Journal of Cardiovascular Imaging

Korean Society of Echocardiography (한국심초음파학회)
권호 표지
DOI QR코드

DOI QR Code

CARDIAC INVOLVEMENT OF CHURG-STRAUSS SYNDROME AS A REVERSIBLE CAUSE OF DILATED CARDIOMYOPATHY

  • JEONG, HAE CHANG (DEPARTMENT OF CARDIOVASCULAR MEDICINE, CHONNAM NATIONAL UNIVERSITY HOSPITAL) ;
  • KIM, KYE HUN (DEPARTMENT OF CARDIOVASCULAR MEDICINE, CHONNAM NATIONAL UNIVERSITY HOSPITAL) ;
  • CHO, JAE YEONG (DEPARTMENT OF CARDIOVASCULAR MEDICINE, CHONNAM NATIONAL UNIVERSITY HOSPITAL) ;
  • SONG, JI EUN (DEPARTMENT OF CARDIOVASCULAR MEDICINE, CHONNAM NATIONAL UNIVERSITY HOSPITAL) ;
  • YOON, HYUN JU (DEPARTMENT OF CARDIOVASCULAR MEDICINE, CHONNAM NATIONAL UNIVERSITY HOSPITAL) ;
  • SEON, HYUN JU (DEPARTMENT OF RADIOLOGY, CHONNAM NATIONAL UNIVERSITY HOSPITAL) ;
  • AHN, YOUNGKEUN (DEPARTMENT OF CARDIOVASCULAR MEDICINE, CHONNAM NATIONAL UNIVERSITY HOSPITAL) ;
  • JEONG, MYUNG HO (DEPARTMENT OF CARDIOVASCULAR MEDICINE, CHONNAM NATIONAL UNIVERSITY HOSPITAL) ;
  • CHO, JEONG GWAN (DEPARTMENT OF CARDIOVASCULAR MEDICINE, CHONNAM NATIONAL UNIVERSITY HOSPITAL) ;
  • PARK, JONG CHUN (DEPARTMENT OF CARDIOVASCULAR MEDICINE, CHONNAM NATIONAL UNIVERSITY HOSPITAL)
  • Received : 2014.07.24
  • Accepted : 2015.02.27
  • Published : 2015.03.27
⟨ Previous Next ⟩

Abstract

A 31-year-old male who had been treated for Churg-Strauss syndrome (CSS) presented with sudden onset of dysarthria. Brain magnetic resonance imaging (MRI) showed acute multifocal bilateral cerebral infarctions suggesting embolic causes. Cardiac MRI showed dilated cardiomyopathy with severe biventricular dysfunction with intracardiac thrombi, and multiple high signal intensity spots in myocardium of the left ventricle with multifocal delayed enhancement suggesting multifocal myocarditis due to small vessel vasculitis associated with CSS. After anticoagulation therapy, treatments for heart failure, and immunosuppressive therapy including parenteral steroids and cyclophosphamide to control CSS, the symptoms and signs of heart failure and cardiac function of the patient were improved. Considering the pathophysiologic mechanism of cardiac involvement in CSS, immunosuppressive therapy to control the disease activity of CSS should be taken into account, besides usual management for heart failure.

