A Rare Case of Macrophage Activation Syndrome Presenting as the First Manifestation of Systemic Lupus Erythematosus. (November 2018)

• Record Type: Journal Article
• Authors: Poudel, Pooja; Swe, Thein; Rayancha, Sheetal
• Is Part Of: Journal of investigative medicine high impact case reports. Volume 6(2018)
• Publication Date: 2018-11
• Subjects: MAS -- SLE; Internal medicine -- Periodicals; Medical care -- Periodicals; 616.005
• DOI: 10.1177/2324709618812196
• Languages: English
• ISSNs: 2324-7096

Abstract

Macrophage activation syndrome (MAS) itself is a rare, potentially life-threatening complication of a rheumatic disease, mostly seen in juvenile idiopathic arthritis. 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.