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
Studysummary This case report describes a rare instance where a young woman initially presented with macrophage activation syndrome as the first sign of systemic lupus erythematosus, and ultimately experienced multiorgan failure and death.
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