PICKERING SYNDROME Mandie DiMarco, Kristen Williams Jack Herbert, William Gibson MD, Domonique Smith MD, Brian Coe MD , Arash Ataei MD , Seth Vignes MD; Department of Medicine, LSU Health New Orleans, New Orleans, LA.
Introduction: Bilateral renal artery stenosis (BRAS) is seen often secondary to atherosclerosis in patients over 45 years old and fibromuscular dysplasia in women under 50 years old. A strong clinical 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 rise of arterial pressure leading to left ventricular failure. While the prevalence of PS is difficult to estimate, 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. The presence of RAS can be confirmed by imaging. Treatment options include medical therapy, renal stenting, or surgical revascularization. Case: A 77-year-old woman with COPD and left subclavian artery occlusion presented with progressive dyspnea for two days associated with bilateral lower extremity edema. She was afebrile, non-tachycardic, tachypneic to 38, blood pressure of 215/96 mmHg to right arm, and had an oxygen saturation of 77% on room air. Notably, her left arm blood pressure was dramatically lower; however, this was determined to be inaccurate due to her subclavian occlusion. A point-of-care bedside ultrasound demonstrated elevated jugular venous pressure, adequate cardiac contractility, and fluid in all lung fields. Initial labs demonstrated an elevated
BNP and minimally elevated high-sensitivity troponin. She was placed on CPAP and within minutes rapidly improved. Computed tomography angiography of her kidneys revealed near-total occlusion of the bilateral renal arteries. She clinically improved over a few days with diuresis and blood pressure control and was 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 alone was not inferior to renal artery stenting and had less adverse events. In contrast, several case cohort 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 hemodynamically significant RAS with associated recurrent unexplained pulmonary edema or congestive heart failure for percutaneous renal artery revascularization. Ultimately, a multidisciplinary team should be utilized to determine the most appropriate treatment plan for a patient with clinically significant RAS to determine procedural versus medical intervention on a case-by-case basis.
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