WEGENER'S GRANULOMATOSIS & ANCA-ASSOCIATED DISEASES: THE STORY CONTINUES The disease now designated as Wegener's granulomatosis (WG) was first described in 1931 by Heinz Klinger, who considered it to be a special form of polyarteritis nodosa. Klinger's friend, Friedrich Wegener, expanded on the first observations and interpreted the pathological and clinical fmdings to represent a distinct disease entity (Wegener, 1939). He described this entity as a "peculiar rhinogenous granulomatosis with a unique participation of the arterial system and the kidneys". Later, Godman and Churg (1954) established the classical diagnostic criteria (the "WG triad"): granuloma, vasculitis, and glomerulonephritis. In 1958 Walton pointed out the poor prognosis of WG based on a small number of published cases (mean survival time: 5 months). In 1966 Carrington and Liebow reported "limited forms" of WG with a defmitely more favorable prognosis. Since then positive results have been reported with cyclophosphamide therapy. In addition, a retrospective study of combined low-dose cyclophosphamide and prednisolone in 85 WG patients over a period of 21 years found a similarly encouraging outcome. The·latter experience led to the current "standard" treatment protocol (FAUCI et al., 1973 and 1983). More recently, strong evidence has emerged that some of the morbidity and mortality ofWG - and other types of systemic vasculitis - may be a consequence of this treatment (Hoffman et al., 1992).

Preface: Pathophysiological Athophysiological Aspects and Antigen Specificity: cANCA-Targets. Proteinase 3 etc. pANCA-Targets: a. Myeloperoxidase. pANCA-Targets: b. Other Lysosomal Antigens. ANCA-Targets: Endothelial Cell Elements. Animal Studies and Animal Models. Clinical aspects of Wegener''s Granulomatosis and other ANCA Associated Diseases: Wegener''s and ANCA Associated Vasculitis. Therapeutic Strategies. ANCA and Inflamatory Bowel and Liver Diseases: Gastroenterologic Aspects. 100 articles. Index.