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It infrequently occurs in systemic lupus erythematosus (SLE), and it is extremely rare to be the first presentation of SLE. In a study of 511 patients with SLE, 7 cases (1.4%) of MAS were identified. In all the cases, MAS was simultaneous to the presentation of SLE in this article, we report a case of a patient with MAS who presented with fever, rash, and high ferritin level up to 16911 ng\/mL. A high degree of suspicion is required that high fever and rash can be clues to MAS. Early diagnosis is necessary since mortality rates remain high for untreated cases.<\/jats:p>","DOI":"10.1177\/2324709618812196","type":"journal-article","created":{"date-parts":[[2018,11,16]],"date-time":"2018-11-16T02:14:06Z","timestamp":1542334446000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":7,"title":["A Rare Case of Macrophage Activation Syndrome Presenting as the First Manifestation of Systemic Lupus Erythematosus"],"prefix":"10.1177","volume":"6","author":[{"given":"Pooja","family":"Poudel","sequence":"first","affiliation":[{"name":"State University of New York Upstate Medical University, Syracuse, NY, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thein","family":"Swe","sequence":"additional","affiliation":[{"name":"Interfaith Medical Center, New York, NY, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sheetal","family":"Rayancha","sequence":"additional","affiliation":[{"name":"State University of New York Upstate Medical University, Syracuse, NY, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2018,11,15]]},"reference":[{"key":"bibr1-2324709618812196","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21039"},{"key":"bibr2-2324709618812196","doi-asserted-by":"publisher","DOI":"10.4081\/reumatismo.2018.1023"},{"key":"bibr3-2324709618812196","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-013-2227-1"},{"key":"bibr4-2324709618812196","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/ken342"},{"key":"bibr5-2324709618812196","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-03-278127"},{"key":"bibr6-2324709618812196","doi-asserted-by":"publisher","DOI":"10.1002\/art.34473"},{"key":"bibr7-2324709618812196","first-page":"435","volume":"34","author":"Albert A","year":"1992","journal-title":"Nouv Rev Fr Hematol"},{"key":"bibr8-2324709618812196","first-page":"451","volume":"11","author":"Stephan JL","year":"1993","journal-title":"Clin Exp Rheumatol"},{"key":"bibr9-2324709618812196","first-page":"121","volume":"20","author":"Athreya BH.","year":"2002","journal-title":"Clin Exp Rheumatol"},{"key":"bibr10-2324709618812196","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-114-5-387"},{"key":"bibr11-2324709618812196","doi-asserted-by":"publisher","DOI":"10.1177\/0961203309354906"},{"key":"bibr12-2324709618812196","doi-asserted-by":"publisher","DOI":"10.1182\/asheducation-2009.1.127"},{"key":"bibr13-2324709618812196","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0609.2004.00318.x"},{"key":"bibr14-2324709618812196","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21441"},{"key":"bibr15-2324709618812196","doi-asserted-by":"publisher","DOI":"10.1002\/art.24883"},{"key":"bibr16-2324709618812196","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.1141"},{"key":"bibr17-2324709618812196","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21423"},{"key":"bibr18-2324709618812196","doi-asserted-by":"publisher","DOI":"10.1002\/acr.20002"},{"key":"bibr19-2324709618812196","doi-asserted-by":"crossref","unstructured":"McGhee A. 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