ISSN:1040-8711    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Monoclonal antibody and recombinant DNA technologies have led to the development of biologic therapies capable of directly targeting selected components of the immune response. With the steady expansion of knowledge regarding the mechanisms of vascular inflammation, the safety and efficacy of biologic agents in the vasculitic diseases are being increasingly investigated. By targeting specific effector mechanisms involved in the pathogenesis of vasculitis, these agents may provide a less toxic means of inducing remission and lessening relapse. However, the study of biologic therapies in the vasculitides must be approached with caution, as unanticipated effects on disease activity and disease-specific toxicities can occur. Studies to examine these agents must recognize the potential for active vasculitis to be organ- or life-threatening as well as the current existence of effective therapies. In the research setting, investigation of biologic agents in the treatment of vasculitic diseases may also provide important insights into pathogenesis of these syndromes.

ISSN:1040-8711    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

The past year has seen some notable publications in the field of vasculitis epidemiology. The appearance of reports from several different parts of the world has permitted comparisons to be made. In Europe, Wegener granulomatosis appears to be more common at high latitudes, whereas microscopic polyangiitis shows the reverse pattern. Kawasaki disease is most common in Japan. In China, the United States, and the United Kingdom, the incidence is lower, but the incidence appears to be increasing globally. Studies of infectious triggers continue to be disappointingly negative. Immunogenetic associations with cytokine polymorphisms are beginning to be reported.

ISSN:1040-8711    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Systemic vasculitis refers to a condition of blood vessel inflammation, of which the causes are various. In a substantial number of cases, autoantibodies against neutrophil cytoplasm constituents (ANCAs) are present. The authors then refer to the systemic vasculitis as ANCA-associated systemic vasculitis. Renal disease is an unfavorable component, leading to dialysis dependency in a considerable number of patients. This review aims to summarize in brief what was reported about ANCA-associated vasculitis in the recent past. What the exact pathogenic role of ANCAs in the development of systemic vasculitis is remains uncertain, and it is still not clear how their presence leads to the histopathologic lesions called vasculitis.

ISSN:1040-8711    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Despite important therapeutic improvements, permanent organ failure may develop in primary systemic vasculitides and affect the heart, the lungs, and especially the kidneys. In systemic vasculitides associated with antineutrophil cytoplasmic antibodies (AASV), end-stage renal failure develops in 20% of cases. Renal transplantation became a beneficial option in these patients, with a graft and patient survival comparable to that in nondiabetic patients. This review summarizes the current knowledge on indications and contraindications for renal transplantation in AASV and discusses the impact of posttransplant immunosuppression on the course of the patients.

ISSN:1040-8711    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

This past year, 2002, marked the 100th anniversary of the death of Adolf Kussmaul. To rheumatologists, he is important as the author of the first case of idiopathic vasculitis, polyarteritis nodosa, which even today is the basis of our understanding of vasculitis. To general internists, his name is linked to Kussmaul's sign and Kussmaul breathing, and to gastroenterologists, he is in many ways—through the development of the gastric pump and the first gastroscope—the father of modern gastroenterology. His reputation as an excellent teacher, his compassion for his patients, and his ability as a clinician scientist led him to be regarded as a giant of 19th century medicine. Kussmaul's life and career, dedicated foremost to reducing human suffering and advancing medicine as a scientific discipline with a strong humanistic foundation, make him a role model for physicians of today.

ISSN:1040-8711    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

The relation between rheumatic syndromes and an underlying malignancy is a complex one. As a result of autoimmunity, an aberrant immune response, or the use of immunomodulatory drugs, many of the rheumatic diseases appear to pose an increased risk for the development of malignancy. Unfortunately, for many of the same reasons, the presence of an underlying malignancy can result in the development of features of rheumatic disease. Awareness of the associations between rheumatic syndromes and malignancy will aid the clinician in the accurate diagnosis of underlying pathology, more effective treatment of both the symptoms and underlying disease, and appropriate surveillance for the development of later complications.

