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Recent developments in the classification and assessment of vasculitis

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The systemic vasculitides are a group of multisystem diseases characterized by inflammation of blood vessels. The aetiopathogenesis is unknown, and therefore nomenclature and classification are often descriptive and based on pathological features. Generally agreed classification schemes are vital to enable large multicentre or multinational clinical trials to be undertaken. An algorithm has recently been developed to harmonize use of the American College of Rheumatology (ACR) 1990 criteria and the Chapel Hill Consensus Conference definitions. Despite this, a revision of the classification criteria is still needed, and diagnostic criteria need to be developed ab initio. The very complexity of the diseases makes accurate objective assessment critical, especially for the conduct of clinical trials. Several standardized assessment tools for both disease activity and damage have been developed over the past two decades and are now widely used in both clinical trials and routine practice. A second generation of tools is now under development.

Section snippets

Historical background

Kussmaul and Maier [1] are generally accepted as having provided the first description of ‘periarteritis nodosa’ when they described a patient with a systemic illness characterized by numerous nodules along the course of small muscular arteries. Earlier descriptions suggest that the formal recording of this condition is at least 200 years old (reviewed by Matteson [2].

Zeek [3] reviewed the literature relating to vasculitis and periarteritis nodosa, and used the generic term ‘necrotizing

Assessment of vasculitis

The assessment of patients with systemic vasculitis has become much more objective over the past 20 years with the development of several scoring systems. All of these were originally developed for use in clinical trials, but are increasingly used in routine clinical practice.

The systemic vasculitides were until relatively recently associated with significant mortality and morbidity. However, modern treatment protocols have converted these diseases into chronic relapsing remitting conditions.

Summary

Many of the systemic vasculitides are of unknown causation, and therefore the current classification tools are based on clinical features and pathology. The validated ACR criteria predate the introduction of ANCA testing and the widespread recognition of MPA as a specific entity. The CHCC recognized MPA but did not incorporate ANCA. Use of the two systems results in conflicting classification, which has been partially resolved by the development of the EMEA algorithm which incorporates both

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