Macrophage activation syndrome in juvenile systemic lupus erythematosus: A multinational multicenter study of thirty‐eight patients

Abstract

Objective. To describe the clinical and laboratory features of macrophage activation syndrome as a complication of juvenile systemic lupus erythematosus (SLE).Methods. Cases of juvenile SLE-associated macrophage activation syndrome were provided by investigators belonging to 3 pediatric rheumatology networks or were found in the literature. Patients who had evidence of macrophage hemophagocytosis on bone marrow aspiration were considered to have definite macrophage activation syndrome, and those who did not have such evidence were considered to have probable macrophage activation syndrome. Clinical and laboratory findings in patients with macrophage activation syndrome were contrasted with those of 2 control groups composed of patients with active juvenile SLE without macrophage activation syndrome. The ability of each feature to discriminate macrophage activation syndrome from active disease was evaluated by calculating sensitivity, specificity, and area under the receiver operating characteristic curve.Results. The study included 38 patients (20 with definite macrophage activation syndrome and 18 with probable macrophage activation syndrome). Patients with definite and probable macrophage activation syndrome were comparable with regard to all clinical and laboratory features of the syndrome, except for a greater frequency of lymphadenopathy, leukopenia, and thromDr Pringe is recipient of an Alpha Scholarship from the European Union (contract no. AML/B7-311/970666/II-0246-FI).

Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

“…Gene signatures consistent with chronic TLR/IL-1b signaling are present in sJIA patients. 53 Lupus, another rheumatic condition associated with MAS, 4 has also long been associated with hyperactive TLR9 function. 54 EBV, perhaps the most common infectious trigger of secondary HLH, is a DNA virus that triggers TLR9.…”

“…Macrophage activation syndrome (MAS) is a serious, potentially fatal complication of rheumatic diseases, which is seen most frequently in systemic juvenile idiopathic arthritis (sJIA) and in its adult equivalent, adult-onset Still disease, 1 --3 although it is increasingly reported in other pediatric inflammatory disorders, namely juvenile systemic lupus erythematosus 4 and Kawasaki disease. 5 --7 In recent years, this condition has also been observed in periodic fever syndromes.…”

Young Male with Secondary Acute Hemophagocytic Lymphohistiocytosis

“…The list of possible etiologies for secondary HLH is long and includes infections (mainly EBV, herpes simplex virus, cytomegalovirus, avian influenza) [2][3][4] , rheumatologic diseases (rheumatoid arthritis, systemic lupus erythemtosus, kawasaki disease, adult onset still's disease ) [5,6] , malignacy (natural killer cell leukemia, peripheral T cell lymphoma) [7,8] , acquired immune deficiency states (after organ transplants), and drugs [9] .…”

Macrophage activation syndrome

“…Może również wystąpić w przebiegu choroby Stilla, TRU (0,9-4,6% przypadków) [15,16], w reumatoidalnym zapaleniu stawów (RZS), zespole Sjögrena, zapaleniu skórno-mięśniowym, chorobie Kawasaki, mieszanej chorobie tkanki łącznej oraz w twardzinie układo-wej [5,7,12,17].…”