Oxford Textbook of Vasculitis (Oxford Textbooks in Rheumatology) 3rd Edition

Oxford Textbook of Vasculitis (Oxford Textbooks in Rheumatology) 3rd EditionThe third edition of this definitive and highly-regarded reference work provides a comprehensive review of vasculitis, a fascinating array of life-threatening and minor conditions caused by inflammatory syndromes and diseases that affect the blood vessels. The text uniquely brings together concepts from both the biological and clinical aspects of vasculitis.

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Research in clinical immunology now invigorates the entire area of vasculitis and shapes a rational approach to pathogenesis, diagnosis, and treatment, which is the substance of this text. Over 40 chapters cover known vasculitic conditions, and are illustrated with over 250 full-colour photographs of clinical and pathologic findings, diagrams, and tables. Separate sections are devoted to basic science, clinical manifestations commonly seen in vasculitis, imaging and percutaneous interventions, individual diseases and syndromes, as well as conditions which can mimic vasculitis. Many of the conditions are rare, and it is the exceptional clinician who has much experience with more than a few of these; thus, the invaluable experience of the international team of authors makes this edition truly indispensible.

Now in the Oxford Textbooks in Rheumatology series, this new edition of Vasculitis is published with a concurrent online version, which features access to the full content of the textbook, contains links from the references to primary research journal articles, allows full text searches, and provides access to figures and tables that can be downloaded to PowerPointRG.

This volume is the definitive reference for rheumatologists, clinical immunologists, and general internists, and will also be of interest to dermatologists, gastroenterologists, cardiologists, pulmonologists, nephrologists, neurologists, pathologists, vascular surgeons, and pediatricians.

Oxford Textbook of Vasculitis (Oxford Textbooks in Rheumatology) 3rd Edition
by Gene V. Ball (Editor), Barri J. Fessler (Editor), Bridges Jr., S. Louis (Editor)
ISBN-13: 978-0199659869
ISBN-10: 0199659869

