Preliminary guidelines for MAS in juvenile SLE

A group of researchers have developed preliminary diagnostic guidelines for macrophage activation syndrome in juvenile systemic lupus erythematosos (SLE). Representing the Lupus Working Group for of the Paediatric Rheumatology European Society the international team of researchers compared the clinical and laboratory features of 38 juvenile SLE patients with MAS with that of 2 control groups of patients with active juvenile SLE without MAS. The ability of each feature to discriminate MAS from active disease was evaluated by calculating sensitivity, specificity, and area under the receiver operating characteristic curve. Fever was present in as many as 90% of patients with macrophage activation syndrome, the researchers found. All of the other clinical features provided high specificity rates, but were not as good in terms of sensitivity, they said. Writing in the November issue of Arthritis and Rheumatism the authors advised that when a patient with juvenile SLE presents with unexplained fever and cytopenia, an evaluation for macrophage activation syndrome, including assessment for hyperferritinemia, should be carried out. Diagnostic confirmation through the demonstration of macrophage hemophagocytosis in the bone marrow may not be necessary in the presence of the typical clinical and laboratory features of the syndrome, they said. “Although first-line treatment is based on the parenteral administration of high-dose corticosteroids, cyclosporine, and perhaps etoposide, may play a role when...