Background Churg-Strauss syndrome is a systemic necrotizing vasculitis of small and medium vessels of unknown etiology associated to asthma and characterized by peripheral eosinophilia, extravascular granulomatous inflammation with eosinphilic infiltrates that may be associated to ANCA. Heart involvement has been reported in about 50% of cases and is considered one of the most feared complications1.
Objectives A 56-years-old woman with a past medical history of poorly controlled bronchial asthma, was admitted to hospital complaining of shortness of breath, dry cough and lower extremities pitting edema developed over the last two months. Her background history in the presence of peripheral eosinophilia, multiple lung infiltrates on xray and high resolution computed tomography, mononeuritis multiplex and ANCA positivity established the diagnosis of Churg-Strauss syndrome. Echocardiography revealed an anterior pericardial effusion and a pericardial mass of 3 x 5 cm completely attached to the right ventricular wall. These features were confirmed by a trans esophageal approach and subsequently by a heart MRI that defined the presence of a large cardiac mass indistinguishable from the right atrium and ventricular wall, invading the atrioventricular sulcus and incasing the origin and the proximal tract of the right coronary artery. Biopsy ruled out a cardiac tumor showing features of granulomatous inflammation with diffuse eosinophilic infiltration and no cellular atypia. The patient was treated with boluses of cyclophosphamide and high doses of methylprednisolone with prompt clinical improvement and complete resolution of the cardiac mass as shown by clinical- radiological follow up.
Conclusions The patient presented features resembling right heart impairment secondary to a large inflammatory pseudotumor associated to Churg-Strauss syndrome. MRI is the gold standard to define cardiac constriction syndromes and Churg-Strauss heart involvement2,3. Cyclophosphamide and corticosteroids represent the cornerstone of treatment with remarkable response even in advanced cases if promptly administered1.
Chiara Baldini, MD, PH, Rosaria Talarico, MD, PHD, Alessandra Della Rossa MD, PHD, Stefano Bombardieri, MD. Clinical Manifestations and Treatment of Churg Strauss Syndrome. Rheum Dis Clin N Am 36 (2010) 527–543
Rory O’Hanlon, MRCPIa, DudleyJ. Pennell, MD, FRCP, FACCa,b,*. Cardiovascular Magnetic Resonance in the Evaluation of Hypertrophic and Infiltrative Cardiomyopathies. Heart Failure Clin 5 (2009) 369–387
J.P. Smedema, P. van Paassen, et al. Cardiac involvement of Churg Strauss syndrome demonstrated by magnetic resonance imaging. Clin Exp Rheumatol 2004; 22 (Suppl. 36): S75-S78.
Disclosure of Interest None Declared