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Case Reports in Medicine
Volume 2015, Article ID 294041, 4 pages
Case Report

Macrophage Activation Syndrome as Onset of Systemic Lupus Erythematosus: A Case Report and a Review of the Literature

1UOC Immunologia Clinica A, Dipartimento di Medicina Clinica, Policlinico Umberto I, Sapienza Università di Roma, 00185 Roma, Italy
2I Divisione Dermatologica, Istituto Dermopatico dell’Immacolata IRCCS, 00167 Roma, Italy

Received 20 March 2015; Accepted 23 April 2015

Academic Editor: Kenneth C. Kalunian

Copyright © 2015 Guido Granata et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Macrophage activation syndrome (MAS) is a potentially fatal condition. It belongs to the hemophagocytic lymphohistiocytosis group of diseases. In adults, MAS is rarely associated with systemic lupus erythematosus, but it also arises as complication of several systemic autoimmune disorders, like ankylosing spondylitis, rheumatoid arthritis, and adult-onset Still’s disease. Several treatment options for MAS have been reported in the literature, including a therapeutic regimen of etoposide, dexamethasone, and cyclosporine. Here we report a case of 42-year-old woman in whom MAS occurred as onset of systemic lupus erythematosus.