MD, PhD Wolfgang L. Gross (Professor of Internal Medicine and Rheumatology)
{"title":"4 Systemic necrotizing vasculitis","authors":"MD, PhD Wolfgang L. Gross (Professor of Internal Medicine and Rheumatology)","doi":"10.1016/S0950-3579(97)80046-5","DOIUrl":"10.1016/S0950-3579(97)80046-5","url":null,"abstract":"<div><p>The revival of interest in systemic necrotizing vasculitis was initiated by the discovery of its association with anti-neutrophil cytoplasmic antibodies (ANCA). The close association of certain ANCA subspecificities, for example, proteinase 3 (Pr3) and myeloperoxidase ANCA, with Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome has led to their designation as ‘ANCA-associated vasculitides’. This article describes the common and divergent clinical and immunological features of the members of this ‘new’ family of systemic necrotizing vasculitis, which continues to grow with the widespread use of ANCA testing. In addition, the ‘standard’ treatment for systemic necrotizing vasculitis (daily ‘low dose’ cyclophosphamide plus glucocorticosteroids or ‘Fauci's scheme’) is compared with new stage and activity adapted therapeutic regimens.</p></div>","PeriodicalId":77032,"journal":{"name":"Bailliere's clinical rheumatology","volume":"11 2","pages":"Pages 259-284"},"PeriodicalIF":0.0,"publicationDate":"1997-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3579(97)80046-5","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20164506","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"8 Vasculitis by organ systems","authors":"MBBS, MRCP, DGM, FACP(Hon) Kuntal Chakravarty (Consultant Rheumatologist)","doi":"10.1016/S0950-3579(97)80050-7","DOIUrl":"10.1016/S0950-3579(97)80050-7","url":null,"abstract":"<div><p>Systemic vasculitides, hitherto thought to be a rare clinical entity, are now rarely considered to be an uncommon disorder and patients are often seen between several departments, suffering from a non-infectious systemic disease with multiorgan involvement. Systemic vasculitis not only poses a major management problem but also has a significant impact on healthcare resources. The clinical outcome of a vasculitic illness depends on a number of factors, such as aetiology of the vasculitic process, site, size and number of blood vessels affected, duration and severity of the disease and also the complications associated with the disease or its therapy.</p></div>","PeriodicalId":77032,"journal":{"name":"Bailliere's clinical rheumatology","volume":"11 2","pages":"Pages 357-393"},"PeriodicalIF":0.0,"publicationDate":"1997-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3579(97)80050-7","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20164510","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
BMedSci, DM, MRCP Raashid A. Luqmani (Consultant Rheumatologist/Senior Lecturer), MA, MRCP, MRCPath, MD Andrew R. Exley (Honorary Clinical Lecturer in Immunology/Senior Registrar), PhD, MRCP George D. Kitas (Consultant Rheumatologist and Senior Lecturer in Rheumatology), FRCP Paul A. Bacon (Professor of Rheumatology)
{"title":"10 Disease assessment and management of the vasculitides","authors":"BMedSci, DM, MRCP Raashid A. Luqmani (Consultant Rheumatologist/Senior Lecturer), MA, MRCP, MRCPath, MD Andrew R. Exley (Honorary Clinical Lecturer in Immunology/Senior Registrar), PhD, MRCP George D. Kitas (Consultant Rheumatologist and Senior Lecturer in Rheumatology), FRCP Paul A. Bacon (Professor of Rheumatology)","doi":"10.1016/S0950-3579(97)80052-0","DOIUrl":"10.1016/S0950-3579(97)80052-0","url":null,"abstract":"<div><p>The improvement in survival with chemotherapy has resulted in a change of the natural history of the systemic vasculitis syndromes. The vasculitides are now viewed as chronic disease rather than fatal conditions. Their course is frequently characterized by relapse as well as the scars of irreversible organ damage from disease and drug toxicity. Assessment tools are available which can serve as outcome measures in clinical trials as well as a guide to better management of individual patients. Improvements in therapy in future are dependent on a better understanding of the pathogenesis of these conditions and the ability to assess disease accurately.</p></div>","PeriodicalId":77032,"journal":{"name":"Bailliere's clinical rheumatology","volume":"11 2","pages":"Pages 423-446"},"PeriodicalIF":0.0,"publicationDate":"1997-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3579(97)80052-0","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20164512","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
MD, PhD Ferdinand C. Breedveld (Professor of Rheumatology, Head of Department)
