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[Macrophage activation syndrome in lupus]
R M Javier1, J Sibilia, C Offner
1Service de Rhumatologie, CHU Hautepierre, Strasbourg.
Revue Du Rhumatisme (Ed. Francaise : 1993)
|November 30, 1993
Summary
Macrophage activation syndrome (MAS) is a rare but serious complication in patients with systemic lupus erythematosus (SLE). Prompt diagnosis and consideration of MAS in SLE patients with febrile pancytopenia are crucial.
Area of Science:
- Rheumatology
- Hematology
- Pathology
Context:
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease.
- Macrophage activation syndrome (MAS) is a life-threatening condition characterized by excessive immune activation.
- MAS is rare in SLE patients but shares features with other conditions like hemophagocytic lymphohistiocytosis.
Purpose:
- To report a case of MAS in a patient with SLE.
- To highlight the diagnostic challenges and clinical presentation of MAS in SLE.
- To discuss potential management strategies for MAS in SLE.
Summary:
- A patient with SLE presented with fever, pancytopenia, hypertriglyceridemia, and elevated ferritin, leading to a diagnosis of MAS with hemophagocytosis.
- Despite negative infectious workup, pulmonary nocardiosis developed. Initial treatment with intravenous immunoglobulin yielded transient remission, followed by cyclophosphamide.
- MAS in SLE is rare, mimics other conditions, and may stem from lupus-related immune dysregulation, necessitating consideration in SLE patients with febrile pancytopenia.
Impact:
- This case underscores the importance of considering MAS in the differential diagnosis of SLE patients presenting with severe cytopenias and fever.
- It highlights the diagnostic overlap between MAS and infections in SLE, emphasizing the need for thorough investigation.
- The report contributes to understanding the complex interplay of autoimmunity and secondary hemophagocytosis in SLE, informing clinical practice and future research.