Summary
Churg–Strauss syndrome (CSS) is a rare necrotizing, systemic vasculitis that is almost invariably associated with bronchial asthma. Although overall prognosis is good and treatment with corticosteroids alone or in combination with other immunosuppressive agents is typically successful, there are reports of patients that do not show signs of clinical improvement under the usual pharmacotherapy. Small clinical studies suggested that cardiac or gastrointestinal involvement is associated with an adverse prognosis.
We here report the case of a 38 year old male patient with a history of bronchial asthma who was admitted to our hospital for further evaluation of progressive dyspnea. Blood eosinophilia, infiltrates of both lungs, signs of necrosis and eosinophil deposits on myocardial biopsy combined with a history of bronchial asthma established the diagnosis of CSS with cardiac involvement. We initiated an immunosuppressive therapy with prednisone and methotrexate. Upon tapering of the dosage of prednisone, we noticed worsening of symptoms and further deterioration of cardiac function. Despite the addition of cyclophosphamide and adjustment of heart failure medication, we were not able to stabilize the cardiac situation. Due to rapid progressive eosinophilic cardiomyopathy associated with CSS refractory to medical therapy, our patient was placed on the urgent heart transplantation waiting list and, in the meantime, has undergone successful cardiac transplantation.
Similar content being viewed by others
References
Watts RA, Lane SE, Bentham G et al (2000) Epidemiology of systemic vasculitis: a ten year study in the United Kingdom. Arthritis Rheum 43:414– 419
Noth I, Strek ME, Leff AR (2003) Churg–Strauss syndrome. Lancet 361:587–594
Masi AT, Hunder GG, Lie JT (1990) The American College of Rheumatology 1990 criteria for the classification of Churg–Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 33:1094–1100
Fauci A, Katz P, Barton H et al (1971) Cyclophosphamide therapy of severe necrotizing vasculitis. NEJM 301:235– 238
Guillevin L, Cohen P, Gayraud M et al (1999) Churg–Strauss syndrome: clinical study and long term follow up of 96 patients. Medicine 78:26–37
Solans R, Bosch JA, Perez–Bocanegra C et al (2001) Churg–Strauss syndrome: outcome and long term follow up of 32 patients. Rheumatology 40:763–771
Keogh KA, Specks U (2003) Churg– Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med 115:284–290
Sinicio RA, Di Toma L, Maggiore U et al (2005) Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg–Strauss syndrome. Arthritis & Rheumtism 52:2926–2935
Sablé–Fourtassou R, Cohen P, Mahr A et al (2005) Antineutrophil cytoplasmic antibodies and the Churg–Strauss syndrome. Ann Intern Med 143:632– 638
Lanham JG, Elkon KB, Pusey CD et al (1984) Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg–Strauss syndrome: clinical study and long–term follow up of 96 patients. Medicine (Baltimore) 78:26–37
Tai PC, Hayes DJ, Clark JB et al (1982) Toxic effects of human eosinophil secretion on isolated rat heart cells in vitro. Biochem J 204:75–80
Rothenberg ME (1998) Eosinophilia. NEJM 338:1592–1600
Brito–Babapulle F (2003) The Eosinophilia, including the idiopathic hypereosinophilic syndrome. British Journal of Haematology 121:203–223
Heger M, Bergler–Klein J, Zehetgruber M et al (2003) Linksventrikuläre Dysfunktion im Rahmen von Churg– Strauss syndrom. Z Kardiol 92:677– 681
McGavin CR, Marshall AJ, Lewis CT (2002) Churg–Strauss syndrome with critical endomyocardial fibrosis: 10 year survival after combined surgical and medical management. Heart 87:e5
Wolf M, Rose H, Smith RN (2005) Case 28–2005: a 42–year old man with Weight loss, Weakness, and a Rash. NEJM 353:1148–1157
Thomson D, Chamsi–Pasha H, Hasleton P (1989) Heart transplantation for Churg–Strauss syndrome. Br Heart J 62:409–410
Henderson RA, Hasleton P, Hamid BN (1993) Recurrence of Churg– Strauss vasculitis in a transplanted heart. Br Heart J 70:553
Simon HU, Seelbach H, Ehmann R et al (2003) Clinical and immunological effects of low–dose IFN–alpha treatment in patients with corticoid–resistant asthma. Allergy 58:1250–1255
Kruit WH, Punt KJ, Goey SH et al (1994) Cardiotoxicity as a dose–limiting factor in a schedule of high dose bolus therapy with interleukin–2 and alpha–interferon. An unexpectedly frequent complication. Cancer 74:2850–2856
Arbach O, Gross WL, Gause A (2002) Treatment of refractory Churg–Strauss syndrome (CSS) by TNF–α blockade. Immunobiol 206:496–501
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Rosenberg, M., Lorenz, H.M., Gassler, N. et al. Rapid progressive eosinophilic cardiomyopathy in a patient with Churg–Strauss syndrome (CSS). Clin Res Cardiol 95, 289–294 (2006). https://doi.org/10.1007/s00392-006-0364-0
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00392-006-0364-0