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The way forward in approaching a diagnosis of vasculitis

Vasculitides are a group of related disorders characterized by inflammation of blood vessels leading to tissue or end-organ injury. However, the classification of vasculitis has been controversial for many years. In 1990, the ACR proposed the criteria for classification of some vasculitides based on an analysis comparing the clinical features of patients with seven types of established vasculitis: GCA, Takayasu’s arteritis (TA), WG, Churg–Strauss syndrome (CSS), PAN, HScP and hypersensitivity vasculitis (HSV). The sensitivities of these criteria ranged from 71.0 to 95.3% and the specificities ranged from 78.7 to 99.7% [1].

The ACR criteria have been widely accepted and used, and helped advance the field of vasculitis research; however, over the past 20 years, some important limitations have been recognized that affect the application of the criteria to both clinical research and practice. Patients do not always fall neatly into one of the seven categories or overlap between two or more classifications. For example, a patient who presents with weight loss, myalgias, polyneuropathy, urinary sediment, respiratory symptoms, pulmonary infiltrates on chest radiograph and paranasal sinus abnormalities could potentially fit the ACR classification criteria for PAN, CSS or WG.

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