Oxford Textbook of Vasculitis 3rd Edition – Content

Abbreviations vii
Contributors xi
Introductory chapters
1. Nomenclature and classification
of vasculitic syndromes 3
Gene V. Ball and S. Louis Bridges, Jr
2. Epidemiology of vasculitis 7
Richard A. Watts and David G.I. Scott
Basic science
3. Hypersensitivity 29
T. Prescott Atkinson
4. Biology of endothelial cells 41
Zoltán Szekanecz, György Kerekes, and Alisa E. Koch
5. Complement in vasculitis 53
John E. Volanakis
6. Autoantibodies in vasculitis 61
Jan Damoiseaux and Jan Willem Cohen Tervaert
7. Pathogenesis of vasculitis 71
Gene V. Ball, S. Louis Bridges, Jr, and T. Prescott Atkinson
8. Animal models of vasculitis 93
Peter Heeringa
9. Pathological features of vasculitis 101
Andrew Churg
Clinical manifestations common
to vasculitis
10. Cutaneous manifestations of vasculitis 121
Nicole M. Fett and Victoria P. Werth
11. Ocular manifestations of systemic
vasculitis 131
Russell W. Read
12. Cardiopulmonary manifestations
of vasculitis 143
Stephen K. Frankel and Marvin I. Schwarz
13. Vasculitic neuropathy 157
Shin J. Oh
14. Vasculitic manifestations in the
gastrointestinal tract 179
Gaafar Ragab
15. Renal manifestations 197
Cees G.M. Kallenberg and Jan Willem Cohen Tervaert
16. Digital ischaemia and Raynaud’s
phenomenon 209
K. Kwasind Huston, John H. Stone, and Fredrick M. Wigley
Imaging and percutaneous
17. Angiography and percutaneous
interventions 227
Souheil Saddekni and Rachel F. Oser
vi contents
18. Cross-sectional imaging in vasculitis 247
Enrique A. Sabater and Anthony W. Stanson
19. PET imaging in vasculitis 267
Daniel Blockmans
Vasculitic diseases and syndromes
and related disorders
20. Approach to the diagnosis of
vasculitis in adult patients 275
Barri J. Fessler
21. Vasculitis in infancy, childhood,
and adolescence 283
Ross E. Petty
22. Assessment of disease activity and damage 299
Raashid Luqmani
23. Giant cell arteritis and polymyalgia
rheumatica 307
Kenneth J. Warrington and Cornelia M. Weyand
24. Takayasu’s arteritis 319
Yasushi Kobayashi, Tomohiro Ishii, and Hideo Harigae
25. Polyarteritis nodosa 331
Loïc Guillevin and Benjamin Terrier
26. Microscopic polyangiitis 351
Loïc Guillevin and Benjamin Terrier
27. Cutaneous polyarteritis 363
Angelo L. Gaffo
28. Kawasaki’s disease 373
Jane C. Burns
29. Granulomatosis with polyangiitis (Wegener’s
granulomatosis): pathogenesis 385
Peter Lamprecht, Konstanze Holl-Ulrich,
and Wolfgang L. Gross
30. Granulomatosis with polyangiitis
(Wegener’s granulomatosis): clinical
and immunodiagnostic aspects 401
Susanne Schinke, Wolfgang L. Gross, and Elena Csernok
31. Granulomatosis with polyangiitis (Wegener’s
granulomatosis): treatment 417
Julia U. Holle, Bernhard Hellmich, and Wolfgang L. Gross
32. Eosinophilic granulomatosis with polyangiitis
(Churg–Strauss syndrome) 433
Jeremy M. Clain and Ulrich Specks
33. Vasculitis in primary connective
tissue diseases 443
Laura B. Hughes
34. Behçet’s syndrome: pathogenesis, clinical
manifestations, and treatment 467
Emire Seyahi, Gulen Hatemi, Izzet Fresko,
Melike Melikoglu, and Hasan Yazici
35. Juvenile Behçet’s syndrome 491
Gülsevim Azizlerli and Rifkiye Sarica-Kucukoglu
36. Vasculitis of the central nervous system 497
Rula A. Hajj-Ali, George F. Duna,
and Leonard H. Calabrese
37. Thromboangiitis obliterans
(Buerger’s disease) 507
Michael Frank and Jean-Noel Fiessinger
38. Cutaneous small-vessel vasculitis 517
Anna Haemel, Lindy Fox, and M. Kari Connolly
39. IgA vasculitis (Henoch-Schönlein purpura) 527
Miguel A. González-Gay, Ricardo Blanco,
and Trinitario Pina
40. Cryoglobulinaemic vasculitis 547
Massimo Galli, Salvatore Sollima, Francesco
Saccardo, and Giuseppe Monti
41. Miscellaneous forms of vasculitis 569
Gim Gee Teng, Sumapa Chaiamnuay,
and W. Winn Chatham
42. Experimental therapies for vasculitis 599
Sebastian Unizony and John H. Stone
Mimickers of vasculitis
43. Antiphospholipid syndrome 615
David P. D’Cruz, Munther A. Khamashta,
and Graham R.V. Hughes
44. Imitators of vasculitis 635
Sharon A. Chung and Kenneth E. Sack
Index 657

Oxford Textbook of Vasculitis 3rd Edition – SECTION 1

Introductory chapters


Nomenclature and classification of vasculitic syndromes

Gene V. Ball and S. Louis Bridges, Jr

The inadequacies of schemes for classifying vasculitis, and their misapplication to the diagnosis of an individual’s illness, are well recognized. The most frequently cited classification in the recent past was devised by a committee of the American College of Rheumatology (ACR) for the purpose of facilitating research (Bloch et al. 1990; Fries et al. 1990; Hunder et al. 1990a).

