title: Recurrent macrophage activation syndrome as the primary manifestation in systemic lupus erythematosus and the benefit of serial ferritin measurements: a case-based review.
abstract: Macrophage activation syndrome (MAS) is a fatal complication in rheumatic diseases. It is characterized by prolonged fever, pancytopenia, and hepatosplenomegaly, which are consequences of uncontrolled macrophage activation. MAS in children is most commonly associated with systemic juvenile idiopathic arthritis. Its association with systemic lupus erythematosus (SLE) is relatively rare, so we report a Thai boy who initially presented with MAS and eventually was diagnosed as having SLE. He also had recurrent MAS during the course of therapy. Hyperferritinemia is one of the abnormal laboratory findings in MAS and it has been used as an inflammatory marker. However, its correlation with disease activity remains unclear. Therefore, a review of literature regarding MAS-associated SLE in children and ferritin level in this disease was carried out.
authors.affiliation: Division of Allergy, Immunology, and Rheumatology, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Rachatevi, Bangkok 10400, Thailand. soamarat21@hotmail.com
authors.forename: Soamarat
authors.initials: S
authors.lastname: Vilaiyuk
authors.forename: Nongnuch
authors.initials: N
authors.lastname: Sirachainan
authors.forename: Suthep
authors.initials: S
authors.lastname: Wanitkun
authors.forename: Kwanchai
authors.initials: K
authors.lastname: Pirojsakul
authors.forename: Jarin
authors.initials: J
authors.lastname: Vaewpanich
mesh_terms: D000293:Adolescent; D005293:Ferritins / Q000097:blood; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000503:physiopathology*; D055501:Macrophage Activation Syndrome / Q000150:complications / Q000503:physiopathology*; D008297:Male; D009765:Obesity / Q000150:complications; D015203:Reproducibility of Results; D013785:Thailand; D013997:Time Factors
chemical_list: D005293:Ferritins