Refractory sweet syndrome with autoimmune organizing pneumonia treated with monoclonal antibodies to tumor necrosis factor.

We describe a 54-year-old man with highly refractory Sweet syndrome associated with autoimmune multifocal organizing pneumonia and underlying myelodysplastic disorder. His lung disease responded to oral cyclophosphamide. However, his skin disease and systemic symptoms followed a chronic course and responded only to very high doses of corticosteroid and were refractory to a number of corticosteroid-sparing agents. He was ultimately treated with infliximab, resulting in remission of his cutaneous and systemic symptoms and successful tapering of his corticosteroid dose. Subsequently, infliximab was replaced with adalimumab to achieve more sustained remission. His pulmonary lesions have not recurred on this treatment. His myelodysplastic syndrome followed a very slowly progressive course consistent with refractory anemia. This case report demonstrates the effectiveness of treatment with monoclonal antibody specific for tumor necrosis factor alpha (TNFalpha) in a patient with severe manifestations of Sweet syndrome refractory to other treatments.