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Overview of the management of vasculitis in adults

Peter A Merkel, MD, MPH
Section Editor
Eric L Matteson, MD, MPH
Deputy Editor
Monica Ramirez Curtis, MD, MPH


The vasculitides are defined by the presence of leukocytes in the vessel wall with reactive damage to mural structures. In general, affected vessels vary in size, type, and location in association with the specific vasculitic disorder. These conditions are often serious and sometimes fatal diseases that require prompt recognition and therapy.

Symptomatic involvement may occur in single or multiple organs. Specific clinical manifestations may suggest a particular vasculitic disorder, but significant overlap is observed among the vasculitides. The wide clinical spectrum of disease in the vasculitides presents particular challenges to management of these complex disorders.

This topic review will provide an overview of the approach to the management of the vasculitides. Treatment recommendations for specific forms of vasculitis are reviewed for each disorder separately (see 'Disease-specific treatments' below), and an overview of the classification and clinical manifestations of the different vasculitides and the approach to the patient with suspected vasculitis is presented separately. (See "Overview of and approach to the vasculitides in adults".)


The framework for the treatment of vasculitis is similar to that used for many other systemic autoimmune rheumatic disorders, while the specific therapeutic regimen depends upon the nature and severity of the particular disorder (see 'Disease-specific treatments' below). The management scheme for vasculitis generally includes the following components:

Remission induction – The goal of initial treatment is to induce remission of the disease. Initial management usually involves use of medium to high doses of glucocorticoids, with the use of an additional immunosuppressive agent in some forms of disease. The initial presentation of vasculitis is often rapid, and delay in diagnosis or failure to recognize the extent of disease and arrest the disease process can lead to substantial morbidity and, in some forms of disease, mortality. The initial treatment phase may thus be more intensive than the subsequent phases of treatment, involving the use of higher doses or drugs with greater risk of toxicity.


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Literature review current through: Sep 2016. | This topic last updated: Oct 14, 2015.
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