ISSN:1040-8711    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

A diagnosis of Wegener granulomatosis requires granulomatous manifestations in the respiratory tract. With the increasing use of antineutrophil cytoplasmic autoantibodies as a diagnostic tool, Wegener granulomatosis is diagnosed earlier than in the past, and not infrequently when only ear, nose and throat manifestations are present, placing the otorhinolaryngologist in a central role in diagnosis and management. Diagnostic biopsies should be obtained from active lesions in the nose and paranasal sinuses and concomitant infection should be identified. Because of the apparent relation between infection and activation of disease, the management of infections—especially those due toStaphylococcus aureus—requires special attention. The increasing numbers of early cases identified warrants further investigations of whether less toxic treatment regimens will be of advantage in such cases. Medical and surgical treatment of the acute and chronic manifestations presents specific problems because of altered immune competence, prevalent superinfection, and tissue destruction, and is therefore best taken care of by specially dedicated otorhinolaryngologists.

ISSN:1040-8711    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Diffuse alveolar hemorrhage (DAH) is a rare yet serious and frequently life-threatening complication of a variety of conditions. DAH may result from coagulation disorders, inhaled toxins, or infections. Most cases of DAH are caused by capillaritis associated with systemic autoimmune diseases such as antineutrophil cytoplasmic antibodies-associated vasculitis, anti–glomerular basement membrane disease, and systemic lupus erythematosus. Early recognition is crucial, because the prompt institution of supportive measures and immunosuppressive therapy is required for survival. Our understanding of DAH and its management is largely empiric and based on small case series and individual reports, many dating back more than one decade. To provide the practicing specialist with a rational diagnostic and management approach to the patient with DAH, this review summarizes the most recent publications and salient information derived from older publications.

ISSN:1040-8711    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Central nervous system involvement in Behçet disease (BD) may be either parenchymal or nonparenchymal. Abnormal cerebrospinal fluid findings and parenchymal involvement are associated with a poorer prognosis. A self-antigenic role for HLA B51 has been postulated in the pathogenesis of BD. The sibling occurrence rate is 3.6%. Familial aggregation may be more pronounced among pediatric cases compared with adult cases. The importance of inherited coagulation abnormalities in the pathogenesis of BD is not clear. Vasculitis of the vasa vasorum seems to be the major site of pathology in large vessel disease. Even among experts, no consensus exists regarding the optimal approach to treatment. Low-dose thalidomide is effective for the mucocutaneous lesions, but polyneuropathy complicates its prolonged use. Neurologic side effects of cyclosporine A should preclude its use in patients with central nervous system involvement except in unusual circumstances. The heightened inflammatory response of BD patients to simple trauma may lead to postoperative complications, but should not be regarded as a contraindication to surgery.

ISSN:1040-8711    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Although the classification of small-and medium-vessel vasculitides involving the skin remains complex and imperfect, a substantial body of knowledge related to cutaneous vasculitis exists. Some components of this knowledge, though beyond the usual purview of rheumatologists, are essential to understanding the vasculitides. The correct interpretation of cutaneous findings combined with a properly performed skin biopsy yields important insights not only into underlying diagnoses, but also into the pathophysiological mechanisms of individual cases. In this review, we discuss a classification scheme for the cutaneous vasculitides, outline the work-up for evaluating patients with these disorders, and describe the essential features of the major categories of skin vasculitis.

ISSN:1040-8711    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Although Henoch-Schönlein purpura (HSP) can occur at any age from infancy to adulthood, it is overwhelmingly a disease of childhood. Indeed, HSP is the most common vasculitis syndrome affecting children. The clinical features of HSP have been well documented, and the diagnosis is generally not difficult. However, there are substantial gaps in our understanding of the etiology, pathogenesis, and treatment of HSP. This article briefly reviews the clinical aspects of HSP and new information concerning therapy. The major focus of this review is recent information concerning abnormalities of immunoglobulin A1 glycosylation and the role of aberrantly glycosylated immunoglobulin A1 in the pathogenesis of HSP.

ISSN:1040-8711    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Conventional x-ray angiography has traditionally played a prominent role in the radiologic evaluation of large vessel vasculitis. Recently, noninvasive magnetic resonance (MR) methods have received much attention for their potential to complement and possibly supplant conventional angiography. In particular, promising advances have been made in the areas of MR angiography and physiologic imaging. MR provides high resolution anatomic information, including lumen configuration and vascular wall thickness, and physiologic data, such as measurements of the degree of wall enhancement and the presence of edema. Moreover, imaging data are obtained noninvasively in a multitude of obliquities. Owing to these features, MR occupies a unique role in the evaluation of large vessel vasculitis.

ISSN:1040-8711    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Over the last fifteen years, collaborative vasculitis studies in Europe have established the association of antineutrophil cytoplasmic antibodies (ANCA) with vasculitis and defined its clinical role as a diagnostic tool. More recently, the European Vasculitis Study Group has developed a consensus approach to the treatment of vasculitis and has aimed to harmonize and optimize current therapy by a series of multicenter randomized controlled trials. The first trial to report, CYClophosphamide or AZathioprine As a REMission therapy for vasculitis (CYCAZAREM), demonstrated that, for generalized vasculitis, azathioprine is as effective as continued cyclophosphamide for the maintenance of remission. Other trials are investigating the roles of methotrexate, plasma exchange, and pulse cyclophosphamide in acute disease, and strategies to prevent relapse over the longer term. Parallel studies are determining prognostic markers, evaluating tools for monitoring disease, and examining the association of ANCA and infection with relapse. The established infrastructure and accumulated database are well placed to facilitate testing of newer therapeutic strategies in the future.

ISSN:1040-8711    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Eosinophilia is occasionally a feature of rheumatic disease. The differential diagnosis of eosinophilia includes parasitic infection, systemic vasculitides, eosinophilic arthritis, and myopathies, together with the idiopathic hypereosinophilic syndrome and malignancy. Careful evaluation of the patient should enable an accurate diagnosis to be made. Parasitic infection is the commonest cause of eosinophilia worldwide and can cause systemic disease, as illustrated by the report ofSarcocystismyositis in a group of military personnel in Malaysia. A persistent arthropathy associated with eosinophilia, but not with parasitic infection, has been reported from the far East. Drugs may also cause eosinophilia, and there has recently been much discussion of the relation between Churg-Strauss syndrome and the leukotriene antagonist zafirlukast. The present view is that reduction of steroid dose allows unmasking of previously undiagnosed Churg-Strauss syndrome. The idiopathic hypereosinophilic syndrome may represent a lymphoproliferative process; evidence for this comes from the demonstration that many patients have a clonally expanded population of aberrant T cells.

ISSN:1040-8711    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Rheumatic disorders associated with cancer include a variety of conditions, most of which have no features distinguishing them from idiopathic rheumatic disorders. It is generally held that an extensive search for occult malignancy in most rheumatic syndromes is not recommended unless the case is accompanied by specific findings suggestive of malignancy. Within the past year information has accumulated on the role of long-standing rheumatic disorders as premalignant conditions and the role of autoantibodies as screening tests for occult cancer. The present article discusses cancer-associated rheumatic syndromes, calls attention to aspects that may suggest the presence of a hidden cancer, and examines the role of laboratory tests as clues of a possible neoplastic etiology of those syndromes.

ISSN:1040-8711    

出版商:OVID    

年代:2001

数据来源: OVID