Journal Information
Vol. 19. Issue 7.
Pages 402-403 (August - September 2023)
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Vol. 19. Issue 7.
Pages 402-403 (August - September 2023)
Case report
Severe and life-threatening onset of systemic lupus erythematosus
Una presentación grave y potencialmente fatal de lupus eritematoso sistémico
Ana Martinsa,b,
Corresponding author

Corresponding author.
, Sofia Pimentaa,b, Daniela Oliveiraa,b, Frederico Martinsc, Beatriz Samõesd, Lúcia Costaa
a Department of Rheumatology, Centro Hospitalar Universitário de São João, Porto, Portugal
b Department of Medicine, Faculdade de Medicina da Universidade do Porto, Porto, Portugal
c Department of Rheumatology, Hospital de Faro, CHA, Faro, Portugal
d Department of Rheumatology, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal
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Macrophage activation syndrome (MAS) is a potentially life-threatening complication of rheumatic diseases. We report a unique case of a previously healthy 20-year-old female presenting with MAS as first presentation of systemic lupus erythematosus. Remission was achieved with hydroxychloroquine, intravenous methylprednisolone pulse followed by oral prednisolone and cyclosporine. However, the management of MAS is still challenging, and the mortality rate remains high.

Systemic lupus erythematosus
Macrophagic activation syndrome

El síndrome de activación macrofágica (SAM) es una complicación potencialmente letal de algunas enfermedades reumáticas. Presentamos un caso único de una mujer de 20 años previamente sana que se presentó con SAM como primera manifestación de lupus eritematoso sistémico. Se logró una remisión completa con hidroxicloroquina, pulsos intravenosos de metilprednisolona seguido de prednisolona oral y ciclosporina. Sin embargo, el manejo del SAM sigue siendo un desafío y la tasa de mortalidad sigue siendo alta.

Palabras clave:
Lupus eritematoso sistémico
Síndrome de activación macrofágica


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