Life-Threatening Macrophage Activation Syndrome as the Initial Presentation of Systemic Lupus Erythematosus: A Case Report and Review of the Literature
January 2023
in “
The Egyptian Rheumatologist
”
macrophage activation syndrome systemic lupus erythematosus hair loss leucopenia neutropenia lymphopenia anemia thrombocytopenia elevated liver enzymes hyperbilirubinemia hypertriglyceridemia hyperferritinemia elevated D-Dimer elevated lactate dehydrogenase ANA anti-dsDNA low serum C3 low serum C4 methylprednisolone prednisolone cyclosporine A intravenous immunoglobulin MAS SLE low white blood cells low neutrophils low lymphocytes low red blood cells low platelets high liver enzymes high bilirubin high triglycerides high ferritin high D-Dimer high LDH antinuclear antibodies anti-double stranded DNA low C3 low C4 IV methylprednisolone IVIG
TLDR Macrophage activation syndrome can be a deadly first sign of systemic lupus erythematosus.
A 20-year-old female presented with macrophage activation syndrome (MAS) as the initial manifestation of systemic lupus erythematosus (SLE), characterized by persistent fever, significant hair loss, and general malaise. Laboratory findings included leucopenia, neutropenia, lymphopenia, anemia, thrombocytopenia, elevated liver enzymes, hyperbilirubinemia, hypertriglyceridemia, hyperferritinemia, elevated D-Dimer, and elevated lactate dehydrogenase. Immunological tests were positive for ANA and anti-dsDNA, with low serum C3 and C4 complement factors. Despite treatment with intravenous methylprednisolone, prednisolone, cyclosporine A, and intravenous immunoglobulin, the patient's condition worsened, leading to multiorgan failure and death. This case highlights MAS as a potentially life-threatening initial presentation of SLE.
