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 pan-arteritis 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 new geographical variation in Takayasu arteritis-disease expression is reviewed. New philosophies of treatment are presented for both diseases. Prognosis in giant cell arteritis and its relationship to treatment is analysed. The utility of the laboratory for diagnosis and monitoring disease activity is appraised for each.

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 myeloperxoidase 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.

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 diagnositc 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.

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.

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 criteria 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.

Guidelines provide explicit recommendations and seek to influence practice using a formal process to disseminate advice on most effective management in the light of scientific evidence. They provide a framework for the evaluation and treatment of common clinical problems, but are not intended to replace clinical judgement. There is considerable variation in rheumatology practice, fueled by uncertainty about the optimal measurement of disease outcome. Guidelines can help identify and eliminate ineffective or unnecessary care as they are systematically developed statements to assist practitioners and patients' decisions about appropriate health care. There are North American guidelines for the initial evaluation of adult patients with acute musculoskeletal symptoms, the management of rheumatoid arthritis and the management of osteoarthritis. These are discussed together with proposed guidelines for the management of early rheumatoid arthritis and areas of research into the value of guidelines. It is recommended that future work in this area should: (i) identify aspects of current guidelines which are directly related to outcome; (ii) educate clinicians in these aspects of care; (iii) ensure they are introduced into practice and the outcome of care subsequently improves; (iv) regularly update the guidelines to reflect current opinion. Present guidelines that give broadly similar recommendations from North American and UK perspectives. However, the available evidence all points to large variations between how clinicians practise and how they make their decisions and it may be unlikely that laying down exact recipes for practice will necessarily influence the clinician. Guidelines may appear relevant but they could prove to have very limited utility.

Economic evaluations of health-care technologies are playing an increasingly central role in determining which therapies are available to clinicians in the treatment of a whole range of conditions. In rheumatology, a large body of work has already been done on the cost effectiveness of alternative non-steroidal anti-inflammatory drugs (NSAIDs), and much of the current work on disease modifying therapies incorporates economic evaluations. This chapter describes the main techniques of economic evaluation and reviews the strengths and weaknesses of each. Two published economic evaluations are discussed in order to highlight what economic evaluations can offer to the care of people with rheumatoid arthritis, as well as the current limitations of economic evaluation. The objective of this chapter is to equip readers with a critical understanding of economic evaluation that can be used in considering the increasing volume of health economic data that they encounter in their clinical work.

Patient education is accepted as an essential component in the management of rheumatoid arthritis (RA) and this chapter provides an overview of patient education for practising clinicians. It includes an explanation of the need for patient education including the results of studies into what patients already know. The effectiveness of patient education and its benefits to patients are discussed in the light of recent research, reviews and meta-analyses. Alternative methods of delivering patient education are compared including, one-to-one teaching, opportunity education, group teaching and self-management programmes. Topics for inclusion in education programmes are suggested and the merits of written literature, audio-visual and computer assisted learning are explained. Practical guidance is given on methods of ensuring that written information is readily understandable by patients, including the use of readability formulae.

In increasingly cost-conscious, accountable and integrated health-care systems, the appropriate role of speciality care is under scrutiny. The data on the impact of rheumatologist care on outcomes in patients with rheumatoid arthritis (RA) is limited and inconclusive. However, based on a review of processes of care known to be related to superior patient outcomes it is suggested that rheumatologists should be the lead physicians in patients with RA. Rheumatologists but usually not generalists have the experience necessary to make an early diagnosis and to initiate appropriate disease modifying anti-rheumatic drug (DMARD) treatment. Rheumatologists have an in-depth understanding of new assessment methods to optimize medical treatment and to make best use of and co-ordinate multi-disciplinary care. To avoid delay of diagnosis and initiation of treatment, patients with polyarthritis should be referred to rheumatologists as soon as possible. This requires that access to rheumatologist care is guaranteed.

As the pathophysiology of rheumatoid arthritis (RA) becomes more clearly defined there is the expectation that biotechnological advances may allow additional forms of therapeutic intervention that are specific for the disease process. The purpose of this review is to describe the use of biological agents in the treatment of RA. Encouraging results in animal models using vaccines based on the pathogenic T-cell or the autoantigen have prompted the design of selective immune-based therapies. Preliminary studies following this strategy have not yet shown clinical efficacy. The results of early studies with monoclonal antibodies against leukocyte surface antigen were promising but were not sustained in controlled studies. Exciting data have been collected from placebo-controlled studies of monoclonal antibodies against TNF alpha. The development of biological agents in RA may not be as quick as expected but the steady progress makes it likely that our weapons to combat unwanted autoimmune responses will become more accurate and effective.

