Systemic onset juvenile idiopathic arthritis is a rare form of juvenile arthritis in which, contrary to autoimmune diseases in general, no association with a certain tissue type has been detected. Together with this fact, the lack of autoantibodies and the general symptoms belonging to the diagnostic criteria of the illness such as high fever, rather speak for its classification into autoinflammatory diseases. Treatment is usually started with anti-inflammatory drugs, often requiring combination with a systemic glucocorticoid. Recognition of interleukins 1 and 6 as central mediators in the pathogenesis of the disease has brought new possibilities for its treatment.
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Systemic onset juvenile idiopathic arthritis is a rare form of juvenile arthritis in which, contrary to autoimmune diseases in general, no association with a certain tissue type has been detected. Together with this fact, the lack of autoantibodies and the general symptoms belonging to the diagnostic criteria of the illness such as high fever, rather speak for its classification into autoinflammatory diseases. Treatment is usually started with anti-inflammatory drugs, often requiring combination with a systemic glucocorticoid.
View Article and Find Full Text PDFInhibition of IL-6 for the treatment of inflammatory diseases.
Curr Opin Pharmacol
August 2004
Laboratory of Immune Regulation, Graduate School of Frontier Biosciences, Osaka University, 1-3 Yamada-oka, Suita-City, Osaka, 565-0871, Japan.
Interleukin-6 (IL-6) is a pleiotropic cytokine with various biological activities. Deregulated overproduction of IL-6 has been found to play pathological roles in chronic inflammatory diseases such as rheumatoid arthritis, Castleman's disease, juvenile idiopathic arthritis and Crohn's disease. Humanized anti-IL-6 receptor antibody has been developed as a therapeutic agent for these diseases, and therapeutic benefits have been revealed in clinical studies.
View Article and Find Full Text PDFPathogenesis of hemophagocytic syndrome (HPS).
Autoimmun Rev
February 2004
Department of Internal Medicine, Hôpital Avicenne, 125, rue de Stalingrad, Bobigny 93009, France.
Hemophagocytic syndrome (HPS) is a clinicopathologic entity characterized by increased proliferation and activation of benign macrophages with hemophagocytosis throughout the reticuloendothelial system. Uncontrolled T-lymphocyte activation is responsible for increased T(H)1 cytokines secretion such as IFN-gamma, IL-12 and IL-18 that promotes macrophage activation. Genetic defects specific for cytotoxic T lymphocytes (CTL) and natural killer (NK) cells have been identified in patients with primary HPS that are responsible for altered cell death and apoptosis induction or target killing.
View Article and Find Full Text PDFFever of unknown origin in Turkey: evaluation of 87 cases during a nine-year-period of study.
J Infect
January 2004
Department of Infectious Diseases and Clinical Microbiology, Medical Faculty, University of Cukurova, Adana, Turkey.
Objectives: To investigate fever of unknown origin (FUO) in 87 patients.
Methods: We investigated 87 (61 male) patients with FUO using the criteria of Petersdorf and Beeson [Medicine 40 (1961) 1] hospitalized between January 1994 and August 2002 at Cukurova University Hospital.
Results: The median age of the patients was 38.
[Adult-onset Still-disease: survey of 18 cases].
Orv Hetil
January 2003
Fóvárosi Szent László Kórház, VI. Fertózó Belgyógyászati Osztály, Budapest.
Introduction: Adult onset Still's disease (juvenile rheumatoid arthritis with septic appearance) is rare, leading to clinical signs similar to those seen in bacterial sepsis, lymphomas, rheumatological, or systemic autoimmune diseases. The disease can present with a fever of unknown origin, and can cause difficulties in the diagnosis. It is based upon, partly, the exclusion of other diseases and on diagnostic criteria.
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