{"title":"6 Vasculitis associated with connective tissue disease","authors":"MD, PhD Ferdinand C. Breedveld (Professor of Rheumatology, Head of Department)","doi":"10.1016/S0950-3579(97)80048-9","DOIUrl":"10.1016/S0950-3579(97)80048-9","url":null,"abstract":"<div><p>Vasculitis, one of the clinical features shared by connective tissue diseases, should be considered when signs and symptoms are observed that may result from tissue ischaemia due to damaged vessels. The lesions seem to result from specific and non-specific immunopathogenic mechanisms targeted at the vascular endothelium. Because of the therapeutic implications it is the physician's responsibility to document its presence and the extent of organ involvement. Prompt institution of immunosuppressive drugs may be lifesaving. On the other hand there are some forms of vasculitis accompanying connective tissue disease which are entirely benign. Patients with infarctions of extremities and progressive functional disturbances of the central nervous system or internal organs because of vasculitis should be treated with high dosages of corticosteroids in combination with cytostatic drugs. Remissions are frequently obtained within three to six months of initiation of treatment and can be maintained with a less aggressive treatment regimen.</p></div>","PeriodicalId":77032,"journal":{"name":"Bailliere's clinical rheumatology","volume":"11 2","pages":"Pages 315-334"},"PeriodicalIF":0.0,"publicationDate":"1997-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3579(97)80048-9","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20164508","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
MD, PhD Cees G.M. Kallenberg (Professor of Clinical Immunology)
{"title":"9 Laboratory findings in the vasculitides","authors":"MD, PhD Cees G.M. Kallenberg (Professor of Clinical Immunology)","doi":"10.1016/S0950-3579(97)80051-9","DOIUrl":"10.1016/S0950-3579(97)80051-9","url":null,"abstract":"<div><p>The primary vasculitides are diseases of unknown aetiology. They are characterized by inflammation of blood vessel walls. Measuring non-specific laboratory markers of inflammation is useful in the monitoring of patients with vasculitis. The diagnostic specificity of these markers is, however, restricted. In the last decade, autoantibodies reacting with myeloid granule proteins have been detected in the sera from patients with Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome, and the renal limited form of these vasculitides (i.e. idiopathic rapidly progressive glomerulonephritis). Anti-neutrophil cytoplasmic antibodies (ANCA) in the aforementioned disorders react with proteinase 3 (Pr3) or myeloperoxidase (MPO), and only incidentally to other antigens such as elastase and bactericidal-permeability increasing protein. The presence of ANCA alone, in particular perinuclear ANCA, as detected by indirect immunofluorescence, has a low specificity for those vasculitides. However, in combination with the presence of anti-Pr3 or anti-MPO antibodies as detected by enzyme-linked immunosorbent assay, sensitivity and specificity for the vasculitides is high. Several in vitro and in vivo data have suggested a pathophysiological role for anti-Pr3 and anti-MPO in the associated disorders. Measuring levels of the autoantibodies seems useful for the follow-up of patients with these vasculitides. The sensitivity and specificity of rises in ANCA levels for ensuing relapses appears somewhat lower than previously suggested. Refinement of the assays, for example, by measuring subclasses and functional characteristics of the autoantibodies, may improve their value in monitoring patients with vasculitides.</p></div>","PeriodicalId":77032,"journal":{"name":"Bailliere's clinical rheumatology","volume":"11 2","pages":"Pages 395-421"},"PeriodicalIF":0.0,"publicationDate":"1997-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3579(97)80051-9","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20164511","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
MA, DM, MRCP Richard A. Watts (Consultant Rheumatologist), MD, FRCP David G.I. Scott (Consultant Rheumatologist, Honorary Senior Lecturer,)
{"title":"1 Classification and epidemiology of the vasculitides","authors":"MA, DM, MRCP Richard A. Watts (Consultant Rheumatologist), MD, FRCP David G.I. Scott (Consultant Rheumatologist, Honorary Senior Lecturer,)","doi":"10.1016/S0950-3579(97)80043-X","DOIUrl":"10.1016/S0950-3579(97)80043-X","url":null,"abstract":"<div><p>The systemic vasculitides are rare inflammatory conditions of blood vessel walls. A number of different classification schemes have been published since the first in 1952. The important developments have been the recognition of dominant blood vessel size, the distinction between primary and secondary vasculitis and the incorporation of pathogenic markers such as anti-neutrophil cytoplasmic antibodies. In 1990 the American College of Rheumatology (ACR) published criteria for the diagnosis of polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, hypersensitivity vasculitis, Schönlein-Henoch purpura, giant cell arteritis and Takayasu arteritis. Sensitivity and specificity rates varied considerably: 71.0–95.3% for sensitivity and 78.7–99.7% for specificity. The critieria were not tested against the general population or against patients with other connective tissue diseases or rheumatic conditions. Four years later the Chapel Hill Consensus Conference (CHCC) produced definitions for the major types of vasculitis, however, these have proved controversial. Comparison in unselected patients with systemic vasculitis (in particular polyarteritis nodosa and microscopic polyangiitis) has shown that the ACR criteria and CHCC definitions identify different patients. The systemic vasculitides are somewhat more common than previously believed. The overall annual incidence approaches 40/million adults. The most common form of primary systemic vasculitis is giant cell arteritis; Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome have similar incidences. Classical polyarteritis nodosa and Takayasu arteritis are very rare in the UK.