Macrophage activation syndrome: a lifetrheatening complication in a 44 year old woman

Abstract

Macrophage activation syndrome (MAS) is a potentially life-threatening complication of autoimmune diseases. A 44-yearold woman was admitted to the hospital for further evaluation due to swelling in the left latero-cervical region, a mild fever that persisted for two weeks, itchy papular skin lesions, oral ulcers, and diffuse joint pain. Her medical history reported hypertension, diffused painful joints, and a pauci-symptomatic recent SARS-CoV-2 infection. A severe, drug-resistant fever developed while the patient was in the hospital. Infectious diseases were excluded and blood cultures, swabs, and serum antibody dosages were carried out. In a patient with systemic lupus erythematosus, MAS was diagnosed due to the presence of fever, bilinear cytopenia, high ferritin, transaminase, and triglyceride levels with low fibrinogen levels. Methyl-prednisolone at immunosuppressive dosages and cyclosporine were started. Hematobiochemical parameters gradually returned to normal as the fever subsided. The patient’s condition is being monitored while the cyclosporine therapy is continued and the steroidal therapy is gradually tapered off. It was not possible to show a potential link to the SARS-CoV-2 infection.

Keywords

• MAS
• LES
• differential diagnosis