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A 54 year old man presented with a six month history of worsening breathlessness. He had been diagnosed with reversible airflow obstruction at the age of 43 years and a raised peripheral blood eosinophil count of 0.7 × 109/l had been detected. This rose as high as 2.1 × 109/l eight years later, associated with transitory bi-apical shadowing on chestx ray. He was treated with oral steroid throughout this time.
In January 1995 his dyspnoea worsened significantly. Transthoracic echocardiography (TTE) revealed a dilated left ventricle and mild to moderate aortic regurgitation. Three months later repeat TTE showed further left ventricular dilatation and severe aortic incompetence.
The patient proceeded to homograft aortic valve replacement. Macroscopic appearance of the aortic valve showed thickening and fibrosis with retraction of individual leaflets, but no commissural fusion (below left). Histology of the aortic media showed diffuse, chronic inflammatory cell infiltrates of plasma cells and lymphocytes mixed with eosinophils. The aortic valve leaflets (below right) showed dense, chronic inflammation with eosinophils mixed between the lymphocytes and plasma cells (arrowed).
This is a unique case of an eosinophilic aortitis and valvitis presenting with aortic regurgitation. The longstanding airways disease, peripheral blood eosinophilia, and transitory pulmonary infiltrates point to Churg-Strauss syndrome, but there was no evidence of necrotising vasculitis on biopsy. This case highlights the importance of fully investigating new exacerbations of breathlessness in patients with longstanding pulmonary disease and the importance of histological sampling of aortic and valvar tissue in cases of aortic regurgitation.
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