Churg-Strauss Syndrome Revealed by Acute Coronary Artery Disease: A Case Report*


Churg-Strauss syndrome (CSS) is a very rare small-vessel vasculitis. Clinical features include asthma, rhinitis and/or sinusitis, and peripheral eosinophilia. Although cardiac findings are observed in 50% of cases, coronary artery disease is rarely reported and even more rarely suggestive. The value of cardiac MRI for these patients is discussed here. A 52-year-old non-smoker male without family antecedents of cardiovascular disease presented with worsening of atypical asthma that developed 10 months earlier. A month before, he had been admitted to the ICU because of respiratory distress and cardiogenic shock with chest pain. The angiogram revealed stenosis of the three main coronary arteries requiring the placement of several stents. Follow-up cardiac assessments showed good results. General impairment, unstable asthma associated with rhinitis, and eosinophilia suggested a systemic disease. The diagnosis of CSS was established considering that five criteria of the American College of Rheumatology were found. Prednisolone was prescribed at 1 mg/kg/day, which completely suppressed all symptoms and eosinophilia. Cardiac MRI was performed two months later and revealed a good control of myocardial lesions characterized by fibrosis beneath the anterior endocardium and the median septum. Immunosuppressive treatment was then administered together with corticosteroid therapy. These results suggest that acute coronary artery disease can reveal CSS in some cases. Here, the patient’s cardiac assessment was normal apart from the acute episode, and cardiac MRI helped detect signs of myocarditis and establish a prognosis of CSS.

Share and Cite:

Estivals, M. , Périé, M. , Colombier, D. and Farah, B. (2013) Churg-Strauss Syndrome Revealed by Acute Coronary Artery Disease: A Case Report*. Open Journal of Respiratory Diseases, 3, 39-43. doi: 10.4236/ojrd.2013.32007.

Conflicts of Interest

The authors declare no conflicts of interest.


[1] J. Churg and L. Strauss, “Allergic Granulomatosis, Allergic Angiitis and Periarteritis Nodosa,” American Journal of Pathology, Vol. 27, No. 2, 1951, pp. 277-301.
[2] J. C. Lanham, K. B. Elkon, C. D. Pusey and G. R. Hughes, “Systemic Vasculitis with Asthma and Eosinophilia: A Clinical Approach to the Churg-Strauss Syndrome,” Medicine (Baltimore), Vol. 63, 1984, pp. 65-81. doi:10.1097/00005792-198403000-00001
[3] C. Le Gall, S. Pham, S. Vignes, G. Garcia, H. Nunes, D. Fichet, G. Simonneau, P. Duroux and M. Humbert, “Inhaled Corticosteroids and Churg-Strauss Syndrome: A Report of Five Cases,” European Respiratory Journal, Vol. 15, No. 5, 2000, pp. 978-981. doi:10.1034/j.1399-3003.2000.15e29.x
[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 and L. Guillevin, “Antineutrophil Cytoplasmic Antibodies and the Churg-Strauss Syndrome,” Annals of Internal Medicine, Vol. 143, No. 9. 2005, pp. 632-638. doi:10.7326/0003-4819-143-9-200511010-00006
[5] T. Neumann, B. Manger, M. Schmid, C. Kroegel, A. Hansch, W. A. Kaiser, D. Reinhardt, G. Wolf, G. Hein, G. Mall, G. Schett and J. Zwerina, “Cardiac Involvement in Churg-Strauss Syndrome: Impact of Endomyocarditis,” Medicine (Baltimore), Vol. 88, No. 4, 2009, pp. 236-243. doi:10.1097/MD.0b013e3181af35a5
[6] S. Hellemans, J. Dens and D. Knockaert, “Coronary Involvement in the Churg-Strauss Syndrome,” Heart, Vol. 77, 1997, pp. 576-578.
[7] A. D. Wagner, G. P. Meyer, M. Rihl, A. Rathmann, U. Wittkop, H. Zeidler, H. Haller and J. Lotz, “Acute Coronary Syndrome Associated with Churg-Strauss Syndrome,” Vascular Health and Risk Management, Vol. 3, No. 5, 2007, pp. 775-779.
[8] C. R. McGavin, A. J. Marshall and C. T. Lewis, “Churg-Strauss Syndrome with Critical Endomyocardial Fibrosis: 10 Years Survival after Combined Surgical and Medical Management,” Heart, Vol. 87, No. 5, 2002, p. e5. doi:10.1136/heart.87.5.e5
[9] P. Petrakopoulou, W. M. Franz, P. Boekstegers and M. Weis, “Vasospastic Angina Pectoris Associated with Churg-Strauss Syndrome,” Nature Clinical Practice Cardiovascular Medicine, Vol. 2, 2005, pp. 484-489. doi:10.1038/ncpcardio0299
[10] N. Kakouros, R. Bastiaenen, A. Kourliouros and L. Anderson, “Churg-Strauss Presenting as Acute Coronary Syndrome: Sometimes It’s Zebras,” BMJ Case Reports, 2011.
[11] S. E. Petersen, A. Kardos and S. Neubauer, “Subendocardial and Papillary Muscle Involvement in a Patient with Churg-Strauss Syndrome, Detected by Contrast Enhanced Cardiovascular Magnetic Resonance,” Heart, Vol. 91, No. 1, 2005, p. e9. doi:10.1136/hrt.2004.050070
[12] J. Marmursztejn, O. Vignaux, P. Cohen, P. Guilpain, C. Pagnoux, H. Gouya, L. Mouthon, P. Legmann, D. Duboc and 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, No. 1, Suppl. 52, 2009, pp. S70-S76.
[13] L. Guillevin, F. Lhote, M. Gayraud, P. Cohen, B. Jarrousse, O. Lortholary, N. Thibult and P. Casassus, “Prognostic Factors in Polyarteritis Nodosa and Churg-Strauss Syndrome. A Prospective Study in 342 Patients,” Medicine (Baltimore), Vol. 75, No. 1, 1996, pp. 17-28. doi:10.1097/00005792-199601000-00003
[14] L. Guillevin, C. Pagnoux, R. Seror, A. Mahr, L. Mouthon and P. Le Toumelin, “The Five Factors Score Revisited: Assessment of Prognosis of Systemic Necrotizing Vasculitidies Based on the French Vasculitis Study Group (FVSG),” Medicine (Baltimore), Vol. 90, No. 1, 2011, pp. 19-27. doi:10.1097/MD.0b013e318205a4c6
[15] M. Tanaka, N. Mise, N. Kurita, T. Suzuki, K. Hara, A. Fujii, N. Uesugi and T. Sugimoto, “A Case of Churg-Strauss Syndrome with Necrotizing Crescentric Glomerulonephritis Accompanied by Acute Coronary Syndrome due to Vasospasm,” American Journal of Kidney Diseases, Vol. 56, No. 2, 2010, pp. e5-e9. doi:10.1053/j.ajkd.2010.03.011
[16] B. Dunogué, C. Pagnoux and L. Guillevin, “Churg-Stauss Syndrome (CSS): Clinical Symptoms, Complementary Investigations, Prognosis and Outcome, and Treatment,” Seminars in Respiratory and Critical Care Medicine, Vol. 32, No. 3, 2011, pp. 298-309. doi:10.1055/s-0031-1279826

Copyright © 2022 by authors and Scientific Research Publishing Inc.

Creative Commons License

This work and the related PDF file are licensed under a Creative Commons Attribution 4.0 International License.