Journal
ZEITSCHRIFT FUR RHEUMATOLOGIE
Volume 79, Issue 10, Pages 1040-1045Publisher
SPRINGER HEIDELBERG
DOI: 10.1007/s00393-020-00836-w
Keywords
Hyperferritinemia; Fever of unknown origin; Disease flare; Therapy resistance; Pancytopenia
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This article presents a case of fulminant macrophage activation syndrome (MAS) as a rare complication of active systemic lupus erythematosus in a 33-year-old female patient. Initial presentation showed severe lupus disease exacerbation with renal involvement, hemolytic anemia, and neuropsychiatric changes. Early therapy focused on broad immunosuppression (high-dose corticosteroids and cyclophosphamide); however, disease remission could not be achieved. After an additional inflammatory focus and underlying malignancy were excluded, the triplet of pancytopenia, fever, and high ferritin levels indicated MAS, a bone marrow biopsy confirmed secondary hemophagocytic histiocytosis. Treatment with an interleukin-1 antagonist (anakinra) induced a fast, effective therapeutic success.
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