Macrophage activation syndrome as a presenting feature in juvenile systemic lupus erythematosus.
Publication/Presentation Date
10-1-2024
Abstract
BACKGROUND: Macrophage activation syndrome (MAS) is an acquired form of hemo phagocytic lymphohistiocytosis (HLH) and is usually associated with infections, autoimmune, auto inflammatory syndromes and malignancies.
CASE DETAILS: A 14 year old girl presented with sub-acute onset of fever with lymphadenopathy, pancytopenia,high ferritin values and a falling erythrocyte sedimentation rate. She was evaluated with relevant laboratory tests that was suggestive of systemic Lupus erythematosus and associated macrophage activation syndrome She recovered with immunosuppressive therapy and other supportive care.
CONCLUSION: There is a need for a high index of suspicion of occult MAS and MAS in patients with systemic lupus erythematosus as it may be an initial presentation. Delay in diagnosis and initiation of treatment can lead to a higher mortality.
Volume
33
Issue
11
First Page
1254
Last Page
1259
ISSN
1477-0962
Published In/Presented At
Ramadoss, I., Rengabashyam, P., Seetharaman Varadhan, M., & Ponniah Subramanian, A. R. (2024). Macrophage activation syndrome as a presenting feature in juvenile systemic lupus erythematosus. Lupus, 33(11), 1254–1259. https://doi.org/10.1177/09612033241272972
Disciplines
Medicine and Health Sciences
PubMedID
39109623
Department(s)
Department of Medicine
Document Type
Article