Overview of the management of vasculitis in adults
Overview of the management of vasculitis in adults
Author:
Peter A Merkel, MD, MPH
Section Editor:
Kenneth J Warrington, MD
Deputy Editor:
Philip Seo, MD, MHS
Literature review current through: Mar 2024.
This topic last updated: Jun 09, 2023.

INTRODUCTION

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".)

APPROACH TO TREATMENT

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 medium to high doses of glucocorticoids, with the addition of an immunosuppressive agent in most forms of disease. The initial presentation of vasculitis is often rapid, and delay in diagnosis or failure to recognize the extent of disease or 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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