ISSN:1040-8711    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Wegener’s granulomatosis is a multisystem disease characterized by granulomata of the respiratory tract and systemic necrotising vasculitis. There is a strong and specific association with autoantibodies directed against proteinase 3, a constituent of neutrophril azurophilic granules. Antibody titers correlate with clinical disease activity and predict relapses. The disease responds favorably to immunosuppressive therapy. The pathogenicity of antineutrophil cytoplasmic antibodies (ANCA), however, remains unproven.In vitro, the expression of proteinase-3 and other ANCA antigens on the surface of neutrophils and monocytes can be induced by priming with proinflammatory cytokines. Antineutrophil cytoplasmic antibodies are then able to activate these leukocytes, stimulating degranulation, the production of reactive oxygen species, and the secretion of further cytokines. Neutrophils activated by ANCA, and possibly ANCA alone, directly damage endothelial cellsin vitro. An animal model of proteinase 3-ANCA-induced vasculitis has not been found. Antineutrophil cytoplasmic antibodies directed against another antigen, myeloperoxidase, are not sufficient to cause vasculitis but they promote damage in certain animal models. Thus, a considerable amount of evidence supports the notion that Wegener’s granulomatosis is an autoimmune disease.

ISSN:1040-8711    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Several rodent models have been proposed for various forms of systemic vasculitis. The MRL-lprmouse has been studied extensively as a model for systemic lupus erythematosus. Backcross experiments in combination with genetic linkage studies have firmly established that the phenotype of autoimmune disease is dependent on the combination of various background genes. It has also become apparent that environmental factors, particularly infections, modulate the disease phenotype. Specific interventions, such as the treatment of Brown Norway rats with agents resulting in polyclonal B cell stimulation or immunization with human myeloperoxidase and subsequent localized perfusion with neutrophil lysosomal extract and H2O2, have provided substantial insights into the cellular and molecular mechanisms leading to the development of vasculitis and glomerulonephritis. Even though the existing models may not exactly mirror any specific human disease, they offer reproducible, highly controlled conditions to answer specific questions about pathogenesis and novel therapeutic approaches.

ISSN:1040-8711    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Information detailing the socioeconomic impact of the vasculitides on society has been difficult to obtain. Recent published studies provide preliminary information suggesting that the impact of systemic vasculitis may be significant and far greater than previously anticipated. A rough estimate of expenditures for vasculitis-related hospitalizations for polyarteritis nodosa, hypersensitivity vasculitis, Wegener’s granulomatosis, giant cell arteritis and Takayasu’s arteritis in the US amounted to $150 million per year. Costs associated with outpatient care, disability, and death were not calculated. A more comprehensive investigation into the economic and social burden of these conditions is necessary. Aspects to consider include quantifying the incidence and prevalence of disease, estimating the direct, indirect, and intangible costs of care, assessing long-term clinical outcomes, and measuring quality of life from the patient perspective. Curr Opin Rheumatol 2000, 12:20–23 © 2000 Lippincott Williams & Wilkins, Inc.

ISSN:1040-8711    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Chronic or recurrent urticarial lesions are common in both primary care and referral medicine. Diagnosis and treatment are usually a challenge for both the patient and the medical practitioner. Most patients are eventually diagnosed with chronic idiopathic urticaria. IgG autoantibody to IgE receptor or IgE itself causes urticarial lesions in 30% of these patients. Only a minority (approximately 10%) of patients with chronic urticarial lesions have urticarial vasculitis. Although some cases are benign, urticarial vasculitis by itself can cause significant morbidity, and it is often a manifestation of a serious illness. Successful diagnosis and treatment of urticarial vasculitis requires careful assessment over time for underlying diseases like systemic lupus erythematosus, hypocomplementemic urticarial vasculitis syndrome, Sjögren’s syndrome, and mixed cryoglobulinemia.

ISSN:1040-8711    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Immune-mediated inner ear disease (IMIED) is a syndrome that includes the subacute onset of sensorineural hearing loss, often accompanied by vertigo and tinnitus. This constellation of symptoms may occur as a primary disorder in which no other organ involvement is evident, or it may complicate certain systemic conditions, including Wegener’s granulomatosis, Cogan’s syndrome, polyarteritis nodosa, and systemic lupus erythematosus. The precise disease mechanisms remain undefined, largely because of the difficulty obtaining relevant tissue specimens in untreated patients. However, if treated promptly with aggressive immunosuppression, the devastating sequelae of IMIED may be avoided. In this article, we review the pathophysiology, clinical evaluation, diagnostic testing, and therapy of IMIED.

