Fever, Anasarca and Arthralgia

  • Erika Sifuentes-Rodriguez
  • Francisco Rivas-Larrauri
  • Marco Antonio Yamazaki-Nakashimada


  • Less than 1% of systemic lupus erythematosus (SLE) cases are associated with complement deficiencies

  • Complete C1q, C1s and C1r deficiencies are associated with a high risk to of juvenile SLE

  • Macrophage activation syndrome (MAS) is a known complication of pediatric rheumatic disorders and a secondary/reactive form of hemophagocytic lymphohistiocytosis

  • In SLE, MAS can mimic an acute exacerbation of the underlying disease, associated with fever, lymphadenopathy, splenomegaly and cytopenia


Systemic lupus erythematosus C1q deficiency Macrophage activation syndrome 


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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Erika Sifuentes-Rodriguez
    • 1
  • Francisco Rivas-Larrauri
    • 1
  • Marco Antonio Yamazaki-Nakashimada
    • 1
  1. 1.Clinical Immunology DepartmentInstituto Nacional de PediatríaMexico CityMexico

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