Successful Treatment of Sulfasalazine-Induced DRESS Syndrome with Corticosteroids and N-Acetylcysteine

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a rare, severe drug hypersensitivity syndrome characterized by fever, rash, hematologic abnormalities, and systemic involvement. The pathogenesis of DRESS is unclear, but it is thought that the offending drug induces an immune-mediated hypersensitivity reaction through defects in metabolism. Severe cases of DRESS often require aggressive treatment; however, current pharmacologic treatment options are limited. We describe a 66-year-old woman who presented with fever and rash 3 weeks after starting sulfasalazine treatment for rheumatoid arthritis. Investigation revealed diffuse lymphadenopathy, leukocytosis, eosinophilia, and hepatitis. She was diagnosed with DRESS syndrome, sulfasalazine was discontinued, and she was treated with methylprednisolone. Her condition deteriorated, and she developed severe acute liver failure prompting evaluation for liver transplantation. Corticosteroid treatment was escalated to high-dose methylprednisolone, and N-acetylcysteine was started, which prompted improvement in her symptoms and liver function. To our knowledge, this is the first case report to describe the safe and successful use of corticosteroids and N-acetylcysteine to treat sulfasalazine-induced DRESS syndrome. Given the potential morbidity and mortality of DRESS syndrome, effective treatment alternatives for severe cases are needed. N-Acetylcysteine may be a safe adjunct to corticosteroid therapy for severe cases of sulfasalazine-induced DRESS syndrome.