vascular involvement in BD patients presenting with new onset fever, dyspnea, hemoptysis or syncope. It is also highlights the complex management of these patients and the ongoing challenge to prevent recurrent vascular complications. Reference #1: Desbois AC; Wechsler B, Cluzel P, Helft G, Boutin D, Piette JC, Cacoub P, Saadoun D; Cardiovascular Involvement in Behcet’s Disease. Rev Med Interne. 2014 Feb; 35 (2): 103-11. Epub 2014 Jan 13. DISCLOSURE: The following authors have nothing to disclose: Aiza Tariq, Hailey Gupta, Alfredo Astua No Product/Research Disclosure Information