Churg-Strauss syndrome (CSS) is characterized by asthma and/or a history of allergy, eosinophilia and an often life-threatening systemic necrotizing vasculitis. We describe a patient with CSS and hypoxemia with a high alveolar-arterial oxygen gradient (AaDO2), but no pulmonary parenchymal involvement. The patient also had a low diffusion capacity with normal lung volume and a high level of serum thrombomodulin, a marker of endothelial cell injury. Treatment for CSS, such as corticosteroid, improved both hypoxemia and AaDO2 consistent with amelioration of diffusion capacity and serum thrombomodulin level, suggesting that this pathosis involves microangiopathy with endothelial cell damage induced by vasculitis in pulmonary blood vessels.
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