ISSN:1040-8711    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

&NA;The current lack of knowledge about the factors underlying both the etiopathogenesis and extent of the clinical expression of necrotizing vasculitis is illustrated by recent articles. “Benign” vasculitis may present as localized disease in skin, gut, or even muscle; but on long‐term follow‐up, systemic involvement frequently occurs. Exciting studies on the occurrence of viruses such as hepatitis C in vasculitis compound these problems, because they are associated with multiple syndromes and the mechanism underlying their relationship to vasculitis remains to be elucidated. Further subdivision of antineutrophil cytoplasmic antibodies may aid in classification, but currently, their association with disease activity and extent obscures that role. Age is also less useful, because Kawasaki syndrome may occur rarely in adults and polyarteritis nodosa was reported in a large series of children. Differentiation is important because of the various responses to therapy. The first controlled study of cyclophosphamide as therapy for necrotizing vasculitis confirms its early benefit but, interestingly, shows no difference in 10‐year survival rates. The addition of plasma exchange is of benefit only in the small number of patients with severe renal involvement who are dialysis‐dependent at onset.Current Opinion in Rheumatology 1993,5:5‐10

ISSN:1040-8711    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

&NA;In recent years, interest in Wegener's granulomatosis has been stimulated by an increasing appreciation of the chronic relapsing nature of this disease and its association with antibodies to proteinase 3. Although conventional therapy with cyclophosphamide and glucocorticoids has produced remission in most patients, remission may not occur immediately and, in at least 50% of patients, may be followed by relapse. As a result, most patients experience some form of permanent morbidity from disease or treatment, or both. These observations have led to renewed efforts to identify more effective and less toxic therapies. Preliminary studies have evaluated other cytotoxic agents, such as methotrexate, and biologic products, such as high‐dose immunoglobulin and monoclonal antibodies. It is hoped that a better understanding of the possible pathogenic role of anti‐proteinase 3 antibodies may contribute to improved therapy. Unfortunately, research is handicapped by lack of an animal model, without which it will be difficult to prove convincingly that anti‐proteinase 3 antibodies are important in expressing disease.Current Opinion in Rheumatology 1993,5:11‐17

ISSN:1040-8711    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

&NA;The diagnosis and classification of vasculitis has been revolutionized by the discovery and characterization of serum antineutrophil cytoplasmic autoantibodies. These autoantibodies are highly specific, objective markers for the major subset of vasculitis that includes Wegener's granulomatosis, polyarteritis nodosa (microscopic polyangiitis), Churg‐Strauss syndrome, primary or idiopathic pauciimmune necrotizing and crescentic glomerulonephritis with or without pulmonary hemorrhage, as well as some poorly characterized and overlapping forms of these vasculitides. The finding of antineutrophil cytoplasmic antibodies throughout this group identifies these syndromes as a single category or spectrum of disease. The sensitivity of antineutrophil cytoplasmic antibodies for this group of conditions is high when there is systemic involvement, as defined by the presence of renal involvement. However, the antibodies are only moderately sensitive markers in limited or localized cases of these vasculitides (without renal involvement), and hence diagnosis of these conditions based on histologic and clinical criteria remains important. Two significant types of antineutrophil cytoplasmic antibodies have been identified: anti‐proteinase 3 and antimyeloperoxidase antibodies. Both have proven to be of diagnostic value for the spectrum of vasculitis listed above. Remarkably, patients with this spectrum of vasculitis have only one or the other of these two types of antibodies.Current Opinion in Rheumatology 1993,5:18‐24

ISSN:1040-8711    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

&NA;Although the causes of giant cell arteritis, polymyalgia rheumatica, and Takayasu's arteritis are not known, the immune system participates in the pathogenesis of these disorders. Many reports indicate that cellular immunity is active in giant cell arteritis. The diagnosis of Takayasu's arteritis has been advanced by magnetic resonance imaging and ultrasonography. Neither technique has replaced angiography as the gold standard for diagnosis, but both may help monitor the response of Takayasu's arteritis to therapy. The diagnosis of giant cell arteritis with a low erythrocyte sedimentation rate is unusual but is more frequent in patients with a history of polymyalgia rheumatica or corticosteroid use. One recent retrospective study suggests that 20 mg/d of prednisolone is as effective as higher doses in treating giant cell arteritis. Other preliminary studies indicate that methotrexate may effectively spare corticosteroid use in giant cell arteritis and Takayasu's arteritis. Angioplasty for renal artery stenosis in Takayasu's arteritis initially cures 30% of patients and improves the condition in 70%; however, the long‐term success rate is 50%. New studies demonstrate that patients with giant cell arteritis and polymyalgia rheumatica have an increased risk of hypothyroidism.Current Opinion in Rheumatology 1993,5:25‐32

