ISSN:1040-8711    

出版商:OVID    

年代:1994

数据来源: OVID

摘要:

Vasculitis is a clinicopathologic process that involves inflammation and necrosis of blood vessels, resulting in a wide range of clinical diseases. The pathogenesis of vasculitis has been attributed to immunologic mechanisms, including immune complexes, cellular immunity, and humoral immunity, with numerous inciting events such as infection, drugs, malignancy, or toxins. Inflammatory cytokine production and adhesion molecule activation or upregulation are important determinants of the pathogenic inflammatory responses noted in vasculitis. Endothelial cells may be targeted by anti-endothelial cell antibodies and are central targets of numerous proinflammatory cytokines in vasculitis pathogenesis. Finally, antineutrophil cytoplasmic antibodies (ANCA) and T-cell responses to the protein targets of ANCA may play a role in vessel damage in ANCA-associated vasculitis.

ISSN:1040-8711    

出版商:OVID    

年代:1994

数据来源: OVID

ISSN:1040-8711    

出版商:OVID    

年代:1994

数据来源: OVID

摘要:

Although precise diagnosis of the systemic vasculitides can provide general prognostic information and help to guide initial therapy, recent studies on the long-term clinical course have revealed considerable variation in clinical severity. Therefore, anatomic distribution of involvement and speed of progression should be the principle determinants of the intensity of immunosuppressive therapy. In progressive pulmonary or renal disease,eg, Wegener's granulomatosis, aggressive “standard” therapy is obligatory,eg, daily cyclophosphamide and glucocorticoids. Such regimens, however, should be applied with caution in chronic or indolent and abortive forms of systemic vasculitis, because follow-up studies (eg, in Wegener's granulomatosis) have revealed treatment-associated morbidity rates of up to 42%, disease-related morbidity, and a high incidence of relapse under treatment. Moreover, less toxic therapeutic strategies are being pursued with remarkable success: low-dose weekly methotrexate, monthly intravenous or oral pulses of cyclophosphamide plus glucocorticoids, and high-dose intravenous immuno-globulin. Long-term remission of intractable (non-antineutrophil cytoplasmic antibody-associated) systemic vasculitis has been achieved using humanized monoclonal antibodies (ie, anti-CD4/anti-CDw52); and amelioration of glomerulonephritis in immune complex diseases (eg, systemic lupus erythematosus) has been achieved with nafamostat mesilate, an inhibitor of complement serine proteases. In addition, leukocytoclastic vasculitis has been effectively controlled with pentoxifylline, presumably by neutralizing proinflammatory cytokines, and hepatitis C virus-associated mixed cryoglobulinemia has been successfully treated with interferon alfa.

ISSN:1040-8711    

出版商:OVID    

年代:1994

数据来源: OVID

摘要:

Cutaneous vasculitis and vasculopathic processes continue to be difficult to define, diagnose, and treat. Historically, the complexity of these disorders has been compounded by imprecision in terminology and classification and the presence or absence of underlying systemic illness. Approaching the literature with consistent diagnostic constraints and accepted terminology can, it is hoped, eliminate some of the ambiguity. During the past year, several case reports and brief communications regarding cutaneous vasculitis or vasculopathies have appeared, as well as thoughtful basic science reports whose authors have attempted to further the understanding of the underlying pathophysiology. Recent technologic advances have produced recombinant cytokines, growth factors, and thrombolytics that have been used therapeutically for a variety of medical illnesses. The role of these agents in the treatment of cutaneous vasculitis and vasculopathies has varied from provocative in some to therapeutic in others. A number of these reports are discussed.

ISSN:1040-8711    

出版商:OVID    

年代:1994

数据来源: OVID

摘要:

Henoch-Schonlein purpura (HSP) is the most common vasculitis syndrome of childhood. It is also known as anaphylactoid purpura, leukocytoclastic vasculitis, allergic vasculitis, and rarely, as rheumatoid purpura. It is generally a benign, self-limited disorder that follows an intercurrent illness, usually of the upper-respiratory tract. The classic triad of clinical symptoms and signs includes purpuric rash, abdominal cramping, and hematuria, but the spectrum of the clinical expression of HSP may vary from only minimal petechial rash to severe gastrointestinal, renal, neurologic, pulmonary, and joint disease. Most children have self-limited disease, and on long-term follow-up, systemic involvement or serious sequelae are not frequent. The current lack of knowledge about the factors underlying both the etiology and pathogenesis and the extent of clinical expression of HSP is illustrated in the recent literature. In addition, there is no agreement regarding the role of corticosteroids in the clinical management of HSP, and this subject has not received adequate attention during the past year. The recent clinical literature emphasizes the distinction between HSP and other hypersensitivity vasculitides and provides several in-depth reviews and multiple case reports illustrating the expanding clinical spectrum of the disorder. Exciting developments regarding immunologic aspects of HSP have been published and are summarized. The utility of antineutrophil cytoplasmic antibody as it applies to HSP remains to be elucidated.

ISSN:1040-8711    

出版商:OVID    

年代:1994

数据来源: OVID

摘要:

Takayasu's arteritis has become increasingly recognized as a worldwide entity with a variable spectrum of disease expression. Although evidence for immune mechanisms of disease exist, the precise etiopathogenesis remains elusive. Improvements in the evaluation of disease activity beyond the current parameters of clinical symptoms, erythrocyte sedimentation rate, and angiographic findings are needed. Assessment of disease is handicapped by radiologic techniques that may detect diseased vessels but do not distinguish active from chronic lesions. A large proportion of patients with active disease respond to treatment with glucocorticoids, but a significant proportion will either require additional immunosuppression to prevent steroid toxicity or will remain unresponsive to treatment. At present, methotrexate appears to be an effective steroid-sparing agent in the treatment of active Takayasu's arteritis. Surgery continues to play a critical role in the long-term management of Takayasu's arteritis and is recommended when appropriate during inactive disease. Renal autograft transplantation has been offered as a new technique in the management of hypertension in Takayasu's arteritis.

ISSN:1040-8711    

出版商:OVID    

年代:1994

数据来源: OVID

摘要:

The International Study Group's diagnostic criteria for Behçet's disease are gaining acceptance and may be further refined before the decade is over. Previously neglected clinical features of the disease, especially arterial aneurysms and phlebitis, are increasingly reported. Each poses a dilemma in the choice of treatment, which ranges from anticoagulant to immunosuppressant regimens, old and new. T-lymphocyte abnormalities, both in subpopulation and function, suggest ongoing presentation of an unknown antigen. Research on the role of HLA-B51, a split of B5, is moving centromeric to it in pursuit of a better disease marker. The choice of immunosuppressive therapy in severe disease such as uveitis and arteritis is between alkylating agents and agents that inhibit interleukin-2 production.

ISSN:1040-8711    

出版商:OVID    

年代:1994

数据来源: OVID

摘要:

Thromboangiitis obliterans (Buerger's disease) is an inflammatory obliterative, nonatherosclerotic, vascular disease that affects the small-and medium-sized arteries, veins, and nerves. It is causally related to tobacco use, although the exact mechanism is unknown. Its clinical presentation is manifested by distal arterial ischemia and superficial thrombophlebitis. Thromboangiitis obliterans usually becomes quiescent if the patient is able to stop smoking cigarettes. However, if smoking continues, amputation commonly results.

ISSN:1040-8711    

出版商:OVID    

年代:1994

数据来源: OVID

ISSN:1040-8711    

出版商:OVID    

年代:1994

数据来源: OVID