Do vasculitis categorization systems really matter?

Abstract

The diagnosis of systemic vasculitides is challenging for many reasons. The etiology and pathogenesis of most vasculitides are unknown or incompletely known. Vasculitides have protean and overlapping clinical and pathologic features. There are conflicting if not contradictory approaches to diagnostic categorization. In spite of these challenges, precise diagnostic categorization is essential for appropriate treatment. This overview reviews the history behind the modern approach to diagnosis of selected vasculitides, including giant cell arteritis, Takayasu arteritis, polyarteritis nodosa, Kawasaki disease, Henoch-Schönlein purpura, cryoglobulinemic vasculitis, Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. Evidence is provided that the categorization for systemic vasculitis really does matter.