The systemic vasculitides are relatively uncommon but important conditions which cause significant morbidity and mortality and often confusion at first presentation to a broad spectrum of clinicians. Recent advances in treatment have made it possible for most conditions to be effectively controlled and sometimes even cured. Thirty or forty years ago, these were diseases with a high mortality, but the introduction of appropriate immunosuppressive treatment has changed the outcome for most patients, such that they are now part of the spectrum of chronic inflammatory diseases requiring ongoing treatment and monitoring.

The aim of this book is to provide easy access to the common clinical presentations of the systemic vasculitides, the investigations required to assess the severity of the disease as well as diagnose specific vasculitic syndromes and an approach to treatment based on the important studies undertaken over the last 15 years, particularly within Europe, using conventional immunosuppressive drugs and also more recent studies looking at the role of modern biologic therapy.

There have been major changes in our understanding of the effects of treatment of vasculitis. Recently completed studies described in this book, involve the current use of intravenous pulse cyclophosphamide which is almost as effective as continuous oral cyclophosphamide but safer and the role of B-cell depletion which is as effective in inducing remission as cyclophosphamide, but with potentially less long term toxicity. Azathioprine and methotrexate are equally effective in maintaining remission after induction remission with cyclophosphamide and/or B cell depletion. At present there is also convincing evidence that patients with particularly severe systemic vasculitis (especially those with severe renal and pulmonary disease) are more responsive to plasma exchange as compared to intravenous methylprednisolone.

The long-term outcome for most vasculitides has substantially improved over the last two or three decades but there is still an early mortality (as high as 5 or 10 %) in the case of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis. As outlined above, vasculitis is usually brought under control with intensive immunosuppression and maintained by lower dose/less intensive drug treatment and then it is seen as a more chronic, sometimes relapsing disease with an accumulative mortality and morbidity which after 10 years or so may be influenced by an increased cardiovascular risk.