Life-Threatening Macrophage Activation Syndrome as the Initial Presentation of Systemic Lupus Erythematosus: A Case Report and Review of the Literature
November 2022
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.
This case report and literature review discuss a rare and fatal initial presentation of systemic lupus erythematosus (SLE) with macrophage activation syndrome (MAS) in a 20-year-old female. The patient experienced persistent fever, significant weight loss, and hematologic abnormalities, and despite aggressive treatment, the MAS was fatal. The report highlights the diagnostic challenges and high mortality of MAS in SLE patients, stressing the need for early recognition and intervention. The review of literature underscores the rarity and critical importance of prompt treatment for MAS in the context of SLE.
