ISSN:1040-8711    

出版商:OVID    

年代:1992

数据来源: OVID

摘要:

The Ipr gene has been identified as a defect in the fas gene, which encodes a lymphocyte surface protein associated with apoptosis and shares homology with the tumor necrosis factor-a receptor. This finding is important as it may quickly lead to identification of the gld gene product, which is thought to be a ligand for Fas. Also, it clearly identifies autoimmune disease as originating from a defect in the ability to induce cell death in lymphocytes. The major challenge in the future will be to directly demonstrate the relationship of abnormal apoptosis pathways to the development of autoimmunity and, in the case of Ipr and gld mice, to lymphadenopathy, and to eventually determine if this is a fundamental defect at the root of all autoimmune diseases in both mice and humans.

ISSN:1040-8711    

出版商:OVID    

年代:1992

数据来源: OVID

摘要:

Animal models of autoimmunity are used to identify important immunologie factors and pathways involved in the different autoimmune diseases. Identification of the role of cytokines and development of new disease models have led to new possibilities for treatment.

ISSN:1040-8711    

出版商:OVID    

年代:1992

数据来源: OVID

摘要:

B-cell hyperactivity is characteristic of lupus and appears to be, in many instances, T-cell driven. Recent work continues to examine the paradox of T-cell activation in vivo and depressed T-cell functionin vitro. The role of intrinsic B-cell abnormalities, particularly in CD5+B cells, is an area of active investigation.

ISSN:1040-8711    

出版商:OVID    

年代:1992

数据来源: OVID

摘要:

Antinuclear antibodies occur prominently in systemic lupus erythematosus and serve as markers of underlying pathogenetic disturbances. Although these antibodies display features indicative of genetic control andin vivoselection by self-antigen, other mechanisms shaping the B-cell repertoire may influence their production. Provocative new animal models provide systems for analyzing the cellular and genetic disturbances promoting these responses, as well as the role of pathogenic specificities in inducing tissue injury.

ISSN:1040-8711    

出版商:OVID    

年代:1992

数据来源: OVID

摘要:

Refinements in molecular genetic technology as well as in the organization of the major histocompatibility complex and the genes contained therein continue to lead the way to elucidation of the immunogenetics of systemic lupus erythematosus and Sjögren's syndrome. In this article, recent advances in these areas are reviewed, and major histocompatibility complex associations with systemic lupus erythematosus and Sjögren's syndrome will be explored with particular emphasis on autoantibody subsets of these diseases. Data thus far support the hypothesis that systemic lupus erythematosus and Sjögren's syndrome are clinically and serologically heterogeneous disorders of major histocompatibility complex class II allele-associated autoantibody subsets, to which other major histocompatibility complex genes (C4 null alleles) and non-major histocompatibility complex genes (such as T-cell receptor genes) may contribute in susceptibility.

ISSN:1040-8711    

出版商:OVID    

年代:1992

数据来源: OVID

摘要:

Sjögren's syndrome is an autoimmune exocrinopathy with characteristics of both organ-specific and generalized autoimmune disease. The main features are keratoconjunctivitis sicca and xerostomia, but patients may also show signs of extraglandular involvement. Sjögren's syndrome can occur in the absence (primary Sjögren's syndrome) or in the presence of other connective tissue disease (secondary Sjögren's syndrome). Sjögren's syndrome has often been considered a link in the spectrum between autoimmune disease and lymphoproliterative disorders. The cause of Sjögren's syndrome is still unknown, although the role of viruses in the pathogenesis is disputed. There is still no consensus about the diagnostic criteria, due to lack of specificity and sensitivity of the diagnostic procedures performed thus far. Recent studies using immunohistochemical and molecular biologic techniques highly suggest that disturbances in the target organs, such as salivary glands, are the consequence of local processes, rather than the reflection of systemic alterations. This underlines the importance of implementing these techniques for diagnostic purposes. Based on increasing understanding of the pathogenetic processes, new therapeutic possibilities will come into the picture. Over the past few years, interesting studies have contributed to our knowledge of the disease.

ISSN:1040-8711    

出版商:OVID    

年代:1992

数据来源: OVID

摘要:

Over the past year, many reports have been published on a variety of clinical manifestations related to antiphospholipid antibodies. The low prevalence of anticardiolipin antibodies with the rare occurrence of thrombosis and a low rate of fetal loss in studies in Malaysia and China showed a potential role for local factors. A study of cross-reactive idiotype of the anticardiolipin antibody suggested that anticardiolipin antibodies are derived from a set of natural autoreactive clones. Regarding the pathogenic role of the antiphospholipid antibody, evidence has been presented that the epitopes formed between cardiolipin and β2glycoprotein I are the targets of the antiphospholipid antibody. Complement activation, abnormalities of natural anticoagulants such as protein S deficiency, and genetic association with DR4, DR7, and DRw53 have also been studied.

ISSN:1040-8711    

出版商:OVID    

年代:1992

数据来源: OVID

摘要:

Studies of renal involvement in systemic lupus erythematosus continue to dominate the clinical literature. Reports of the prognostic significance of both clinical and histologie parameters at the time of renal biopsy are discussed. Renal vascular as well as glomerular lesions are considered. The potential impact of anticardiolipin antibodies on the development of renal insufficiency is described. The outcome of renal transplantation in patients with systemic lupus erythematosus is assessed in a study concerned with both allograft survival and recurrence of active nephritis in the transplanted kidney. The incidence and prognosis of various features of neuropsychiatrie systemic lupus erythematosus are discussed, while the search for an accurate indicator of lupus involvement of the central nervous system continues. Magnetic resonance imaging and single-photon-emission computed tomography are considered. Abnormalities of pulmonary gas exchange are featured in several reports. Features of the antiphospholipid antibody syndrome are presented, stressing predisposition to thrombosis. The definition and characteristics of systemic lupus erythematosus disease flares is discussed, in relation to several recently developed disease activity indexes. The attempt to distinguish systemic lupus erythematosus activity from infection and preeclampsia is also considered. Finally, the association of systemic lupus erythematosus with the development of cancer is discussed.

ISSN:1040-8711    

出版商:OVID    

年代:1992

数据来源: OVID

摘要:

The prognosis of systemic lupus erythematosus has improved over the past four decades. We now have data for 15-year survival, which has also improved. Several factors may be associated with improved survival, including earlier diagnosis, better treatment, and improved medical therapy. Specific organ damage continues to be an issue, primarily with regard to kidney disease and to neurocognitive impairment and lung involvement. Infection and vascular disease emerge as important factors. With improved survival, other outcome measures, including specific organ function and health status, need to be considered.

ISSN:1040-8711    

出版商:OVID    

年代:1992

数据来源: OVID