ISSN:1040-8711    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Patients with sickle cell disease often seek treatment for rapid periorbital swelling due to infarction of the orbital bones. Because of resulting orbital compression syndrome, treatment with corticosteroids and antibiotics is advisable. If spinal tuberculosis occurs in patients with sickle cell anemia, radiologic signs will be a combination of the two conditions. The diagnosis of juvenile rheumatoid arthritis is usually delayed in patients with sickle cell disease. Sulphasalazine is the disease-modifying drug of choice for treating juvenile rheumatoid arthritis, because it also reduces the adhesiveness of sickled red cells. TNF-&agr; inhibitors may also be useful for treating these patients.A volumetric method to determine the size and special distribution of the necrotic lesions of the femoral head has been developed using magnetic resonance imaging scans. With this method it will be easier to determine which early lesions require core decompression, or which ones should be treated conservatively. Osteomyelitis can be differentiated from bone infarction with the use of segmental radionuclide bone-marrow and bone scans. Reduction in frequency of painful crises can be achieved by increasing fetal hemoglobin with the use of hydroxyurea. The treatment of the actual pain requires decisions about the analgesics that are used as well as the route of their administration. Ketorolac monotherapy is likely to fail in the presence of an initial high pain score or with involvement of four or more pain sites.

ISSN:1040-8711    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Musculoskeletal problems remain among the main limitations of the quality of life of renal failure patients, in particular of those treated with long-term maintenance dialysis. Renal osteodystrophy continues to receive great attention. The mechanisms of uremic skeletal resistance to parathormone (PTH) are further investigated. The assay used for the dosage of “intact PTH” has been found to detect 7-84 fragments with an inhibitory effect on the action of the whole hormone. A decrease in the density of PTH receptor on osteoblasts is another recently evidenced factor. Investigations of the recently described RANK-RANKL system have demonstrated an increase in serum osteprotegerin levels, which, together with the two above-mentioned abnormalities, may explain bone resistance to PTH. These are important advances in the understanding of renal osteodystrophy as skeletal resistance to PTH appears to play an important part in the pathophysiology of secondary hyperparathyroidism and of adynamic bone disease. Because of this skeletal resistance, it has been recommended for several years that serum PTH level be monitored and kept twofold to threefold above the upper value of the normal level to maintain normal bone turnover in dialysis patients. Relative hypoparathyroidism has recently been found to be associated with increased spontaneous fracture rate and mortality, so this recommendation appears to hold adequate, despite the demonstration that serum PTH levels in this range are a poor predictor of bone turnover and that chronic parathyroid gland hyperplasia is likely to favor parathyroid gland autonomization. Recent publications have insisted on the role that hyperphosphatemia plays not only in the development of secondary hyperparathyroidism, but also of vascular, especially coronary, calcification and as a predictor of mortality. This “silent killer” of uremic patients is one of the main targets for therapeutic intervention. Extensive use of calcium-containing phosphate binders has been recently criticized as calcium overload appears to favor vascular calcification. Sevelaner (RenaGel) is a calcium- and aluminum-free phosphate binder that is an important advance in the management of renal osteodystrophy, especially in patients with extraskeletal calcification and hypercalcemia. The use of vitamin D derivatives has also raised concern because they enhance calcium and phosphorus absorption and reduce bone turnover. New metabolites with fewer hypercalcemic effects have been developed. Calcium-sensing receptor agonists are stimulating interest and are likely to take an important place in the future management of renal osteodystrophy. Uremic myopathy has received recent attention. Impaired muscle capillary oxygen transfer has been identified as a pathophysiologic factor, and progressive resistance training has been shown to improve the condition. Finally, a new entity, nephrogenic fibrosing dermopathy, has been described, which must be distinguished from calciphylaxis and scleromyxedema.

ISSN:1040-8711    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Deficiency of an early component of the classical complement pathway, C1q, C1r/C1s, C4, or C2, regularly produces autoimmunity in man, especially systemic lupus erythematosus. It has long been suggested that disruption of this pathway would lead to the inappropriate handling of immune complexes. An intriguing hypothesis that builds on this idea relates to a defect in clearance of one's own cellular debris, namely apoptotic cells. An attractive feature of this emerging concept is that blebs on apoptotic cells are decorated with antigens to which much of the autoantibody specificity is directed in systemic lupus erythematosus. A second hypothesis, generated primarily from complement deficiencies, relates to an impairment in the humoral immune response or in the regulation of autoreactive B cells. This review begins by summarizing the recognized autoimmune manifestations of complement deficiency and then describes new data derived from targeted gene deletions of complement proteins.

ISSN:1040-8711    

出版商:OVID    

年代:2003

数据来源: OVID