Hemophagocytic Syndrome In Association With Systemic Lupus Erythematosus and Rheumatoid Arthritis

Abstract

    Hemophagocytic syndrome (HPS) is a potentially life-threatening complication of rheumatic disorders characterized by the infiltration of morphologically benign hemophagocytic macrophages in the bone marrow and in various other organs. It is known to be associated with several conditions, such as viral or bacterial infection, malignancies, and autoimmune diseases such as systemic lupus erythematosus (SLE) and adult onset Still’s disease and less commonly with rheumatoid arthritis(RA). We report a case of a 59-year-old lady known to have SLE and RA who developed worsening anemia, thrombocytopenia, coagulopathy, renal failure and was diagnosed with hemophagocytic syndrome. In spite of aggressive supportive management, intravenous steroids and immunoglobulins, the patient eventually died due to intracranial bleeding. Although cases of HPS have been previously described in association with SLE or RA, this is the first case of HPS to our knowledge to be diagnosed in a patient who had both RA and SLE.




doi:10.4021/jmc589w