The authors of this scheme for classifying seven distinct vasculitic syndromes (Arend et al. 1990; Calabrese et al. 1990; Hunder et al. 1990b; Leavitt et al. 1990; Lightfoot et al. 1990; Masi et al. 1990; Mills et al. 1990) stated explicitly that the criteria were not meant for diagnostic purposes in an individual patient even though they
have been useful in differentiating one vasculitis syndrome from another.

Recognizing the widespread use of the criteria for diagnosis, Rao, Allen, and Pincus (1998) evaluated their effectiveness in diagnosing Wegener’s granulomatosis (WG) (now referred to as granulomatosis with polyangiitis), giant cell arteritis (GCA), polyarteritis nodosa (PAN), and hypersensitivity vasculitis in 198
patients who were referred to medical centres with diagnoses of possible vasculitis. They concluded that when the criteria were applied to these patients, their positive predictive values for WG, GCA, PAN, and hypersensitivity vasculitis ranged from only 17 to 29%.

Thus, there is empirical evidence that the ACR criteria should not be used for diagnosis of specific syndromes in a patient who does not have a confirmed diagnosis of vasculitis. This is in accordance with the intent of the committee: that the criteria not be used to distinguish vasculitis from other diseases, and that the criteria
were established to insure uniformity in epidemiological and other research studies (Hunder et al. 1990a). The ACR and later definitional criteria require emphatically that vasculitis be established as a prerequisite for defining a specific vasculitic syndrome.

For most syndromes, a histological diagnosis of vasculitis, whose classical features are endothelial swelling, inflammatory infiltrates, and fibrinoid necrosis, has been required or recommended strongly but
the biopsy findings must be correlated with clinical and laboratory data, and with the understanding that the histological features of vascular lesions may change with time (see Chapter 9). The concept of surrogate markers of vasculitis such as antineutrophil cytoplasmic antibodies (ANCA) and imaging studies, which are not features of the ACR classification criteria, recognizes that histological diagnosis is not always possible.

Although the ACR classification criteria have been useful for establishing uniform criteria for inclusion of patients in research studies, they were promulgated before widespread availability of reliable assays for ANCA. They did not differentiate PAN and microscopic polyangiitis (MPA); however, the specificity for granulomatosis with polyangiitis (WG) and eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome (CSS)) appear to be 92% and 99.7%, respectively.

Because of confusion concerning classification vis-à-vis diagnosis, the dictionary definitions of these two words are useful. The Oxford English Dictionary defines classification as arranging in classes according to common characteristics or affinities. Diagnosis is defined as identification of a disease by careful investigation of
its symptoms and history, or distinctive characterization in precise terms. The definition of diagnosis stresses the identification of the nature and cause of a disease through evaluation of all clinical and laboratory data.

Jennette et al. (1994), in their influential proposal on the nomenclature of systemic vasculitides, observed
that ‘it is advantageous for everyone to use the same name for the same disease, and to do this, there must be clear agreement on the definition of the name and therefore of the disease’. This seems straightforward; however, while acknowledging that a diagnosis is, in essence, the name of a disease, they disavowed determination of
clinical criteria required to classify or diagnose individual patients.

Diagnostic criteria would require analysis of large numbers of patients with unequivocal defining features. Jennette et al. (1994) tabulated the names and definitions of ten of the more common vasculitides. These definitions are expanded in the chapters of this text dealing with individual diseases. The ambiguities of clinical presentations, limited diagnostic laboratory tests, and difficulty in obtaining appropriate tissue for histological examination (which may not provide categorical information) may impede precise diagnosis/ classification at any time.

Furthermore, a diagnosis that is perfectly tenable at a given time, may later be inappropriate for a patient whose disease has evolved over time. This is particularly true of cutaneous leukocytoclastic angiitis, probably the most common of the vasculitides, which may be the sole manifestation of a vasculitis that later blossoms into a systemic disease. This concept of a changing clinical condition, requiring the rethinking of a diagnosis, applies as well to many  non-vasculitic diseases……….

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