ACCELERATED DEVELOPMENT OF PULMONARY ARTERIAL HYPERTENSION IN THE SETTING OF ABATACEPT THERAPY

Abstract

SESSION TITLE: Pulmonary Vascular Disease SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/22/2019 3:45 PM - 4:45 PM INTRODUCTION: Pulmonary arterial hypertension (PAH) is a rare and debilitating disease, commonly related to connective tissue disease (CTD), idiopathic or hereditary in nature or due to certain drugs. New medications have been acknowledged as being a risk factor for developing PAH.There are case reports of some biologic drugs as a possible risk factor. Abatacept is a chimeric protein that inhibits T-lymphocyte activation and is used as a biologic drug to treat autoimmune diseases. We present a patient with PAH and acceleration of symptoms after initiation of Abatacept for underlying autoimmune disease. CASE PRESENTATION: We present a 46-year-old woman with mixed connective tissue disease and rheumatoid arthritis for 5 years, who presented with six weeks of rapidly progressed dyspnea on exertion, orthopnea and pedal edema. She had been evaluated 2 months prior, while on Infliximab, for mild intermittent dyspnea for the past 6 months and noted to have normal pulmonary function and echocardiogram. She was subsequently switched to Abatacept due to a intolerance with Infliximab. A month after initiation of this medication her dyspnea worsen and she presented 10 weeks after with severe dyspnea. Echocardiogram revealed moderate right ventricle (RV) dilation and estimated pulmonary artery systolic pressure (PASP) of 93 mmHg and right atrial pressure (RAP) of 15 mmHg. Pulmonary embolism was ruled out. Right heart catheterization (RHC) measured a mean PAP of 50 mmHg and PVR of 20 Woods units. PAH was diagnosed with WHO functional class 3. Abatacept was discontinued as a potential risk factor that accelerated symptoms.Combination therapy was initiated with Tadalafil, Ambrisentan and Furosemide, and her WHO functional class improved to 1. Repeat echocardiogram at 6 months showed PASP: 35 mmHg, RAP: 3 mmHg with normal RV size and function and markedly improved six-minute walk distance. DISCUSSION: PAH is an uncommon debilitating disease with nonspecific symptoms, making early diagnosis challenging. This makes identification of risk factors for development and progression of PAH crucial. CTD is a well-established risk factor as are certain medications. With the growing use of biologic drugs for CTD therapy, these drugs as risk factors for PAH might grow as well. To the best of our knowledge this is the second case reported with Abatacept potentially accelerating PAH symptoms in the setting of treatment for CTD. CONCLUSIONS: In an era of increasing biologic agents use in patients with CTD, who are already at risk for PAH, physicians need to be aware of this potential association. Reference #1: Promislow, S. et al. Pulmonary arterial hypertension associated with abatacept treatment for rheumatoid arthritis: A case report. Canadian Journal of Respiratory, Critical Care, and Sleep Medicine. 2018 Reference #2: Gibbson, E. et al Reversible pulmonary arterial hypertension associated with interferon-beta treatment for multiple sclerosis. Can Respir J. 2015 DISCLOSURES: No relevant relationships by Selvin Jacob, source=Web Response No relevant relationships by Ginny Marmolejos, source=Web Response No relevant relationships by Kartik Ramakrishna, source=Web Response