Eleven Themes in the History of Systemic and Nervous System Vasculitides.

Vasculitis is defined as inflammation of blood vessel walls for at least some time during the course of the disease, and affects arteries and veins of varying caliber. Two Chapel Hill Consensus Conferences, in 1994 and 2012, provide consensus on nosology and definitions for the commonest forms of vasculitis. The category of single-organ vasculitis, suggesting the limited expression of a systemic vasculitis, includes primary central nervous system vasculitis and nonsystemic peripheral nervous system vasculitis. The historical aspects of systemic and limited forms of vasculitis are reviewed in 11 relevant themes.

A Closer Look at Angiitis of the central nervous system.

Angiitis of the central nervous system (ACNS) is a rather new disease entity that is defined as vascular inflammation limited to the central nervous system and was formally nominated in 1959. Etiologically, it can be divided into primary and secondary ACNS. However, the potential pathogenesis of ACNS remains unclear. The clinical presentation is variable, and there is no consensus concerning its diagnosis and management. Although the incidence is relatively low, ACNS is still a life-threatening condition. It is essential to get a comprehensive and updated understanding of the disease. In this paper, we reviewed the history, definition, classification, pathogenesis, and clinical manifestations of ACNS. In addition, we focused on the latest investigations and viewpoints regarding the diagnosis and treatment of ACNS.

Nosology of primary vasculitis.

PURPOSE OF REVIEW: Recent developments in the nosology of primary systemic vasculitis are placed in the context of an historical overview. The ongoing attempts to develop criteria for classification and diagnosis are discussed. RECENT FINDINGS: Giant cell arteritis has supplanted temporal arteritis as the preferred term for chronic granulomatous arteritis in older adults. A new classification system for childhood vasculitis has been proposed by a European collaborative group. A study of idiopathic polyarteritis nodosa demonstrates greater similarity to microscopic polyangiitis in relapse rate than previously reported. Controversy has arisen over the eponym Wegener's granulomatosis because of alleged involvement of Friedrich Wegener in the Nazi regime during World War II. Diagnostic criteria for Kawasaki disease are problematic because many patients with coronary artery involvement do not fulfill current criteria at the time of presentation. Classification of antineutrophil cytoplasm autoantibody-associated small vessel vasculitis based on antineutrophil cytoplasm autoantibody specificity has been complicated by the finding that different ethnic groups may have very different clinical features relative to antigen specificity; for example, most patients with Wegener's granulomatosis in China have myeloperoxidase-antineutrophil cytoplasm autoantibodies rather than proteinase 3-antineutrophil cytoplasm autoantibodies. SUMMARY: Within the past year, new classification systems for primary vasculitis have been proposed, new classification criteria have been developed, and the appropriateness of a longstanding eponym has been challenged.