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).
Juvenile idiopathic arthritis (JIA) is the most common autoimmuneautoinflammatory disease in childhood and affects approximately 1 in 1,000 children. Despite advances in diagnosis and treatment options, JIA remains a chronic condition for most affected children. Recent evidence suggests that disease control at onset may determine the tempo of subsequent disease course and long-term outcomes, and raises the concept of a therapeutic window of opportunity in patients with JIA. This underscores the importance of early aggressive treatment in patients with JIA. With the advent of novel biologic therapeutics, the repertoire of agents available for treatment of children with JIA has greatly increased. The present article will summarize recent developments in the medical treatment of children with JIA and will offer insights into emerging therapies. IntroductionJuvenile idiopathic arthritis (JIA) is a chronic autoimmuneautoinflammatory disease of unknown etiology. It is estimated that JIA affects up to 1 in 1,000 children worldwide and is the most common cause of autoimmune musculoskeletal disease in children [1]. By definition, children with JIA have disease onset prior to age 16 years, and present with joint pain, stiffness and swelling that persists for longer than 6 weeks. Formerly referred to as juvenile rheumatoid arthritis, the classification scheme for JIA was updated by the International League of Associations for Rheumatology in 2001 to reflect the unique nature of arthritis in childhood and to distinguish JIA from adult-onset rheumatoid arthritis (RA) [2]. Based on these criteria, JIA is subdivided into categories based on the number of joints affected and the presence or absence of specific serologic findings and systemic manifestations (Table 1).Without appropriate treatment, JIA may result in devastating consequences. Children may experience permanent disability from joint destruction, growth deformities or blindness (from chronic uveitis associated with JIA) [3,4]. In the case of the systemic-onset form of JIA (SOJIA), untreated disease may even result in multiple organ failure and death.Twenty years ago it was commonly believed that childhoodonset arthritis might subside in adulthood. Recent studies, however, have demonstrated that sustained resolution of disease occurs in only a small minority of JIA patients (as many as 50% of children with JIA enter adulthood with ongoing, active disease) [3]. Additional information from a recent large, multicenter, retrospective study indicates that patients diagnosed with JIA experience a chronic course involving cycling of disease between active and inactive states over the course of years. Although 196 out of 437 JIA patients followed over a median of 7 years achieved a period of 1 year without any JIA symptoms off all medications, less than 20% of patients had two consecutive years without symptoms and only 4% had a 5-year disease-free period [5]. These studies indicate that many patients diagnosed with JIA will be exposed to extended periods of medicatio...
Primarily self-directed online self-management training and online disease education comparably and modestly improve pain and HRQOL in youth with JIA.
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