Localized, single-organ vasculitis: clinical presentation and management

Abstract

The term vasculitis usually evokes a systemic disease with catastrophic outcomes; however, vasculitides may also present in a localized form, with a better prognosis when compared with their systemic counterpart. In order to avoid confusion and facilitate classification, the term single-organ vasculitis (SOV) has been proposed. Remarkably, current criteria for the classification of the vasculitis do not include the SOV term, due in part to the lack of appropriate definitions, since most data come from case series; moreover, the scarce information available is also extremely heterogeneous. This review focuses on the epidemiology, clinical course, prognosis, and suggested treatment of the SOV, with emphasis in the most recent information available.