1,721,437 research outputs found
Etanercept - TNF receptor and IgG1 Fc fusion protein: is it different from other TNF blockers?
No full textTNF blockers have been available to treat various inflammatory disorders since more than a decade. T cells and macrophages mainly express TNF and activate many cells through two types of receptors. Pharmaceutical companies developed two types of TNF blockers: soluble receptors and monoclonal antibodies. Understanding of differences of structure and function can explain divergence of efficacy or side effects. Etanercept has the best retention rate in rheumatic diseases, but is less or not effective in granulomatous diseases, such as inflammatory bowel diseases or uveitis. However, etanercept induces less tuberculosis infections than anti-TNF blocker monoclonal antibodies
Therapeutic innovations in the management of juvenile arthritis
No full textMuch remains to be learned about the optimal therapy for patients with juvenile arthritis. Better classification criteria, advances in knowledge of etiopathogenesis, and definition of uniform response criteria are all required. At the present time methotrexate is the only drug that has been shown to be superior to placebo in the treatment of juvenile arthritis. A review of current accepted management strategies is presented
Gravidanza a rischio e complicanze neonatali nelle malattie autoimmuni: Il neonato e il bambino
No full textMucopolysaccharidoses
No full textThe mucopolysaccharidoses (MPSs) are a group of rare genetic disorders of glycosaminoglycan catabolism, caused by a deficiency of lysosomal enzymes required for GAG degradation. Incomplete breakdown of glycosaminoglycans leads to progressive accumulation of these substances in many tissues throughout the body. Different residual enzymatic activity can result in different phenotypes of the same MPS disorder, from severe to attenuated. Musculoskeletal manifestations are common across all forms of MPS. Skeletal and joint abnormalities are prominent features of many MPS disorders, particularly attenuated phenotypes. However, diagnostic delays occur frequently for patients with an MPS, especially those with more attenuated forms of disease. In the absence of appropriate treatment, these conditions are chronic, progressive and often debilitating, but treatment for many types of MPS is now available. Therefore, increasing awareness of MPS among rheumatologists is extremely important
Pediatric rheumatic disease : treating lupus nephritis in children--is there a gold standard?
No full textSystemic lupus erythematosus (SLE) is fairly rare in children and adolescents; therefore, performing controlled studies in this particular patient group is difficult. Nowadays, the prognosis of this disorder has greatly improved, however renal involvement still carries very high morbidity
Recent advances in antiphospholipid antibodies and antiphospholipid syndromes in pediatric populations
No full textIn recent years, antiphospholipid antibodies (aPL) and their associated clinical features have been recognized increasingly in various pediatric autoimmune and non-autoimmune diseases. Pathogenic mechanisms involved in pediatric antiphospholipid syndrome (APS) appear to be the same as in adults. However, since pediatric patients do not have prothrombotic risk factors present in adults, there clearly are differences in the spectrum of clinical findings. The frequency of aPL-related thrombotic events is generally low in pediatric populations. On the other hand, various commonly acquired infections are likely to be responsible for higher percentage of non-pathogenic and transient aPL in childhood. Such points have to be considered in clinical judgment of elevated aPL in children. In this review we summarize the recent data on the prevalence and clinical significance of aPL in neonates, children and adolescents
Epilepsy as part of systemic lupus erythematosus and systemic antiphospholipid syndrome (Hughes syndrome)
No full textThe antiphospholipid syndrome (APS) is defined by the presence of antiphospholipid antibodies (aPL), demonstrated by ELISAs for antibodies against phospholipids and associated phospholipid-binding cofactor proteins and/or a circulating lupus anticoagulant (LA) together with diverse systemic clinical manifestations such as thrombosis, and recurrent spontaneous abortions. According to the criteria set out in Sydney the only neurological manifestations that can be suitable as APS classification criteria are ischemic events (stroke and transient ischemic attacks). However, other neurological manifestations, including seizures in particular, have been repeatedly reported in APS patients. The present review will summarize recent research on the association of aPL, as well as other autoantibodies, with seizure disorders, with or without concomitant SLE
Osteoporosis
No full textBone mass is determined primarily by genetic influences, but exogenous factors may also play a major role. The prevention of osteoporosis can start at childhood. Optimal achievement of peak bone mass during childhood and adolescence is important to minimize future fracture risk. Chronic inflammatory diseases can have a detrimental effect on bone mass through a variety of mechanisms. Different diagnostic methods for detecting osteoporosis (eg, dual x-ray absorptiometry, quantitative computed tomography, ultrasounds) are in use or under investigation. New treatment options are available; among these, the use of bisphosphonates seems to be the more promising approach
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