SILENT CORONARY VASCULITIS PRECEDING FULMINANT ANTI-NEUTROPHILIC CYTOPLASMIC ANTIBODY-ASSOCIATED PULMONARY-RENAL SYNDROME: A RARE DIAGNOSTIC AND THERAPEUTIC DILEMMA

Abstract

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Various forms of vasculitides ranging from benign and self-limiting to potentially life-threatening exist. Here, we report a case of a patient who harbored an asymptomatic coronary vasculitis followed by a life threatening pulmonary-renal syndrome (PRS) secondary to anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV). CASE PRESENTATION: 75-year-old male presented to us with hemoptysis. Four months prior to current admission, he reported dyspnea upon exertion and was diagnosed with atrial fibrillation with rapid ventricular response for the first time. A chest x-ray was unremarkable whereas an echocardiogram revealed worsening of his aortic stenosis compared to the prior year. Elevated troponin and ST-T wave changes in EKG prompted a left heart catheterization that revealed multivessel coronary artery disease necessitating consideration of coronary artery bypass graft (CABG), surgical aortic valve replacement, and a maze procedure. During the procedures, his left atrial appendage was excised and sent for histopathological examination (HPE). Post CABG, on a routine outpatient follow up, he was found to have hyperkalemia with a potassium of 6.0 mmol/L and acute renal failure with a creatinine of 6 mg/dL (baseline creatinine of 1.0 mg/dl), prompting a second hospitalization. Bilateral renal ultrasound were unremarkable. Serum myeloperoxidase anti-neutrophilic cytoplasmic antibodies (MPO-ANCA) were elevated. Renal biopsy revealed a severe vasculitis of small vessels without any granulomas. No immune deposits were noted. He was diagnosed with AAV and was treated medically. Later in the course, he was also started on hemodialysis. Four months after the onset of his initial dyspnea patient presented with frank hemoptysis leading to a third hospitalization. CT of the chest with contrast revealed bilateral new extensive mixed ground-glass and consolidative opacities. He had developed severe PRS. Patient failed to improve with medical management; decided to undergo comfort care measures and passed away the same day. Retrospective chart review of the patient's left atrial appendage biopsy revealed vasculitis involving medium-sized myocardial and pericardial blood vessels. DISCUSSION: In this case, coronary vasculitis was a clinically silent disease with a histopathologic diagnosis whereas PRS secondary to AAV was a fulminant vasculitis with a fatal course despite therapy with pulse steroids, cyclophosphamide, plasmapheresis and hemodialysis. It is unclear whether coronary vasculitis in the patient was a prodrome to an impending serious illness or merely an incidental finding with no association to subsequent AAV. CONCLUSIONS: Vasculitis of the coronary arteries is uncommon, yet when involved in a patient portends a poor prognosis. While immunosuppression and plasma exchange therapy remain the cornerstone in management of AAV, therapeutic dilemma persists with regard to the clinically silent coronary vasculitis. DISCLOSURES: No relevant relationships by Ismael ABU LIMON, source=Web Response Speaker/Speaker's Bureau relationship with Otsuka America Pharmaceutical, Inc Please note: $1001 - $5000 Added 05/24/2020 by Arvind Garg, source=Web Response, value=Honoraria Consultant relationship with Fresenius Kidney Care Please note: $1001 - $5000 Added 05/24/2020 by Arvind Garg, source=Web Response, value=Honoraria no disclosure on file for Odalys Estefania Lara-Garcia; No relevant relationships by Ruby Maini, source=Web Response No relevant relationships by Priyanka Parajuli, source=Web Response No relevant relationships by Manjari Regmi, source=Web Response