The systemic vasculitides are characterized by inflammation of blood vessels resulting in end organ, or tissue damage or necrosis. They are defined by the Chapel Hill nomenclature according to the calibre of the predominantly affected vessels. Other forms of vasculitis not defined by a predominant vessel size are also recognized (e.g. Behcet's syndrome). Large vessel vasculitides include giant cell arteritis and Takayasu arteritis; medium vessel vasculitides includes Kawasaki disease and PAN; small vessel vasculitides are divided into: immune complex small vessel vasculitis and anti-neutrophil cytoplasm antibody (ANCA) - associated vasculitis (AAV). The immune complex group, with moderate to marked vessel wall deposits of immunoglobulin and/or complement, is represented by anti-glomerular basement membrane disease, cryobulinaemic vasculitis, hypocomplementaemic urticarial vasculitis (anti-C1q vasculitis) and IgA vasculitis (Henoch–Schönlein). By contrast, AAV has few or no immune deposits and is associated with (in most cases) the presence of ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). Depending on their clinical presentation and ANCA specificity, AAV is divided into three major variants: granulomatosis with polyangiitis (GPA) (Wegener's granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA) (Churg–Strauss syndrome). In some cases, vasculitis is relatively trivial and may lead to minor, often asymptomatic clinical features such as splinter haemorrhages. However, in severe forms of ANCA associated vasculitis, the consequences of rapid onset of ischaemia and occlusion of blood vessels can lead to organ failure and death.
The multisystem involvement in most forms of vasculitis can be a real challenge. Patients may present to different specialists resulting in diagnostic delay. The investigation of patients with suspected vasculitis should follow on from a careful history and examination to determine the likely diagnosis. The differential diagnosis is very wide. It is important to correctly identify patients with vasculitis as early as possible, but it is also important to rule out more common causes. In acutely unwell patients, the differential diagnosis depends on the combination of clinical features. Vasculitides tend to involve multiple organ systems. In fact, the more organ systems affected, the more likely it is that the patient has vasculitis. We will review some examples of cases with what might appear to be unusual clinical features which form a more consistent pattern. Initial treatment is generally straightforward, but the evaluation of patients during the course of their illness is often difficult due to variation in disease, as well as drug toxicity, damage and co-morbidity. We will discuss examples of patients where relapse is suspected but not always confirmed.
In this overview we will summarise current practice in vasculitis, illustrated by cases to provide a clinical context in which to interpret and implement evidence based management of vasculitis
Disclosure of Interest R. Luqmani Grant/research support from: Arthritis Research UK, GSK, MRC, UCSF/OIF, Canadian Institutes of Health Research, The Vasculitis Foundation;, Consultant for: GSK, Medpace, MedImmune, Roche
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