ArticleZeitschrift fur Rheumatologie2020
Macrophage activation syndrome triggered by active systemic lupus erythematosus : Successful treatment by interleukin-1 inhibition (anakinra).
Article in Zeitschrift fur Rheumatologie, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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Who cites it
9 citing papers in PubMed, 10 citations in OpenAlex.
- Pathogenesis and therapeutic strategies for neuropsychiatric lupus centered on innate immune activation.Human cell · 2026Review
- Emergency in pediatric rheumatology: a narrative review.Frontiers in medicine · 2026Review
- Dengue Virus and the Host Immune System: A Battle of Immune Modulation, Response and Evasion.Pathogens (Basel, Switzerland) · 2025Review
- Induction of LY6E regulates interleukin-1β production, potentially contributing to the immunopathogenesis of systemic lupus erythematosus.Cell communication and signaling : CCS · 2025Article
- Heterogeneity of macrophage activation syndrome and treatment progression.Frontiers in immunology · 2024Review
- Loss of interleukin-1 beta is not protective in the lupus-prone NZM2328 mouse model.Frontiers in immunology · 2023Article
- Crosstalk between Interleukin-1β and Type I Interferons Signaling in Autoinflammatory Diseases.Cells · 2021Review
- Macrophage Polarization and Plasticity in Systemic Lupus Erythematosus.Frontiers in immunology · 2021Review
- Efficacy of high-dose anakinra in refractory macrophage activation syndrome in adult-onset Still's disease: when dosage matters in overcoming secondary therapy resistance.Therapeutic advances in musculoskeletal disease · 2020Article
Corrections and comments
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Authors and funding
3 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
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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