[Churg-Strauss syndrome with right ventricular tumor].
Cuvinte cheie
Abstract
METHODS
A 47-year-old woman was admitted because of diarrhoea (3-5 stools daily) for 9 days, weakness for 3 weeks and painful wrist swelling, arthralgia and hyperesthesia over the medial aspect of the right thigh for 6 weeks. For 6 years she had been treated for asthma. Two operation had been performed for chronic sinusitis. ADMISSION FINDINGS AND INVESTIGATIONS: There was marked eosinophilia (5889/microliter) and thrombocytopenia (96,000/microliter), markedly increased serological inflammatory parameters and a raised total IgE level (134 IU/ml). The chest radiogram showed pulmonary infiltrates and bone marrow biopsy indicated eosinophilia. Echocardiography revealed a mass, 3 x 8 cm, in the ventral wall of the right ventricle with extension into the outflow tract.
METHODS
These findings met the criteria of the American College of Rheumatology for Churg-Strauss syndrome (CSS). On methylprednisolone (1 mg/kg daily) the eosinophil and platelet counts became normal within 5 days and erythrocyte sedimentation rate and the level of C-reactive protein fell. Under cardiopulmonary bypass the tumour was removed, the tricuspid valve replaced, and the right ventricle reconstructed. Histology of the mass revealed it to be an organized thrombus.
CONCLUSIONS
The relationship between the thrombus formation and CSS is unclear. Hypercoagulability connected with the inflammatory process may have played a part in the pathogenesis.