</p></div>","PeriodicalId":77032,"journal":{"name":"Bailliere's clinical rheumatology","volume":"11 2","pages":"Pages 191-217"},"PeriodicalIF":0.0,"publicationDate":"1997-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3579(97)80043-X","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20164567","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"2 Biopsy diagnosis of systemic vasculitis","authors":"MD, MS, FACC, FACA, FACR, FCCP J.T. Lie (Professor of Pathology)","doi":"10.1016/S0950-3579(97)80044-1","DOIUrl":"10.1016/S0950-3579(97)80044-1","url":null,"abstract":"<div><p>A definitive diagnosis of virtually all vasculitides requires histological documentation. Although each major type of systemic vasculitis may have its own unique features, variability and overlaps still exist, and histopathological specificity is rarely an absolute discriminator. The interpretation of biopsies for the diagnosis of vasculitis remains more an art than a science, and it requires full and complete correlation with historical, clinical, laboratory, and angiographic findings.</p></div>","PeriodicalId":77032,"journal":{"name":"Bailliere's clinical rheumatology","volume":"11 2","pages":"Pages 219-236"},"PeriodicalIF":0.0,"publicationDate":"1997-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3579(97)80044-1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20164568","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"5 Large vessel vasculitis (giant cell arteritis, Takayasu arteritis)","authors":"MD William S. Wilke (Senior Staff Rheumatologist)","doi":"10.1016/S0950-3579(97)80047-7","DOIUrl":"10.1016/S0950-3579(97)80047-7","url":null,"abstract":"<div><p>Giant cell arteritis and Takayasu arteritis are separate but similar idiopathic diseases clinically characterized by constitutional symptoms, shared surrogate markers of systemic inflammation and indistinguishable granulomatous panarteritis of large vessels. This review emphasizes and analyses changing perceptions about the diseases. Recent series suggest that aortic involvement in giant cell arteritis may be more common than was previously appreciated. The case for and against inflammatory arthritis in giant cell arteritis is discussed. Ethnic and geographical variation in Takayasu arteritis-disease expression is reviewed. New philosophies of treatment are presented for both diseases. Prognosis in giatn cell arteritis and its relationship to treatment is analysed. The utility of the laboratory for diagnosis and monitoring disease activity is appraised for each.</p></div>","PeriodicalId":77032,"journal":{"name":"Bailliere's clinical rheumatology","volume":"11 2","pages":"Pages 285-313"},"PeriodicalIF":0.0,"publicationDate":"1997-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3579(97)80047-7","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20164507","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
MD Cem Mat (Professor of Dermatology), MD Sebahattin Yurdakul (Professor of Medicine), MD Nükhet Tüzüner (Professor of Pathology), MD Yalçin Tüzün (Professor of Dermatology)
{"title":"3 Small vessel vasculitis and vasculitis confined to skin","authors":"MD Cem Mat (Professor of Dermatology), MD Sebahattin Yurdakul (Professor of Medicine), MD Nükhet Tüzüner (Professor of Pathology), MD Yalçin Tüzün (Professor of Dermatology)","doi":"10.1016/S0950-3579(97)80045-3","DOIUrl":"10.1016/S0950-3579(97)80045-3","url":null,"abstract":"<div><p>Cutaneous vasculitis is a heterogeneous group of disorders, which can be confined to the skin or may be part of an associated systemic disease. Various aetiological agents as well as conditions that mimic skin vasculitis, usually present with similar clinical features; mainly palpable purpura. The skin biopsies usually show leukocytoclastic vasculitis. This poses a great diagnostic and therapeutic challenge for the physician. The aetiologies, clinical features, diagnosis and treatment modalities for each form (drugs, infections, malignancies, systemic vasculitides, connective tissue disorders, Schönlein-Henoch purpura, cryoglobulinaemia, cutaneous periarteritis nodosa, livedoid vasculitis, erythema elevatum diutinum and urticarial vasculitis) are reviewed.</p></div>","PeriodicalId":77032,"journal":{"name":"Bailliere's clinical rheumatology","volume":"11 2","pages":"Pages 237-257"},"PeriodicalIF":0.0,"publicationDate":"1997-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-3579(97)80045-3","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20164569","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}