Abstract
We report a case of severe Churg-Strauss syndrome (CSS) complicated with diffuse alveolar hemorrhage. A 47-year-old female patient complicated with diffuse alveolar hemorrhage, disseminated intravascular coagulation and intracerebral hemorrhage was admitted to our ICU due to acute respiratory failure. As a result of several examinations, the patient was first diagnosed as having CSS. After treatment with high-dose steroid under mechanical ventilation, acute respiratory failure with diffuse alveolar hemorrhage was improved. However, when the steroid dose was reduced, diffuse alveolar hemorrhage recurred. Therefore, plasma exchange therapy was performed and resulted in dramatic improvement of recurrent diffuse alveolar hemorrhage, and the patient was weaned from mechanical ventilation and survived. Generally, immunosupressant agents are recommended for treatment of CSS when there is no response to steroid therapy. However, it has been demonstrated that the main cause of death in CSS patients with diffuse alveolar hemorrhage is sepsis. Therefore, plasma exchange rather than immunosuppressant therapy is a useful option for treatment of severe CSS to prevent complication with severe infection.
