PICKERING SYNDROME. Ross Dies, Kristen Williams, Mandie DiMarco, Jack Hebert, William Gibson, Domonique Smith, Brian Coe, Arash Ataei, Seth Vignes, Louisiana State University, New Orleans, LA.
Introduction: Bilateral renal artery stenosis (BRAS) is often secondary to atherosclerosis in patients over 45 years of age and fibromuscular dysplasia in women under 50 years of age. A strong predictor for renovascular disease is rapidly developing severe hypertension. Pickering Syndrome (PS) is diagnosed in the setting of BRAS with flash pulmonary edema from rapid arterial pressure rise leading to left ventricular failure. The prevalence of PS is difficult to estimate, but renal artery stenosis (RAS) has a prevalence of less than 1% for patients with mild hypertension and up to 40% for more severe cases. Less than half of these patients have BRAS. Treatment options include medical therapy, renal stenting, or surgical revascularization. Case: A 77-year-old woman with chronic obstructive pulmonary disease and left subclavian artery occlusion presented with acute dyspnea and bilateral lower extremity edema. She was afebrile, non-tachycardic, tachypneic to 38 bpm, blood pressure of 215/96 mmHg (right arm), and had an oxygen saturation of 77% on room air. Notably, her left arm blood pressure was lower though inaccurate due to subclavian occlusion. A point- of-care ultrasound demonstrated elevated jugular venous pressure, adequate cardiac contractility, and
fluid in the lung fields. Initial labs demonstrated elevated B-type Natriuretic Peptide and minimally elevated troponin. She was placed on continuous positive airway pressure with symptomatic improvement. A computed tomography angiography of kidneys revealed near-total occlusion of bilateral renal arteries. She improved over a few days with diuresis and blood pressure control and discharged with a plan for renal artery stenting. Discussion: This case demonstrates the importance of considering renal pathology when determining the cause of recurrent flash pulmonary edema. PS should be considered in patients with resistant hypertension manifesting recurrent pulmonary edema with preserved left ventricular function. The CORAL and ASTRIAL trials demonstrated that medical therapy was not inferior to renal artery stenting. Several studies have shown that up to 75% of patients who underwent revascularization did not have further episodes of pulmonary edema. Notably, the ACC/AHA guidelines demonstrate a class I indication for stenting in patients with significant RAS and recurrent unexplained pulmonary edema. Ultimately, a multidisciplinary team should determine the most appropriate treatment plan for patients with significant RAS.
SYSTEMIC LUPUS ERYTHEMATOSUS PRESENTING WITH SEVERE MIXED AUTO-IMMUNE HEMOLYTIC ANEMIA Deekshitha Manney, Jorge Martinez Bencosme, Nisheem Pokharel; St Francis Medical Center, Monroe, LA.
Introduction: Autoimmune hemolytic anemia (AIHA) is a rare and acquired autoantibody mediated destruction of red blood cells (RBCs). AIHA can be categorized as warm, cold and mixed based on the temperature dependent activity of autoantibodies. Warm AIHA is mediated by IgG while cold AIHA is mediated by complement component c3d (cold agglutinins). Mixed AIHA has features of both and is a very rare presentation, usually developing later on in the disease process of autoimmune conditions such as systemic lupus erythematosus (SLE).
one day. On examination, she was tachycardic, pale and had axillary lymphadenopathy. Initial laboratory investigations revealed low hemoglobin and hematocrit, with 28.24% reticulocytes (N<2.5%). Further labs revealed an elevated total bilirubin, high LDH and low haptoglobin necessitating a hemolytic work up. Her antibody screen came back positive for IgG and C3d, and patient’s blood agglutinated at room temperature suggesting the presence of both warm and cold hemolysis. Concurrent autoimmune work up showed positive ANA with positive anti-smith antibodies and low complement levels meeting the clinical and immunological criteria for the diagnosis of SLE. Lymph node biopsy ruled 8
Case: A 38-year-old female with no past medical history presented with dyspnea and dizziness of
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