Cardiac Tamponade: An Uncommon Presentation of Granulomatosis With Polyangiitis

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM INTRODUCTION: Cardiac tamponade is a life-threatening condition requiring pericardial drainage to restore hemodynamic balance. We describe a case of tamponade as the presenting manifestation of granulomatosis with polyangiitis (GPA). CASE PRESENTATION: A 58 year-old farmer acutely developed chest pain and dyspnea. He was tachycardic, tachypneic and diaphoretic. He described a productive cough, recurrent sinusitis, malaise, polyarthralgias, night sweats and a 50 pound weight loss. On examination he appeared ill. Jugular venous pulsations were elevated, heart tones were distant and trace pretibial pitting edema was present. Labs demonstrated leukocytosis, anemia, thrombocytosis and normal renal function. Troponin-T was negative. Electrocardiogram revealed sinus tachycardia. Computed tomography ruled out pulmonary embolism but showed a pericardial effusion [image 1] with pericardial enhancement. Irregular nodules were noted in the right lung [image 2], as well as a left pleural effusion and mediastinal lymphadenopathy. Echocardiogram revealed right ventricular and atrial collapse and respirophasic flow reversal in the inferior vena cava, consistent with tamponade. Pericardiocentesis yielded 500 mL of sero-sanguineous fluid. Further studies revealed microscopic hematuria, erythrocyte sedimentation rate of 130 mm/hr, C-reactive protein of 203 mg/L, cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA) titer of 1:256 and markedly elevated proteinase (PR-3). Pericardial cytology and cultures were negative. Treatment for GPA was initiated with prednisone and cyclophosphamide, in addition to colchicine and indomethacin. Four months after diagnosis he is in remission. DISCUSSION: The reported frequency of cardiac involvement with GPA ranges from 6-44% (1); the largest GPA case series found a 10% overall incidence of pericarditis (2). To our knowledge this is the first description of tamponade leading to the diagnosis of GPA, though four cases of tamponade in the setting of established GPA have been previously described. This patient was successfully treated with pericardiocentesis and immunosuppression. CONCLUSIONS: Pericarditis is not uncommon in GPA and can rarely result in tamponade. Reference #1: Grant et al. Wegener's granulomatosis and the heart. Br Heart J. 1994 Jan;71(1):82-6. Reference #2: Hoffman et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992 Mar 15;116(6):488-98. Reference #3: Schiavone et al. Unusual cardiac complications of Wegener's granulomatosis. Chest. 1985 Nov;88(5):745-8. DISCLOSURE: The following authors have nothing to disclose: Erin Sullivan, Rodrigo Cartin-Ceba, Fabien Maldonado No Product/Research Disclosure Information