Does One Size Fit All?

In 1954, when Godman and Churg reviewed 22 published cases with granulomatosis with polyangiitis (GPA; previously Wegener granulomatosis), it was concluded that patients suffered from a triad of pathological features: (1) systemic necrotizing angiitis, (2) necrotizing granulomatous inflammation of the respiratory tract, and (3) necrotizing glomerulonephritis1. At that time, the insidious onset of the disease was already appreciated. From these first cases, it was postulated that it should be possible to make an early diagnosis before the full-blown disease is manifest. In 1966, Carrington and Liebow presented the first patients with more limited forms of GPA2. These cases were identical in clinical and pathologic manifestations when compared to the extended form of the disease except for the absence of renal involvement. Most importantly, it was questioned whether this limited form represents a unique and separate entity or an earlier state of the same disease. Nearly 5 decades later, this question is still valid. Nonetheless, the European Vasculitis Society (EUVAS) set out in the 1990s to harmonize treatment strategies for the different stages of disease. It was agreed that immunosuppressive treatment should reflect the severity of the disease3. During the last decade, results from clinical trials have been published, and more recently, data on longterm followup of patients included in these trials have been reported. In this editorial, we will discuss whether there are indeed different disease states in antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) and question whether treatment should be tailored to these states. Since the seminal paper of Carrington and Liebow, several definitions have been used to characterize the severity and/or extent of the disease. The classification of the EUVAS group and the Wegener’s Granulomatosis Etanercept Trial (WGET) group are best known and will be discussed here (Figure 1)3,4 … Address correspondence to Dr. Cohen Tervaert, PO Box 5800, 6202AZ Maastricht, The Netherlands. E-mail: jw.cohentervaert{at}maastrichtuniversity.nl