Keywords

Acknowledgement

Grant : 분자의과학 융합사업단

References

  1. Grau RG. Churg-Strauss syndrome: 2005-2008 update. Curr Rheumatol Rep 2008;10:453-8. https://doi.org/10.1007/s11926-008-0074-x
  2. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, et al. The American College of Rheumatology 1990 criteria for the classification of Churg- Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33:1094-100.
  3. Solans R, Bosch JA, Perez-Bocanegra C, Selva A, Huguet P, Alijotas J, Orriols R, Armadans L, Vilardell M. Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients. Rheumatology (Oxford) 2001;40:763-71. https://doi.org/10.1093/rheumatology/40.7.763
  4. Aakeroy L, Amundsen BH, Skomsvoll JF, Haugen BO, Soma J. A 50-year-old man with eosinophilia and cardiomyopathy: need for endomyocardial biopsy? Eur J Echocardiogr 2011;12:257-9. https://doi.org/10.1093/ejechocard/jeq167
  5. Seo JS, Song JM, Kim DH, Kang DH, Song JK. A Case of Loeffler's Endocarditis Associated with Churg-Strauss Syndrome. J Cardiovasc Ultrasound 2010;18:21-4. https://doi.org/10.4250/jcu.2010.18.1.21
  6. Wolf J, Bergner R, Mutallib S, Buggle F, Grau AJ. Neurologic complications of Churg-Strauss syndrome--a prospective monocentric study. Eur J Neurol 2010;17:582-8. https://doi.org/10.1111/j.1468-1331.2009.02902.x
  7. Abu-Shakra M, Smythe H, Lewtas J, Badley E, Weber D, Keystone E. Outcome of polyarteritis nodosa and Churg-Strauss syndrome. An analysis of twenty-five patients. Arthritis Rheum 1994;37:1798-803. https://doi.org/10.1002/art.1780371214
  8. Finsterer J. Neurological manifestations of Churg-Strauss syndrome. Eur J Neurol 2010;17:524-5. https://doi.org/10.1111/j.1468-1331.2008.02903.x
  9. Dennert RM, van Paassen P, Schalla S, Kuznetsova T, Alzand BS, Staessen JA, Velthuis S, Crijns HJ, Tervaert JW, Heymans S. Cardiac involvement in Churg-Strauss syndrome. Arthritis Rheum 2010;62:627-34.
  10. Szczeklik W, Miszalski-Jamka T, Mastalerz L, Sokolowska B, Dropinski J, Banys R, Hor KN, Mazur W, Musial J. Multimodality assessment of cardiac involvement in Churg-Strauss syndrome patients in clinical remission. Circ J 2011;75:649-55. https://doi.org/10.1253/circj.CJ-10-0772
  11. Marmursztejn J, Vignaux O, Cohen P, Guilpain P, Pagnoux C, Gouya H, Mouthon L, Legmann P, Duboc D, Guillevin L. Impact of cardiac magnetic resonance imaging for assessment of Churg-Strauss syndrome:a cross-sectional study in 20 patients. Clin Exp Rheumatol 2009;27(1 Suppl 52):S70-6.
  12. Mavrogeni S, Manoussakis MN, Karagiorga TC, Douskou M, Panagiotakos D, Bournia V, Cokkinos DV, Moutsopoulos HM. Detection of coronary artery lesions and myocardial necrosis by magnetic resonance in systemic necrotizing vasculitides. Arthritis Rheum 2009;61:1121-9. https://doi.org/10.1002/art.24695
  13. Sliwa K, Damasceno A, Mayosi BM. Epidemiology and etiology of cardiomyopathy in Africa. Circulation 2005;112:3577-83. https://doi.org/10.1161/CIRCULATIONAHA.105.542894
  14. Park JH, Kwon DH, Starling RC, Marwick TH. Role of imaging in the detection of reversible cardiomyopathy. J Cardiovasc Ultrasound 2013;21:45-55. https://doi.org/10.4250/jcu.2013.21.2.45
  15. Gharib MI, Burnett AK. Chemotherapy-induced cardiotoxicity: current practice and prospects of prophylaxis. Eur J Heart Fail 2002;4:235-42. https://doi.org/10.1016/S1388-9842(01)00201-X

Cited by

  1. Systemic autoimmunity induced by the TLR7/8 agonist Resiquimod causes myocarditis and dilated cardiomyopathy in a new mouse model of autoimmune heart disease vol.10, pp.3, 2017, https://doi.org/10.1242/dmm.027409
  2. Angina in granulomatosis with polyangiitis: A case report vol.14, pp.4, 2015, https://doi.org/10.3892/etm.2017.4946
  3. A pediatric case of eosinophilic granulomatosis with polyangiitis accompanied by heart failure mimicking an asthma attack vol.7, pp.4, 2015, https://doi.org/10.4168/aard.2019.7.4.212