cough, chest or abdominal pain, dysuria, or new skin rashes or lesions. He has chronically loose stool but has not had a worsening in the past weeks. He does report missing a 12mg dose of prednisone the day prior to this presentation. He denies recent travel history or exposure to sick contacts. He was febrile to 104 degrees Fahrenheit, and tachycardia to 110 bpm, but otherwise stable vitals and not requiring oxygen. Admission labs show neutrophil- predominant leukocytosis to 13.8, chronic macrocytic anemia with hemoglobin of 7.7, and electrolytes within normal limits. The initial procalcitonin level was 0.2 but rose to 3.2 after six hours. A chest radiograph shows mild cardiomegaly with stable pulmonary granulomas without new infiltrates or consolidations. A urinalysis shows no pyuria with negative leukocyte esterase and nitrites. Influenza
and Covid PCR were negative. Blood and urine cultures were collected, with empiric vancomycin and cefepime started and prednisone held. Discussion: Patients with VEXAS syndrome typically develop multi-systemic inflammatory and hematologic signs and symptoms, including fever, chondritis, arthritis, skin rashes, medium-vessel vasculitis and pulmonary infiltrate among others. While there is no specific treatment yet developed for VEXAS, steroids and other immunosuppressants are often used to help prevent and quell flare-ups, which increases patients' risk for infection. Under these circumstances, it is critical to investigate infectious versus autoimmune etiologies when patients with VEXAS present with new-onset fever.
THE CASE FOR ESSENTIAL HYPERTENSION Morgan McCoy, Jorge Martinez, Lee Engel; LSUHSC School of Medicine, New Orleans, LA.
Introduction: Subclavian Steal Syndrome (SSS) is a reversal of flow in the vertebral artery, caused by significant occlusion of the ipsilateral subclavian vein. Aortic stenosis (AS) is a valvular disease that obstructs the left ventricular outflow tract, resulting in a fixed cardiac output. Though both conditions are well studied individually, their co-existence and subsequent management remains sparsely documented. Case: An 82-year-old woman with coronary artery disease, multiple cardiac stents, type II diabetes mellitus, hypertension, hyperlipidemia, first degree AV block, chronic kidney disease, peripheral artery disease s/p bilateral carotid artery stent placement, left subclavian artery stent placement, and bilateral renal artery stent placement, presented to the Emergency Department complaining of a two-day history of mid-sternal chest pressure, diaphoresis, weakness, shortness of breath, and near syncope. On exam, her blood pressure was 201/101 mm/ hg, and she had a 3/6 systolic ejection murmur at the 2nd right intercostal space, consistent with aortic stenosis. A triage electrocardiogram revealed first degree AV block and left ventricular hypertrophy, but no evidence of acute cardiac ischemia. A transthoracic echocardiogram the following day revealed an ejection fraction of 70-75% with only moderate aortic stenosis. Her symptoms persisted and left heart catheterization
did not demonstrate significant stenosis of coronary arteries but revealed a completely occluded left subclavian vein. Her left ventricular pressure was 244/14 mmHg, and her central aortic pressure was 217/58 mmHg. The gradient across the aortic valve was 27mmHg. Finally, a carotid duplex bilateral ultrasound found bidirectional flow in her left vertebral artery, indicating subclavian steal syndrome (SSS). Cardiology recommended a goal range systolic blood pressure of 100-160 mmHg, to be measured in the right arm only. Treatment options were aimed at addressing the three most likely causes of her symptoms: AS, SSS, and hypertension while maintaining adequate perfusion. Discussion: This case illustrates just how difficult it may be to manage concomitant AS and SSS, resulting in chronic, multi-system compensation. With central and peripheral circulation in a tenuous balance, hypertension has proven, in this case, to be essential. We chose to respect the balance that had been struck and treat her symptomatically and her hypertension in accordance with Cardiology’s recommendations.
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