ISSN:1040-8711    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Fibromuscular dysplasia is an uncommon angiopathy that occurs in young to middle-aged, predominately female individuals. The disease consists of a heterogeneous group of histologic changes, which ultimately lead to arterial narrowing. Clinical manifestations reflect the arterial bed involved, most commonly hypertension (renal) and stroke (carotid). Fibromuscular dysplasia is a pathologic diagnosis, but the characteristic changes seen on an angiogram can be used to make the diagnosis in the appropriate clinical setting. This noninflammatory disease is a common mimic of vasculitis. A very limited amount of new literature has been published in the past year about this relatively uncommon condition.

ISSN:1040-8711    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Autoimmune disease, in particular systemic lupus erythematosus (SLE), can be induced by drugs. Over the past couple of years biologic agents have become available for the treatment of inflammatory disease; simultaneously, researchers have realized that these drugs can not only suppress autoimmune disease but may also potentiate it. Interferon-&agr; and interferon-β both may induce autoimmune disease, but this is more frequent with interferon-&agr;. Therapy to block tumor necrosis factor-&agr;, either with monoclonal antibodies or fusion proteins, has been associated with the development of antinuclear antibodies, but only rarely with clinical development of SLE. None of the three reported cases of SLE occuring after anti-tumor necrosis factor-&agr; therapy has developed major organ involvement. The continued use of biologic agents will provide interesting insights into the pathogenesis of autoimmune disease.

ISSN:1040-8711    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Mixed cryoglobulinemia (MC) is a systemic vasculitis of small to medium-sized vessels due to the vascular deposition of circulating immune-complexes (CIC) and complement. A leukocytoclastic vasculitis is the histologic hallmark of cutaneous manifestations of the disease, while a clonal B lymphocyte expansion in blood, bone marrow, liver, and spleen represents the underlying pathologic alteration responsible for the production of cryo-CIC and non-cryo CIC with rheumatoid factor activity.A causative role of hepatitis C virus (HCV) infection has been demonstrated in the large majority of MC patients. Hepatitis C virus is both a hepatotropic and a lymphotropic virus; due to this latter biological peculiarity, HCV may trigger a constellation of autoimmune-lymphoproliferative disorders. Besides MC, other important HCV-related diseases are porphyria cutanea tarda, autoimmune hepatitis, membranoproliferative glomerulonephritis, and B cell neoplasias. Hepatitis C virus-related MC represents a link between autoimmune and lymphoproliferative disorders; moreover, MC is an important model to study the complex relation between infections and immune system alterations in humans. During the last years many other autoimmune manifestations have been correlated with HCV infection; namely, sicca syndrome, chronic polyarthritis, polydermatomyositis, fibromyalgia, autoimmune thyroiditis, lung fibrosis, and diabetes mellitus. It is often difficult to verify whether the above associations are coincidental or a pathogenetic link actually exists. At least for particular patients’ subsets and in some geographic areas, a causative role of HCV seems to be likely. The geographically heterogeneous distribution of HCV-related autoimmune diseases suggests the contribution of important environmental and genetic factors in the pathogenesis of such conditions. In clinical practice, patients with recent-onset, atypical rheumatic and autoimmune disorders should be carefully investigated for possible HCV infection; this is particularly advisable for correct diagnosis and adequate therapeutic strategy.

ISSN:1040-8711    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Familial Mediterranean fever (FMF) is the most prevalent type of hereditary recurrent fever. Although the inflammatory attacks that characterize the disease may sometimes be debilitating, reactive amyloidosis remains the most serious manifestation of FMF. Daily treatment with colchicine can prevent both the attacks and amyloid deposition, but FMF-associated amyloidosis has not been eradicated and is still a cause of chronic renal failure in children and adults. The discovery of the gene responsible for FMF, Mediterranean fever gene (MEFV), and of associated mutations represents a major advance that now allows researchers to establish a strong, although nonexclusive association between one specific mutation, M694V, and the amyloid phenotype.

ISSN:1040-8711    

出版商:OVID    

年代:2000

数据来源: OVID