G Sarlon1, C Durant, Y Grandgeorge, E Bernit, V Veit, M Hamidou, N Schleinitz, J-R Harlé. 1. Service de chirurgie et médecine vasculaire, faculté de médecine de Marseille, université de la méditerranée, hôpital La Timone, 264, rue Saint-Pierre, 13385 Marseille cedex 05, France. gabrielle.sarlon@mail.ap-hm.fr <gabrielle.sarlon@mail.ap-hm.fr>
Abstract
INTRODUCTION: Discordance exists between the results of post-mortem studies and the low number of clinical reported cases of cardiac involvements in Wegener's granulomatosis. CASE REPORTS: Data from four patients were studied retrospectively. Three patients had associated airway localization and three had kidney involvement. All patients had positive test for anti-PR3 antineutrophil antibodies. Two patients presented with dilated cardiomyopathy (one with terminal cardiac failure), another patient with complete atrioventricular block and pericarditis, and the remaining one with myopericarditis. One patient was asymptomatic. For three of these patients, the cardiac manifestations were contemporary of the diagnosis of Wegener's granulomatosis and had a severe disease course. CONCLUSION: Cardiac events in Wegener's granulomatosis are probably underestimated, given the various type of heart damage and the clinical presentation. Cardiac involvement seems to be associated with a poor prognosis. Thus, we recommend systematic and regular cardiac assessment in the follow-up of patients with Wegener's granulomatosis.
INTRODUCTION: Discordance exists between the results of post-mortem studies and the low number of clinical reported cases of cardiac involvements in Wegener's granulomatosis. CASE REPORTS: Data from four patients were studied retrospectively. Three patients had associated airway localization and three had kidney involvement. All patients had positive test for anti-PR3 antineutrophil antibodies. Two patients presented with dilated cardiomyopathy (one with terminal cardiac failure), another patient with complete atrioventricular block and pericarditis, and the remaining one with myopericarditis. One patient was asymptomatic. For three of these patients, the cardiac manifestations were contemporary of the diagnosis of Wegener's granulomatosis and had a severe disease course. CONCLUSION: Cardiac events in Wegener's granulomatosis are probably underestimated, given the various type of heart damage and the clinical presentation. Cardiac involvement seems to be associated with a poor prognosis. Thus, we recommend systematic and regular cardiac assessment in the follow-up of patients with Wegener's granulomatosis.
Authors: Laryssa Passos Sarmento Santos; Victor Guerreiro Bomfim; Camila Fagundes Bezerra; Natália Vieira Costa; Rafael Barreto Paes de Carvalho; Ricardo Sobral de Carvalho; Rogério da Hora Passos; Olivia Carla Bomfim Boaventura; André Luiz Nunes Gobatto Journal: Rev Bras Ter Intensiva Date: 2017 Jul-Sep