Macrophage activation syndrome triggered by active systemic lupus erythematosus : Successful treatment by interleukin-1 inhibition (anakinra)
| Autor: | L Kübler, I Bittmann, J G Kuipers |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
Adult
Anakinra Systemic lupus erythematosus Cyclophosphamide business.industry medicine.medical_treatment Fulminant Macrophage Activation Syndrome Macrophages Immunosuppression medicine.disease Pancytopenia Histiocytosis Interleukin 1 Receptor Antagonist Protein Rheumatology Macrophage activation syndrome Immunology medicine Humans Lupus Erythematosus Systemic Female business medicine.drug Interleukin-1 |
| Zdroj: | Zeitschrift fur Rheumatologie. 79(10) |
| ISSN: | 1435-1250 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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