1,721,509 research outputs found
The time has come to include assessment of radiographic progression in juvenile idiopathic arthritis clinical trials
No full textMacrophage activation syndrome.
No full textMacrophage activation syndrome (MAS) is a serious complication of childhood systemic inflammatory disorders that is thought to be caused by excessive activation and proliferation of T lymphocytes and macrophages. Recent findings in hemophagocytic lymphohistiocytosis, a disease that is clinically similar to MAS, highlight the possible pathogenetic role of a defective function of perforin, a protein involved in the cytolytic processes and control of lymphocyte proliferation. Although the clinical features of MAS have been well documented, early diagnosis can be difficult. Measurement of the serum ferritin level may assist in the diagnosis and may be a useful indicator of disease activity, therapy response, and prognosis. The recognition that MAS belongs to the secondary or reactive hemophagocytic syndromes has led to the proposal to rename it according to the contemporary classification of histiocytic disorders. Cyclosporin A has been found effective in patients with corticosteroid-resistant MAS. A recent report has suggested that etanercept may be a useful adjunctive therapeutic agent
Toward an understanding of the long-term outcome of juvenile idiopathic arthritis.
No full textOver the past four decades, a number of studies have evaluated the long-term outcome of juvenile idiopathic arthritis (JIA) and some of them have also attempted to identify early prognostic factors. This editorial addresses, by reviewing the surveys that have analyzed the outcome of JIA in term of clinical remission, physical disability, and radiographic damage, the clinical questions that are most relevant in this area of study. Altogether, the available data indicate that JIA is not a benign disease because a considerable number of patients still enter adulthood with persistently active disease and a significant proportion of them may develop severe physical disability. Among the different onset forms, the long-term outcome is best in persistent oligoarthritis and worst in RF-positive polyarthritis; the outcome of systemic arthritis is widely variable, perhaps reflecting the heterogeneity of this JIA subtype. The comparison of earlier studies with those published in the last decade shows a decline in the frequency of patients with severe physical disability over the years; however, the proportion of patients who enter adulthood with active disease does not seem to be diminished. Although there is considerable data on prognostic factors in JIA, prediction of long-term outcome early after disease presentation is still difficult because comparisons among studies are hindered for a variety of reasons. Thus, while a considerable body of data is accumulating, the definition of the long-term outcome of JIA remains imperfect. To increase the comparability of future analyses and to obtain generalizable information on the prognosis of JIA and its prediction, a great deal of effort should be directed toward standardizing the study design and the measurement of predictors and outcomes
Should children and adolescents with systemic lupus erythematosus be given statin therapy to prevent early atherosclerosis?
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Macrophage activation syndrome is haemophagocytic lymphohistiocytosis – Need for the right terminology [Reply to letter]
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