ABC | Volume 111, Nº6, December 2018

Brief Communication Costa et al Erdheim-Chester’s Disease Arq Bras Cardiol. 2018; 111(6):852-855 1. Campochiaro C , Tomelleri A , Cavalos G , Berti A , Dagna L . Erdheim- Chester Disease. Eur J Intern Med. 2015;26(4):223-9. 2. Arnaud L, Gorochov G, Charlotte F, Lvoschi V, Parizot C, Larson M, et al. Systemic perturbation of cytokine and chemokine networks in Erdheim-Chester disease: a single-center series of 37 patients. Blood. 2011;117(10):2783-90. 3. Cangi MG, Biavasco R, Cavalli G, Gercessini G, Campochiaro C, Dal-Cin E, et al. BRAFV600E-mutation is invariably present and associated to oncogene-induced senescence in Erdheim-Chester disease. Ann Rheum Dis. 2015;74(8):1596-602. 4. Arnaud L, Hervier B, Nelly A, Neel A, Hamidou MA, Kahn JE, et al. CNS involvement and treatment with interferon- α are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients. Blood 2011;117(10):2778-82. 5. Roei D, Manevich-Mazor M, Shoenfeld Y. Erdheim-Chester Disease: a comprehensive review of the literature. Orphanet J Rare Dis. 2013 Sep; 8:137. 6. Diamond EL, Dagna L, Hyman DM, Cavalli G, Janku F, Estradas-Verces J, et al. Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease. Blood. 2014;124(4):483-92. 7. Haroche J, Arnaud L, Amoura Z, Cohen-Aulbart F, Hervier B, Charlotte F,et al. Erdheim–Chester Disease. Curr Rheumatol Rep. 2014;16(4):412. 8. Haroche J,AmouraZ,DionE,WechslerB,Costedoat-ChaleimewN,Cacoub B, et al. Cardiovascular involvement, an overlooked feature of Erdheim– Chester disease: report of 6 new cases and a literature review. Medicine (Baltimore). 2004;83(6):371– 92. 9. Raptis DA, Raptis CA, Jokerst C, Bhalla S: Erdheim-Chester Disease With Interatrial Septum Involvement. J Thorac Imaging. 2011;27(5):W105-W107. 10. Gianfreda D, Palumbo AA, Rossi E, Buttarelli L, Manari G, Martini C,et al. Cardiac involvement in Erdheim-Chester disease: an MRI study. Blood. 2016;128(20):2468-71. 11. Haroche J, Cluzel P, Toledano D, Montalescot G, Touitou D, Grenier PA, et al. Images in cardiovascular medicine. Cardiac involvement in Erdheim– Chester disease: magnetic resonance and computed tomographic scan imaging in a monocentric series of 37 patients. Circulation. 2009;119(25):e597–8. 12. Maurer AH, BurshteynM, Adler LP, Steiner RM. How todifferentiate benign versus malignant cardiac and paracardiac 18FDG uptake at oncologic PET/ CT. Radiographics. 2011;31(5):1287-305. 13. Pagé M, Quarto C, Mancuso E, Mohiaddin RH. Metabolically Active Brown Fat Mimicking Pericardial Metastasis on PET/CT: The Discriminating Role of Cardiac Magnetic Resonance Imaging. Can J Cardiol. 2016;32(8):1039.e15-7. References This is an open-access article distributed under the terms of the Creative Commons Attribution License (mean of 1.84). 12 The CMR is an important diagnostic tool in the differentiation of findings, since it can better characterize the tissues. The brown adipose tissue is characterized by hypersignal in T1-weighted images and intermediate signal intensity in T2-weighted images. Specific sequences used to suppress the fat signal, such as the triple inversion-recovery pulse sequence, allow for distinguishing between fatty lesions and other types of tissue. In another review of 53 patients, 4 17% had symptomatic valvular disease, mainly aortic and mitral regurgitation and 3 patients required valve replacement. Technically, surgical valve repair is difficult because of the infiltration of adjacent heart tissue. ECD is a disease with poor prognosis, with a mean 5-year survival of 68% 5 and of difficult diagnosis. The scarcity of patients contributes to the lack of knowledge about the disease and the difficulty to develop randomized studies. With this series of cases, we report the largest Brazilian case series to date, focusing on cardiovascular involvement, aiming to contribute to the knowledge of this rare and complex disease. Author contributions Conception and design of the research, acquisition of data and writing of the manuscript: Costa IBSS, Abdo ANR, Bittar CS, Fonseca SMR, Moraes ASHT, Kalil Filho R, Pereira J, Hajjar LA; analysis and interpretation of the data: Costa IBSS, Fonseca SMR, Moraes ASHT, Kalil Filho R, Pereira J, Hajjar LA; critical revision of the manuscript for intellectual contente: Pereira J, Hajjar LA. Potential Conflict of Interest No potential conflict of interest relevant to this article was reported. Sources of Funding There were no external funding sources for this study. Study Association This study is not associatedwith any thesis or dissertationwork. 855