Rapid progressive eosinophilic cardiomyopathy in a patient with Churg–Strauss syndrome (CSS) |
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Authors: | M Rosenberg H M Lorenz N Gassler H A Katus Dr Norbert Frey |
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Institution: | (1) Department of Cardiology, Internal Medicine III, University of Heidelberg, INF 410, 69120 Heidelberg, Germany;(2) Department of Rheumatology, Internal Medicine V, University of Heidelberg, INF 410, 69120 Heidelberg, Germany;(3) Institute of Pathology, University of Heidelberg, INF 220, 69120 Heidelberg, Germany |
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Abstract: | 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. |
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Keywords: | Churg– Strauss syndrome eosinophilic cardiomyopathy allergic asthma eosinophilia |
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