Treatment with slow-acting anti-rheumatic drugs (SAARDs) is nowadays initiated earlier in the disease course, preferably before any radiographic damage has occurred. SAARDs have the ability to decrease inflammatory synovitis as measured by clinical and laboratory variables, and there is some evidence that they improve physical function and decrease the progression rate of joint damage in patients with early rheumatoid arthritis. There is a clear difference in survival time between the various SAARDs. The efficacy/toxicity profiles of the SAARDs show equal variation. Rank order of prescription or disease duration may have an effect on drug survival, but different treatment strategies are also important sources of variation. Efficacy might be improved by combining different SAARDs (starting with a multiple drug regimen, or adding a drug to the first one), but further research is necessary to prove this hypothesis.

In rheumatoid arthritis nowadays a more aggressive treatment strategy is followed based on early consistent use of second-line agents frequently given in combination. This approach requires an accurate monitoring of the disease activity to follow the course of the disease and to evaluate therapeutic interventions. International consensus is reached over a core set of disease activity variables, including: a 28-joint count for tenderness and swelling, an acute phase reactant, patient's pain and global disease activity, physician's global disease activity, functional disability and radiographs. Guidelines for measurement techniques need to be further specified. Indices of disease activity are developed to improve the unambiguous interpretation of disease activity and comparability of trial results. These measures can be divided in measures for current disease activity and improvement criteria. Further validation will be necessary to adapt finally a uniform measurement technique. The usefulness of self-administered joint counts needs to be studied further.

This chapter will describe the reasons why prognostic factors that predict aggressive disease are helpful and what the problems are in interpreting studies in this field. A summary of cohort studies on prognosis of patients with early rheumatoid arthritis are presented. This is done separately for studies predicting radiographic damage, functional outcome and mortality. The overall conclusions of these studies and the value they have for the clinician are demonstrated.

The diagnosis of early rheumatoid arthritis (RA) has inherent difficulties. It requires assessment, not only of the current clinical picture, but of the potential for change. As the pathognomonic feature of RA, is persistence it is not surprising that the American College of Rheumatologists criteria perform better in predicting persistence than severity. An adequate histological/imaging method of diagnosing RA is awaited. In the interim, a pragmatic approach to defining disease has been suggested, which takes a homogeneous group of patients with persistent inflammatory small joint synovitis and secondarily stages them for severity. This proposal is currently being assessed for clinical usefulness.

It is likely that, over the next few years, the rapid technological advancements in ultrafast magnetic resonance imaging and ultrasound resolution improvements, coupled with a drive towards minimally invasive surgery, will further enlarge the scope of imaging guided interventional procedures. Such innovations should increase diagnostic accuracy for optimized treatment regimes as well as probably replace an increasing number of conventional 'open' operative procedures.

Imaging is likely to play an increasingly important role in clinical trials of antirheumatic drugs. The need to evaluate efficacy of a drug quickly often precludes the use of radiological evaluation as an outcome measure, particularly in chronic arthritides. New imaging techniques with the refinement to detect differential early change, often between drugs of comparable potency, would be advantageous. However, the trend to set up large scale multicentre trials that have adequate statistical power for the detection of change mitigates against the use of expensive imaging techniques of restricted access. The majority of drug trials, therefore, still resort to conventional radiology. With the new generation of drugs that have specific actions, sensitivity in comparing changes in synovium, cartilage, bone and adjacent soft tissues would be of value.

The steady improvement in densitometric technique is reviewed in this paper culminating in the newest generation of DXA scanners. The suitability of the lumbar spine, femur and hand as sites for the densitometric measurement of bone loss in rheumatoid disease is assessed. The recent improvement in hand scanning technique using DXA has led to improved clinical utility in the detection of juxta-articular bone loss. The enhanced resolution offered by the newest, solid state detector DXA scanners, and their ability to detect bone loss where no systemic features are evident, makes it possible that DXA scanning of the hand will be a useful modality for the monitoring of early rheumatoid disease.