ISSN:1040-8711    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

&NA;During 1991 and 1992 there was an abundance of articles dealing with vasculitis and its cutaneous manifestations, as well as with the perhaps related disorders known as neutrophilic dermatoses. This brief review will deal with some of the more controversial areas. The specific issues discussed are those relating to the “new” American College of Rheumatology classification system and its application to patients who have skin lesions of vasculitis, the recently associated diseases, and the newer therapies for vasculitis. In addition, recent publications regarding acute neutrophilic dermatosis (Sweet's syndrome) and pyoderma gangrenosum are briefly discussed.Current Opinion in Rheumatology 1993,5:33‐40

ISSN:1040-8711    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

&NA;Kawasaki disease is an acute vasculitis of young children that is complicated by the development of myocarditis and coronary artery abnormalities. Recent studies indicate that the prevalence of cardiovascular abnormalities can be significantly reduced by treating patients during the first 10 days of illness with high‐dose intravenous gammaglobulin, particularly at a dose of 2 g/kg. Thus, early recognition and prompt treatment of this illness is critical for a successful outcome. This process would undoubtedly be facilitated if the etiologic agent or toxin that causes Kawasaki disease were known. In this regard, studies of the past year strongly suggest that a superantigen plays an important role in stimulating the massive immune activation associated with this illness. These observations may provide an important new direction for investigations into the etiology and pathogenesis of Kawasaki disease.Current Opinion in Rheumatology 1993,5:41‐50

ISSN:1040-8711    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

&NA;Behçet's disease is a chronic, relapsing, inflammatory process with clinical features of aphthous oral and genital ulcerations, uveitis, cutaneous vasculitis, arthritis, and central nervous system involvement. Large‐vessel vasculitis, thrombophlebitis, and gastrointestinal ulcerations may occur. As in other rheumatologic diseases, a genetic predisposition coupled with a triggering event leads to alterations in immune function and response, with variable clinical disease manifestations. Mononuclear cell activation and cytokine release, neutrophil chemotaxis, and humoral immune response with elevations of circulating immune complexes contribute to the pathogenesis of Behçet's disease. Vasculitis and vascular involvement are highlighted. There is no single diagnostic test for Behçet's disease. Treatment is comprised of anti‐inflammatory and immunosuppressive agents and, in selected individuals, anticoagulation. Isolated central nervous system vasculitis or noninfectious primary (granulomatous) angiitis of the central nervous system is reviewed briefly. This condition is rare, predominantly affects young women, and includes symptoms of headache, weakness, and confusion. Characteristically, erythrocyte sedimentation rate is normal, serologic studies are negative, and cerebral angiography shows multifocal and segmental vascular narrowing and beading. Typical histopathology is granulomatous in less than 50% of patients, followed by necrotizing or lymphocytic vasculitis, or both, often with thrombosis. Treatment with immunosuppressive agents improves symptoms and outcome.Current Opinion in Rheumatology 1993,5:51‐56

ISSN:1040-8711    

出版商:OVID    

年代:1993

数据来源: OVID

ISSN:1040-8711    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

&NA;Major advances of the past year in the amyloidoses include a better understanding of the polymorphism of the acute‐phase reactant serum amyloid A protein and the appearance of a new mouse model for primary amyloidosis. The list of single point mutations in transthyretin in different families with slightly varied clinical manifestations of the disease continues to grow. Gelsolin, with its asparagine 187 mutation, was found to cause amyloidosis beyond the borders of Finland, where it has been extensively evaluated. The incredible range of osteoarticular lesions due to &bgr;2‐microglobulin in hemodialysis amyloidosis continues to expand and includes severe manifestations of spondyloarthropathy. The greatest number of papers in the amyloid literature have involved amyloid &bgr; protein, amyloid &bgr; protein precursor associated with Alzheimer's disease, and prion protein associated with the spongiform encephalopathies. The widespread systemic involvement of amyloidosis has led to the appearance of a host of manifestations, some common and some rare. Treatment advances focus on the use of liver transplantation in familial amyloid polyneuropathy to remove the source of mutant protein synthesis.Current Opinion in Rheumatology 1993,5:62‐76

ISSN:1040-8711    

出版商:OVID    

年代:1993

数据来源: OVID