Patient with Churg Strauss Syndrome and Myocarditis Treated with Cyclophosphamide

Liam Mullen, Janice Harper, Sukumaran Binukrishnan

Royal Liverpool University Hospital, Liverpool Heart and Chest Hospital, Liverpool, United Kingdom.

DOI: 10.4236/ojneph.2012.23006                

Abstract

Cardiac involvement in Churg Strauss Syndrome is common and a poor prognostic indicator. Myocarditis in Churg Strauss Syndrome can present in different ways. It has been shown that basic cardiac investigations including echocardiography can be normal even in symptomatic patients. More recently cardiac magnetic resonance imaging (MRI) has been shown to be more sensitive in its diagnosis. Our case report describes a 45 year old male who presented with palpitations and breathlessness. Echocardiography was normal but cardiac MRI demonstrated abnormalities consistent with Myocarditis. He was treated with Cyclophosphamide and follow up MRI imaging demonstrated complete resolution of these abnormalities which was accompanied by resolution of symptoms. This case therefore supports the use of cardiac MRI in Churg Strauss Syndrome as a sensitive diagnostic tool in and as a means of monitoring response to therapy. It also supports the therapeutic effectiveness of Cyclophosphamide therapy in Churg Strauss related Myocarditis, something that has yet to be assessed on a large scale.

Keywords

Cardiac MRI; Cyclophosphamide; Myocarditis; Churg Strauss Syndrome

Conflicts of Interest

The authors declare no conflicts of interest.

References

[1]	R. M. Dennert, et al., “Cardiac Involvement in Churg-Strauss Syndrome,” Arthritis and Rheumatism, Vol. 62, No. 2, 2010, pp. 627-634.
[2]	G. Pela, G. Tirabassi, P. Pattoneri, L. Pavone, G. Garini, G. Bruschi, “Cardiac Involvement in the Churg-Strauss Syndrome,” American Journal of Cardiology, Vol. 97, No. 10, 2006, pp. 1519-1524.
[3]	J. Vinit, et al., “Heart Involvement in Churg-Strauss Syndrome: Retrospective Study in French Burgundy Population in Past 10 years,” European Journal of Internal Medicine, Vol. 21, No. 4, 2010, pp. 341-346.
[4]	R. Sablé-Fourtassou, P. Cohen, A. Mahr, C. Pagnoux, L. Mouthon, D. Jayne, D. Blockmans, J. F. Cordier, P. Delaval, X. Puechal, D. Lauque, J. F. Viallard, A. Zoulim, L. Guillevin, “The French Vasculitis Study Group. Antineutrophil Cytoplasmic Antibodies and the Churg-Strauss Syndrome,” Annals of Internal Medicine, Vol. 143, No. 9, 2005, pp. 632-638.
[5]	J. Marmursztejn, O. Vignaux, P. Cohen, P. Guilpain, C. Pagnoux, H. Gouya, L. Mouthon, P. Legmann, D. Duboc, L. Guillevin, “Impact of Cardiac Magnetic Resonance Imaging for Assessment of Churg-Strauss Syndrome: A Cross-Sectional Study in 20 Patients,” Clinical and Experimental Rheumatology, Vol. 27, Suppl. 52, 2009, pp. S70-S76.
[6]	H. Mahrholdt, C. Goedecke, A. Wagner, et al., “Cardiovascular Magnetic Resonance Assessment of Human Myocarditis: A Comparison to Histology and Molecular Pathology,” Circulation, Vol. 109, No. 10, 2004, pp. 1250-1258.
[7]	H. Baccouche, A. Yilmaz, D. Alscher, K. Klingel, J. F. Val-Bernal, H. Mahrholdt, “Images in Cardiovascular Medicine. Magnetic Resonance Assessment and Therapy Monitoring of Cardiac Involvement in Churg-Strauss Syndrome,” Circulation, Vol. 117, No. 13, 2008, pp. 1745-1749.
[8]	J. Marmursztejn, P. Cohen, D. Duboc, C. Pagnoux, L. Mouthon, P. Guilpain, P. Legmann, L. Guillevin, O. Vignaux, “Cardiac Magnetic Resonance Imaging in Churg-Strauss-Syndrome. Impact of Immunosuppressants on Outcome Assessed in a Prospective Study on 8 Patients,” Clinical and Experimental Rheumatology, Vol. 28, Suppl. 57, 2010, pp. 8-13.
[9]	R. M. Dennert, et al., “Cardiac Involvement in Churg-Strauss Syndrome,” Arthritis and Rheumatism, Vol. 62, No. 2, 2010, pp. 627-634.
[10]	G. Pela, G. Tirabassi, P. Pattoneri, L. Pavone, G. Garini, G. Bruschi, “Cardiac Involvement in the Churg-Strauss Syndrome,” American Journal of Cardiology, Vol. 97, No. 10, 2006, pp. 1519-1524.
[11]	J. Vinit, et al., “Heart Involvement in Churg-Strauss Syndrome: Retrospective Study in French Burgundy Population in Past 10 years,” European Journal of Internal Medicine, Vol. 21, No. 4, 2010, pp. 341-346.
[12]	R. Sablé-Fourtassou, P. Cohen, A. Mahr, C. Pagnoux, L. Mouthon, D. Jayne, D. Blockmans, J. F. Cordier, P. Delaval, X. Puechal, D. Lauque, J. F. Viallard, A. Zoulim, L. Guillevin, “The French Vasculitis Study Group. Antineutrophil Cytoplasmic Antibodies and the Churg-Strauss Syndrome,” Annals of Internal Medicine, Vol. 143, No. 9, 2005, pp. 632-638.
[13]	J. Marmursztejn, O. Vignaux, P. Cohen, P. Guilpain, C. Pagnoux, H. Gouya, L. Mouthon, P. Legmann, D. Duboc, L. Guillevin, “Impact of Cardiac Magnetic Resonance Imaging for Assessment of Churg-Strauss Syndrome: A Cross-Sectional Study in 20 Patients,” Clinical and Experimental Rheumatology, Vol. 27, Suppl. 52, 2009, pp. S70-S76.
[14]	H. Mahrholdt, C. Goedecke, A. Wagner, et al., “Cardiovascular Magnetic Resonance Assessment of Human Myocarditis: A Comparison to Histology and Molecular Pathology,” Circulation, Vol. 109, No. 10, 2004, pp. 1250-1258.
[15]	H. Baccouche, A. Yilmaz, D. Alscher, K. Klingel, J. F. Val-Bernal, H. Mahrholdt, “Images in Cardiovascular Medicine. Magnetic Resonance Assessment and Therapy Monitoring of Cardiac Involvement in Churg-Strauss Syndrome,” Circulation, Vol. 117, No. 13, 2008, pp. 1745-1749.
[16]	J. Marmursztejn, P. Cohen, D. Duboc, C. Pagnoux, L. Mouthon, P. Guilpain, P. Legmann, L. Guillevin, O. Vignaux, “Cardiac Magnetic Resonance Imaging in Churg-Strauss-Syndrome. Impact of Immunosuppressants on Outcome Assessed in a Prospective Study on 8 Patients,” Clinical and Experimental Rheumatology, Vol. 28, Suppl. 57, 2010, pp. 8-13.